Chapter 58 - Congenital Heart Disease: Roger B B. Mee M.B. Ch.B. Jonathan J. Drummond-Webb M.B.B.CH

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Townsend: Sabiston Textbook of Surgery, 17th ed., Copyright © 2004 Elsevier
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Chapter 58 - Congenital Heart Disease
Roger B B. Mee M.B. Ch.B.

Jonathan J. Drummond-Webb M.B.B.Ch.
In this brief review, the impressive historical contributions are not discussed. The knowledge base of congenital cardiac disease has undergone a
quantum leap impossible to detail in this chapter. The development of pediatric and congenital cardiac surgery as a separate specialty has resulted in
superior outcomes in institutions adopting this policy. Congenital cardiac disease accounts for 0.8% to 1.0% of all live births. The spectrum of
anomalies ranges from isolated defects to complex lesions, with or without associated systemic abnormalities. Documentation of chromosomal
abnormalities has opened new vistas in this field, especially chromosome 22 microdeletions (velocardiofacial syndrome and DiGeorge’s syndrome
variants) as well as the association of many syndromes (e.g., Turner’s, Marfan’s, Williams’) with congenital cardiac abnormalities.[1]
Diagnosis relies on noninvasive methods, especially transthoracic echocardiography. This technology has advanced to such a degree that catheterization
is required in only certain circumstances, that is, when pressure measurement and specific morphologic details are required or for intervention. Prenatal
echocardiographic diagnosis of congenital lesions allows preemptive planning. Developments in echocardiography include three-dimensional and spin-
echo capabilities. Magnetic resonance imaging (MRI) and nuclear scanning have enhanced noninvasive diagnosis. Interventional catheterization has
become established as a management option, with balloon atrial septostomy, pulmonary and aortic valve dilation, device closure of defects, and major
blood vessel dilation and stenting.
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ANATOMY AND TERMINOLOGY
The assessment of congenital heart disease involves a systematic approach to the heart and its connections. The segmental approach involves
description and analysis of three elements (atria, ventricles, and outlet) and analysis of the nature of the junctions ( Fig. 58–1 ). Connections are
described as concordant or discordant (abnormal); chambers as left or right sided (morphologically); and the valve connections between chambers as
normal, absent, overriding, or straddling. Abnormal communications and specific morphologic anomalies are then described. The shorthand
nomenclature of Van Praagh[2] allows some detail to be communicated effectively and succinctly. The system utilizes a name followed by a sequence of
three letters. The first letter denotes the situs of atrial chambers and usually the abdominal and thoracic organs: S, solitus or normal; I, inversus or
inverse; and A, ambiguus or unknown. The second letter denotes the ventricular loop: d, right-hand topology and l, left-hand topology. The third letter
denotes the aortic valve position relative to the pulmonary valve position: d, right-sided and l, left-sided. The possible combinations are shown in Figure
58–2 .

CARDIOPULMONARY BYPASS AND MYOCARDIAL PROTECTION
Cardiopulmonary bypass (CPB) in congenital heart surgery is very different from that used in adult cardiac surgery, which relates to the smaller size and
the minimization
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Figure 58-1 Cartoon illustrates how the cardiac segments (atria, ventricles, great arteries) are analogous to a train and how the segmental approach analyzes the connection and
alignment of these segments. (From Freedom RM: The application of a segmental nomenclature. In Freedom RM, Culham JAG, Moes CAF [eds]: Angiocardiography of Congenital
Heart Disease. New York, Macmillan, 1984, p 18. Reproduced with permission of The McGraw-Hill Companies.)
Figure 58-2 Model of four normal hearts (excluding situs ambiguus) in the convention of Van Praagh. See text for details. A, anterior; L, left; LA, left atrium; LV, left ventricle; P,
posterior; R, right; RA, right atrium; RV, right ventricle. (From Kirklin JW, Barratt-Boyes BG: General considerations: Anatomy, dimensions, and terminology. In Cardiac Surgery,
2nd ed. New York, Churchill Livingstone, 1993.)
of technology and circuitry needed to achieve appropriate flows. Vulnerability of immature, neonatal organ systems to the stresses imposed by the heart
lesion, the insult of nonphysiologic CPB flow, and the inflammatory response of CPB require highly specialized techniques of perfusion and
postoperative management.[3] Differences in neonates, infants, and children do not translate into linear reductions of adult protocols; rather, these are
very specific, individualized needs.
Neonates have different myocardial metabolic properties than those of older children and adults.[4] These differences require alternative myocardial
protection strategies. Cardioplegia infusion pressures are adjusted to patient size and weight. In neonates, immaturity of myocardial calcium
sequestration leads to a dependency on extracellular calcium for calcium-dependent excitation-contraction coupling, as does the exclusive dependency
of neonatal myocardium on glucose for metabolic substrate. Complex reconstruction and small patient size may require deep hypothermic circulatory
arrest (DHCA). The consequences of low-flow CPB and DHCA in infants are now becoming apparent, and neurologic outcomes are of concern in
congenital heart surgery.[5]

CONGENITAL LESIONS
Lesions Resulting in Increased Pulmonary Blood Flow
Increased pulmonary blood flow, particularly at high pressure, decreases lung compliance. Pulmonary congestion can be added when there is increased
resistance to adequate pulmonary venous outflow. The amount of increased pulmonary blood flow will depend on the absolute size of the defect, the
resistances of the pulmonary and systemic vascular beds, and the total pumping capacity of the ventricular mass.
Patent Ductus Arteriosus and Aorticopulmonary Window
Patent Ductus Arteriosus
Patent ductus arteriosus (PDA) is a common isolated defect affecting 1 in 2000 births, with an increased incidence in premature neonates. In complex
lesions, the PDA may be the only source of pulmonary blood supply. This discussion is limited to isolated PDA.
Anatomy and Pathophysiology
The ductus arteriosus is a fetal structure that allows blood to divert away from the lungs and into the descending aorta. The PDA arises from the
junction between the left and the main pulmonary artery and joins the underside of the distal aortic arch beyond the origin of the left subclavian artery.
The recurrent laryngeal nerve is intimately related to the PDA ( Fig. 58–3 ). Right-sided, bilateral PDAs and connections to the subclavian artery have
been described. After birth, closure of the ductus is an important transition. Functional closure occurs first, mediated by the removal of the placental
source of prostaglandin and its metabolism in the lungs. Functional closure is due to muscular contraction and is reversible. Anatomic closure is
irreversible and develops
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Figure 58-3 The anatomic relationships of a patent ductus arteriosus, exposed from a left thoracotomy. The parietal pleura is incised and reflected medially. The course of the
recurrent laryngeal nerve is shown. (From Castaneda AR, Jonas RA, Mayer JE Jr, Hanley FL: Patent ductus arteriosus. In Cardiac Surgery of the Neonate and Infant. Philadelphia,
WB Saunders, 1994.)
over weeks, involving degenerative changes. Spontaneous closure is rare before birth. A ductus that fails to close after 3 months of age is considered
pathologic. Any PDA causing congestive cardiac failure or preventing ventilator weaning is also pathologic. The physiology of a PDA is left-to-right
shunting and increased pulmonary blood flow with left atrial and ventricular volume overload. Complications of a PDA in older patients include
aneurysm formation, infective endocarditis, calcification, and the risk of pulmonary vascular obstructive disease.
Diagnosis and Intervention
The typical “machinery” murmur is heard in older children. In neonates and infants, pulmonary congestion and failure to thrive and, in premature
infants, difficulty in weaning from ventilatory support should prompt echocardiographic examination. In premature infants, surgical closure is
considered after medical failure (three doses of indomethacin). In older infants, closure of the PDA should be considered in the first 6 months of life.
Echocardiography is diagnostic. Cardiac catheterization is reserved for patients in whom irreversible pulmonary hypertension is suspected.
Closure of the PDA
Inhibition of prostaglandin synthesis in premature infants by indomethacin induces ductal closure. Transcutaneous catheter closure of the PDA is
achieved in older children using coils and occluder devices. Patients with large, calcified, and aneurysmal ducts are not suitable for this approach. Small
patients pose vascular access difficulties. Surgery is through a left posterolateral thoracotomy. The recurrent laryngeal nerve is preserved. The duct is
ligated or, in the case of premature infants, either clipped with a metal clip or ligated. Video-assisted thoracoscopic closure of the PDA has been
described.[6] A very large PDA may require division. In calcified, infected, or aneurysmal PDAs, CPB and patch closure from the aortic or pulmonary
artery side are safer. The mortality rate for uncomplicated PDA ligation approaches 0%. [7] Complications relate to duct trauma with bleeding, recurrent
laryngeal nerve injury, pneumothoraces, and chylothorax.
Aorticopulmonary Window
Aorticopulmonary window, a rare defect, is a conotruncal anomaly, producing a window or communication between the aorta and the pulmonary artery.
A defect of the conotruncal ridges results in this communication between the great vessels. The window is variable in size and situation. Three types of
aorticopulmonary window are recognized ( Fig. 58–4 ). Associated lesions include ventricular septal defect (VSD), coarctation of the aorta, and aortic
arch interruption.[8] Physiology of the defect is similar to that of a large PDA with pulmonary overcirculation, pulmonary hypertension, left ventricular
volume overload, and possible diastolic steal from the coronary circulation.
Diagnosis and Indications for Intervention
Patients present in heart failure when the pulmonary vascular resistance (PVR) falls after birth. Pulmonary vascular disease is an early risk because of
the usual nonrestrictive size of the defect. Echocardiography is diagnostic. Cardiac catheterization is not indicated, unless high PVR is suspected.
Intervention is indicated at the time of diagnosis, unless irreversible pulmonary vascular obstructive disease is already established.
Intervention
Surgical intervention usually requires a median sternotomy and CPB. After aortic cross-clamping
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Figure 58-4 The types of aorticopulmonary window. Type A is the simplest. In type B, both the main pulmonary artery and the left pulmonary artery are involved. In type C, the right
pulmonary artery arises separately from the aorta. (From Chang AC, Wells W: Aorticopulmonary window. In Chang AC, Lee FL, Wernovsky G, Wessel DL [eds]: Pediatric Cardiac
Intensive Care. Baltimore, Williams & Wilkins, 1998.)
and cardioplegic arrest, the defect is incised and exposed. Direct suture closure or patch closure with branch pulmonary artery reconstruction is
performed ( Fig. 58–5 ). Postoperative management requires monitoring of the pulmonary artery pressures (PAPs) and alertness to possible pulmonary
hypertensive episodes. The operative mortality rate should approach 0%. Distortion of the repaired pulmonary artery is possible in the long term.[9]
Atrial Septal Defects
Isolated atrial septal defects (ASDs) are the most commonly encountered congenital cardiac anomalies, occurring in 10% to 15% of patients. These are
the most common isolated cardiac defects encountered in the adult population. ASDs are also associated with complex congenital cardiac anomalies.
The atrial septum consists of the septum primum and the septum secundum. These structures merge superiorly and inferiorly with the caval orifices.
Defects are caused by failure of the septum primum to develop or regression of the interatrial folds at the level of the superior or inferior vena cavae.
Developmentally, a patent foramen ovale allows the placentofetal circulation to function. A defect of the septum primum is classified as an ostium
secundum defect. The ostium primum type of ASD is a form of atrioventricular (AV) canal defect. Other ASDs are either the sinus venosus type—with
the defect at the level of the superior vena cava or inferior vena cava—or the coronary sinus type of ASD. Sinus venosus defects occur in association
with partial anomalous pulmonary venous drainage. Coronary sinus ASD is rare and is due to a defect in the wall between the coronary sinus and the
left atrium. The types of ASDs are shown in Figure 58–6 .
Figure 58-5 Repair of a type A aorticopulmonary window, exposed through a longitudinal aortotomy. Closure is by means of a patch. In infants, a patch is usually unnecessary. Types
B and C may require pulmonary artery reconstruction. (From Chang AC, Wells W: Aorticopulmonary window. In Chang AC, Lee FL, Wernovsky G, Wessel DL [eds]: Pediatric
Cardiac Intensive Care. Baltimore, Williams & Wilkins, 1998.)
Figure 58-6 Types of atrial septal defects (ASDs). The anatomy of various ASDs is shown from the right atrial perspective. A, Ostium secundum defect. B, Superior vena caval, sinus
venosus type of ASD. C, Ostium primum with partial atrial ventricular canal defect. D, The site of the coronary sinus in the right atrium. (From Chang AC, Jacobs J: Atrial septal
defect. In Chang AC, Lee FL, Wernovsky G, Wessel DL [eds]: Pediatric Cardiac Intensive Care. Baltimore, Williams & Wilkins, 1998.)
The direction and amount of shunting depends on the size of the defect as well as the relative diastolic compliance of the ventricles. The shunt is left to
right, resulting in increased flow to the right side of the heart and increased pulmonary blood flow. Congestive heart failure usually occurs after the
second or third decade of life. Pulmonary hypertension is rare in children, but it can occur. The risks of ASD in older patients include paradoxical
embolism and stroke, atrial fibrillation and flutter, sinus node dysfunction, as well as pulmonary vascular
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obstructive disease. Bacterial endocarditis is very rare.[10]

Younger patients are asymptomatic, and the defect is found on routine physical examination. Older patients tend to be symptomatic with subtle signs of
heart failure, exercise intolerance, palpitations, and arrhythmias. Complications such as cryptogenic stroke or pulmonary hypertension may be the
presenting feature. Transthoracic echocardiography is usually diagnostic. Cardiac catheterization is needed to assess pulmonary pressure and PVR in
patients with suspected significant pulmonary hypertension and to exclude coronary artery disease in older patients.
Surgery
Indications for closure of small defects remain controversial. Spontaneous closure of a small patent foramen ovale occurs in up to 80% of infants within
the first year. Closure is indicated in all symptomatic patients and all children with a significant ASD. Adults with a left-to-right shunt greater than 1.5:1
are candidates for closure, provided comorbid conditions do not add excessive risk to the procedure. Severe pulmonary vascular obstructive disease
(resistance greater than 8.0 Wood units/m2 )[11] is a contraindication to closure.
An ASD may be closed surgically or, if appropriate, by percutaneous transcatheter device closure. Surgery requires CPB. A median sternotomy is used,
although a bilateral submammary incision or right anterolateral thoracotomy have all been used. Port access and limited (mini) median sternotomies
have gained popularity. Most surgeons utilize aortic cross-clamping and cardioplegia to operate on a motionless heart, whereas others prefer to fibrillate
the heart. The defect is closed by direct suture or pericardial or other prosthetic patch. For sinus venosus defects, techniques that route the anomalous
veins to the left atrium are used. The surgical risk for death approaches 0% in isolated ASDs. Postoperative complications include pericardial effusions,
postpericardiotomy syndrome, postoperative dysrhythmias, and residual ASDs.
Ventricular Septal Defects
Congenital defects of the interventricular septum may be single, multiple, or part of more complex cardiac anomalies. Congenital VSDs occur in 1 to 2
per 1000 live births, and of those requiring surgical repair, 50% will have another cardiac anomaly.
VSDs are classified by the position they occupy in the ventricular septum. This classification is important because, by defining the position of the
defect, the path of the conducting system can be reliably predicted and avoided during surgery.[12] In addition, the probability of spontaneous closure or
of the predisposition to secondary cardiac pathology can be factored into the management decision making. The ventricular septum is described from
the morphologic right side. The septum is divided into four parts: the membranous septum, the inlet, the trabecular, and the outlet parts of the muscular
septum (the outlet septum is also called the conal or infundibular septum).
Perimembranous or Paramembranous Defects
Perimembranous or paramembranous defects occur around the membranous septum and the fibrous trigone of the heart. The defect is near the aortic
valve, and the annulus of the tricuspid valve contributes to the rim of the defect. The defect may extend into any of the other components of the septum.
The conduction tissue passes along the posteroinferior rim of the defect.
Muscular Defects
Muscular defects have muscular rims. They may be single, but they are commonly multiple. Most commonly, multiple defects occur in the apical
trabecular septum. The term Swiss cheese septum is used for associated spongiform myocardium and not for multiple muscular defects alone.[13]
Prediction of the conducting system depends on whether the defect extends to the membranous septum.
Subarterial, Outlet, or Conal Defects
Subarterial, outlet, or conal defects are located in the outlet portions of the left and right ventricles. The superior edge of the VSD is the conjoined
annulus of the aortic and pulmonary valves. These are also called juxta-arterial or supracristal defects. This VSD is associated with prolapse of the
unsupported aortic valve cusps and progressive aortic regurgitation.
Malalignment Defects
Malalignment defects are created by malalignment between the infundibular and the trabecular muscular septum. This malalignment can be anterior, as
in tetralogy of Fallot (TOF), or posterior. Associated defects occur frequently and include PDA, pulmonary stenosis, ASD, persistent left superior vena
cava, and coarctation of the aorta.
The hemodynamic effect of a VSD is left-to-right shunting leading to increased pulmonary blood flow, left atrial dilation, and left ventricular volume
overload. The size of the shunt is determined by the size of the defect (restrictive is smaller than the aortic root diameter) and the PVR. Compared with
an ASD, the shunting in a VSD occurs mainly during systole. It is useful to quantify the shunt by the ratio of systemic to pulmonary blood flow
(Qp:Qs). At cardiac catheterization, the Qp:Qs can be estimated from the equation:
The severity of pulmonary vascular disease correlates with the size of the shunt. In time, as the PVR increases, histologic changes occur within the
pulmonary vascular bed, which may be irreversible. The time of onset and the severity of pulmonary vascular disease correlate with the size of the shunt
but are also subject to considerable individual variation. As the PVR increases, the left-to-right shunt decreases, causing unloading of the left ventricle.
Congestive heart failure improves, and the patient feels better! If untreated, a reversal of the flow occurs, leading to a right-to-left shunt with the
development of increasing cyanosis (Eisenmenger’s syndrome).
The clinical presentation depends on the size of the shunt and the PVR. The clinical picture varies from an asymptomatic patient with a murmur, to a
patient in fulminant heart failure, to a cyanosed patient with irreversible pulmonary vascular obstructive disease. Associated abnormalities determine the
findings, especially if aortic regurgitation is present.
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The echocardiogram is diagnostic, and the defect, as well as associated cardiac abnormalities, can be assessed. The echocardiogram can also provide an
estimation of RV pressure (and PAP in the absence of pulmonary stenosis) by obtaining the Doppler velocity (V) of the jet through the VSD and/or the
regurgitant jet through the tricuspid valve, using the modified Bernoulli equation:
Cardiac catheterization is indicated when reversibility of the PAP is questionable. The Qp:Qs can be documented, and a dynamic assessment is possible,
obtaining the PVR before and after pulmonary vasodilation. A PVR of more than 8.0 Wood units/m2 with vasodilation is inoperable.
Management
The ideal is to intervene when the likelihood of spontaneous VSD closure is lowest and the risk of irreversible pulmonary vascular disease and
ventricular dysfunction are minimized. Perimembranous and muscular defects tend to close with time. Eighty percent of VSDs seen at 1 month of age
will close spontaneously. Spontaneous closure of malalignment and subarterial defects is unlikely. Bacterial endocarditis is more common with small
and moderate-sized VSDs with an incidence of 0.15% to 3% per year. In subarterial VSDs, the risk of irreversible aortic valve damage owing to cusp
prolapse leads to earlier intervention.[14] Single-stage closure is recommended early, when the defect is large and symptoms and signs of congestive heart
failure and failure to thrive are found.[15] With perimembranous and muscular defects, if the infant is thriving and it is known that the PAP is near
normal, surgery may be delayed reasonably up to 1 year or more. Other defects should be closed. The younger patient with a small defect may be
followed. The ideal management of a small defect (Qp:Qs < 1.5:1; normal PAP) in a patient older than 10 years of age is controversial. Multiple VSDs
present a different problem: if a large shunt is present and persists beyond 6 to 8 weeks, pulmonary artery banding and removal after 2 years of age with
an attempt at septation is reasonable. Banding is also reasonable in VSDs complicated by straddling or overriding of the AV valves. In VSDs associated
with coarctation, aortic arch hypoplasia, or interruption, single-stage repair of both defects through the midline is recommended, provided this can be
achieved with low risk.
Surgery
VSDs are closed using CPB with bicaval cannulation. Circulatory arrest may be required for simultaneous arch reconstruction. Most VSDs can be
repaired through a right atrial approach, except for subarterial defects, which are approached through the pulmonary valve, and multiple apical
trabecular defects, which are sometimes easier to approach through a small apical right ventriculotomy.[13] Prosthetic patch closure using Dacron, Teflon,
or Gore-Tex is recommended ( Fig. 58–7 ). Transcatheter device closure and intraoperative device placement have been used in unusual circumstances
to achieve VSD closure. Postoperatively, monitoring the left atrial and PAP simplifies management in those with large defects, preexisting heart failure,
and known pulmonary hypertension. Precautions are taken to limit the responsiveness of the pulmonary vascular bed, and ventilatory
Figure 58-7 The location of various ventricular septal defects (VSDs) in the ventricular septum. (This is a view of the ventricular septum from the right side.) 1, Perimembranous
VSD. 2, Subarterial VSD. 3, Atrioventricular canal-type VSD. 4, Muscular VSD. (From Tchervenkov CI, Shum-Tim D: Ventricular septal defect. In Baue AE, Geha AS, Hammond GL
[eds]: Glenn’s Thoracic and Cardiovascular Surgery, 6th ed. Stamford, CT, Appleton & Lange, 1996. Reproduced with permission of The McGraw-Hill Companies.)
management becomes an important tool. With persistent, severe pulmonary hypertension, nitric oxide is available.[16]
For uncomplicated VSD repair, the operative mortality rate should approach 0%. The overall risk for VSD repair is less than 5%. Mortality and
morbidity increase with multiple VSDs, pulmonary hypertension, and complex associated anomalies. Postoperative problems are residual VSDs that
may require reoperation if hemodynamically significant. Heart block is infrequent and approaches zero in many centers.
Atrioventricular Canal Defects
AV canal defects are also known as endocardial cushion defects or AV septal defects. There is a high incidence of Down’s syndrome with endocardial
cushion defects. A spectrum of anomalies occurs depending on the presence of atrial and ventricular defects. AV canal defects are either partial (PAVC)
or complete (CAVC). Intermediate types occur. Additionally, hypoplasia of either the left or the right ventricular chamber can lead to an unbalanced
AV canal, which may preclude biventricular repair. Associated anomalies include heterotaxy syndromes, TOF, double-outlet right ventricle (DORV),
and total anomalous venous return.
The actual embryologic origin of this defect remains unclear. Three principal components are found in CAVC: a defect of the AV septum, a defect of
the interventricular septum, and an abnormal AV valve.
An ostium primum defect or PAVC consists of an ASD associated with abnormal AV valve anatomy, a cleft leaflet of the left-sided and right-sided AV
valves. Left-sided AV valve regurgitation is not uncommon. Two separate AV valve orifices are present ( Fig. 58–8 ). There is in fact also
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Figure 58-8 The position of the conducting system in complete atrioventricular canal defect (CAVC). The anatomic relationships and morphology of the common atrioventricular
(AV) valve are shown. The view is through a right atriotomy. Ao, aorta; BB, left bundle branch; CS, coronary sinus; LIL, left inferior leaflet; LLL, left lateral leaflet; LSL, left
superior leaflet; PA, pulmonary artery; PB, penetrating bundle; RBB, right bundle branch; RIL, right inferior leaflet; RLL, right lateral leaflet; RSL, right superior leaflet. (From
Bharati S, Lev M, Kirklin JW: Cardiac Surgery and the Conduction System. New York, Churchill Livingstone, 1983.)
a deficiency of the interventricular septum similar to that of CAVC, but in the PAVC the tissues of both AV valves are continuously adherent to the
septal crest. A transitional or intermediate AV canal defect is an ostium primum defect, with the AV valve only partially adherent to the septal crest.
Classification of CAVC into Rastelli types A, B, and C relates to the superior AV valve leaflet chordal attachments to the ventricular septum. In type A,
the superior leaflet chords are attached to the septum; in type B, the superior leaflet is attached to an abnormal papillary muscle in the right ventricle;
and in type C, the superior leaflet is free floating ( Fig. 58–9 ). Pathophysiology depends on whether all three components are present. In PAVC, the
pathophysiology is that of an ASD, with or without left-sided AV valve regurgitation. In CAVC, the pathophysiology is that of a VSD with an
associated ASD. This results in a large left-to-right shunt at two levels, equalization of right ventricular and left ventricular pressures, and volume
overload of all cardiac chambers. With additional AV valve regurgitation, there is further volume overload.
Diagnosis
These children usually present in congestive heart failure. In PAVC, this is uncommon before 6 months of age but is quite common in CAVC by the age
of 2 months. Of concern is the development of irreversible pulmonary vascular obstructive disease, which may occur before 1 year of age. Down’s
syndrome children with chronic upper airway obstruction have a predilection for pulmonary vascular obstructive disease. Echocardiography is
diagnostic with demonstration of the typical cleft in the anterior leaflet of the AV valve. Cardiac catheterization
Figure 58-9 The Rastelli classification type A, B, or C. A to C, The difference in valve morphology in a normal, partial canal and complete canal defect is illustrated. AL, anterior
leaflet; A-V, atrioventricular; MV, mitral valve; PL, posterior leaflet; RIL, right interior leaflet; RLL, right lateral leaflet; RSL, right superior leaflet; TV, tricuspid valve. (From
Kirklin JW, Pacifico AD, Kirklin JK: The surgical treatment of atrioventricular canal defects. In Arciniegas E [ed]: Pediatric Cardiac Surgery. Chicago, Year Book Medical, 1985.)
is indicated in patients older than 3 to 4 months of age in whom elevated PVR is suspected. On left ventriculography, the “goose-necked deformity” of
the elongated left ventricular outflow tract is seen.
Surgery
For PAVC, surgery has been recommended at preschool age. This may be performed earlier, usually after the age of 8 to 12 months. For CAVC, the
ideal age for surgery relates to the risk for the development of pulmonary vascular obstructive disease. Elective repair in patients by 3 months of age is a
reasonable compromise between heart size and risk of irreversible complications. A median sternotomy and CPB are used. The common denominator in
all forms of endocardial cushion defect is that the fibrous center of the heart is deficient and the conduction system is thus found in an abnormal
position. Careful suture placement for the ASD and VSD patch is essential to avoid heart block ( Fig. 58–10 ). For PAVC, the ASD is closed with an
autologous pericardial patch, leaving the coronary sinus ostium in the right atrium. In CAVC, a one- or two-patch technique is used. The two-patch
technique may be advantageous in a small heart, by minimizing loss of leaflet tissue in the suture line.[17] The VSD portion of the patch is completed
first, with care taken to avoid obstructing the left ventricular outflow tract. The valve leaflets are then attached to the patch, and the ASD is closed with
the pericardial patch (see Fig. 58–10 ).
For PAVC, the mortality approaches zero. CAVC mortality rates in the last 5 to 10 years have been reported between 0 and 10%.[18] Other surgical
procedures include
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Figure 58-10 The two-patch closure of CAVC. A ventricular septal patch is placed first (A), and a separate patch is used to close the ASD component (B). Note the position of the
coronary sinus and conducting system relative to the ASD patch suture line, to avoid injury to the AV node. (From Kirklin JW, Barratt-Boyes BG: Cardiac Surgery. New York,
Churchill Livingstone, 1986.)
Figure 58-11 Classification of truncus arteriosus according to Van Praagh. Type A, with a VSD; Type B, without a VSD. A1, Partially separate main pulmonary artery. A2, Absent
aorticopulmonary septum, both branch pulmonary arteries arise from the common trunk. A3, Absence of either branch pulmonary artery. A4, Hypoplasia, coarctation, atresia, or
absence of the aortic isthmus in association with a large patent ductus arteriosus. (From Hernanz-Schulman M, Fellows KE: Persistent truncus arteriosus: Pathologic, diagnostic and
therapeutic considerations. Semin Roentgenol 20:121–129, 1985.)
pulmonary artery banding, which has a role in patients with respiratory compromise from viral illness, extremely small babies, and patients who have an
unbalanced AV canal with excessive pulmonary blood flow in whom a biventricular repair may or may not be possible.
Truncus Arteriosus
A single arterial trunk arises from both ventricles, from which the coronary and pulmonary arteries originate. It is usually associated with a conotruncal
VSD. There is an association with microdeletion of chromosome 22q11 and the DiGeorge syndrome.
The classification of Van Praagh is useful from a surgical perspective ( Fig. 58–11 ). Failure of the embryologic truncus arteriosus to septate into the
aorta and the pulmonary artery gives rise to the characteristic single arterial trunk from both ventricles. Associated lesions include aortic arch
obstruction, right aortic arch, interrupted aortic arch, and ASDs. The single truncal valve is often dysmorphic and can be either stenotic or regurgitant or
both. Variability of the leaflets is common, and the number of leaflets can vary from two to six, with truncal valve incompetence more common with
four or more leaflets.[19] Associated coronary artery abnormalities may be present. Pathophysiology relates to a pressure and volume overload to both
right and left ventricles, with pulmonary overflow dependent on the PVR. The effect of volume and pressure overload is worsened by truncal valve
stenosis or regurgitation. The fall in PVR after birth causes significant pulmonary overflow and congestive heart failure. Heart failure is more severe
with truncal valve regurgitation. These patients are at risk of early development of pulmonary vascular obstructive disease and of subendocardial
ischemia from coronary diastolic steal.
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Diagnosis and Presentation
Echocardiography is diagnostic. In addition to the obvious conotruncal defect, attention should be focused on the number of VSDs, the anatomy of the
truncal valve, and coronary artery anomalies. These patients should be assumed as having a component of DiGeorge’s syndrome, and only irradiated
blood products are used. Cardiac catheterization is indicated in older infants when pulmonary vascular disease is suspected. Presentation is usually in
the neonatal period.
Surgery
Complete repair is recommended in the neonatal period for severe heart failure, but this can be safely delayed up to 3 months in patients with easily
controlled heart failure. Again, this is a compromise between attaining increased heart size and the development of irreversible complications. The only
absolute contraindication for surgery is the presence of Eisenmenger physiology. Palliative pulmonary artery banding is difficult and is high risk.[20]
Median sternotomy with CPB and DHCA is limited to the period of arch repair if required. The pulmonary arteries are detached from the truncal root,
and the defect is closed. Through a right ventriculotomy, the VSD is closed and a conduit is placed from the right ventricle to the transected pulmonary
arteries ( Fig. 58–12 ). Mortality in truncus arteriosus depends on the associated
Figure 58-12 Surgical repair of truncus arteriosus. A, Origin of truncus arteriosus is excised and the truncal defect closed with direct suture. The incision is made high in the right
ventricle (RV). LPA, left pulmonary artery; RPA, right pulmonary artery. B, Ventricular septal defect (VSD) is closed with a prosthetic patch. C, Placement of a valved conduit into
the pulmonary arteries. D, Proximal end of conduit is anastomosed to the RV. (From Wallace RB: Truncus arteriosus. In Sabiston DC Jr, Spencer FC [eds]: Gibbons Surgery of the
Chest, 3rd ed. Philadelphia, WB Saunders, 1976.)
conditions. The most important factor for nonsurvival is severe incompetence of the truncal valve. Uncomplicated truncus mortality should be less than
5% and perhaps higher in patients with arch obstruction, severe truncal valve stenosis or regurgitation, and coronary artery abnormalities. Low birth
weight is an independent predictor of nonsurvival. Conduit obstruction requiring replacement or revision is usual.[21]
Abnormalities of Venous Return: Systemic and Pulmonary
Abnormal Systemic Venous Return
Abnormal systemic venous return is a frequent finding in complex congenital disease and in the normal population. A persistent left superior vena cava
draining to the coronary sinus may be associated with hypoplasia or atresia of the mitral valve but is harmless in isolation. Absence of an innominate
vein is a clue to this anomaly. More complex variations are found in the heterotaxy (isomeric) syndromes with interrupted inferior vena cava and azygos
or hemiazygos continuations. Implications of these abnormalities are that they complicate cannulation for CPB and may preclude septation of the heart.
Anomalous Pulmonary Venous Return
Anomalous pulmonary venous return may be either partial or complete.

Partial
The most common anomalies are right upper pulmonary veins draining to the superior vena cava (associated with a superior sinus venosus ASD); the
“scimitar” syndrome with partial or complete drainage of the right-sided pulmonary veins to the inferior vena cava; and isolated left upper pulmonary
veins draining to the left innominate vein via a vertical vein.
Diagnosis depends on the magnitude of the associated shunt, the degree of systemic desaturation, and the presence or absence of pulmonary vein
obstruction. Echocardiography is often diagnostic, but catheter study may be required.
Surgery
Redirection of the pulmonary venous return with closure of the ASD or reconnecting the pulmonary veins to the left atrium and division of the systemic
venous connection are performed. Surgery is low risk, and late complications are stenosis of the reconnected pulmonary vein or baffle obstruction.
Cor Triatriatum
Cor triatriatum is a rare anomaly that has a diaphragm or membrane separating either the right or left atrium into two chambers. On the left side the
superior chamber connects all four pulmonary veins and the inferior chamber contains the orifice of the left atrial appendage and the orifice of the mitral
valve. The pathophysiology is similar to mitral stenosis and is affected by the size of the communication between the chambers and the size and position
of the ASD if present. Operative correction involves excision of the obstructing membrane through the fossa ovalis or existing ASD, followed by
closure of the ASD. On the right side the membrane represents a filling in of the Chiari network presumably derived from the venous valves and mimics
tricuspid valve
1822
Figure 58-13 Types of total anomalous pulmonary venous connection (TAPVC). A, Supracardiac type with a vertical vein joining the left innominate vein. CS, coronary sinus; LA,
left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle. B, Intracardiac type with connection to the coronary sinus. C, Infracardiac type with drainage through the
diaphragm via an inferior connecting vein. (From Hammon JW Jr, Bender HW Jr: Anomalous venous connections: Pulmonary and systemic. In Baue AE [ed]: Glenn’s Thoracic and
Cardiovascular Surgery, 5th ed. Norwalk, CT, Appleton & Lange, 1991. Reproduced with permission of The McGraw-Hill Companies.)
stenosis. Operative mortality approaches zero with good long-term results.[22]

Total Anomalous Pulmonary Venous Connection
Total anomalous pulmonary venous connection (TAPVC) results in abnormal drainage of all the pulmonary veins directly or indirectly to the systemic
venous atrium. Thirty percent of patients will have associated cardiac defects. Classification of TAPVC is based on the site of the connection to the
systemic venous system and may be supracardiac, cardiac, infracardiac, or mixed ( Fig. 58–13 ). Supracardiac is the most common (approximately 50%)
and mixed the rarest.
Pathophysiology
All the pulmonary venous return is to the right atrium, and the physiology depends on whether the veins or the ASD is obstructed. With obstructed
TAPVC, the obstructed pulmonary venous return causes pulmonary congestion or edema and pulmonary hypertension. The obstruction is due to a
number of mechanisms. With the supracardiac type, the vertical vein may be compressed between the pulmonary artery and the left bronchus (vascular
vice), or anatomic narrowing of the pulmonary venous confluence and stenosis of the pulmonary veins themselves may occur. In the absence of a VSD,
left ventricular filling depends on the size of the ASD. A restrictive ASD adds the pathophysiology of low systemic arterial output.
Obstructed TAPVC requires immediate surgical intervention. These neonates present with cyanosis, various levels of pulmonary venous congestion,
respiratory compromise, and acidosis. If the ASD is obstructive, systemic cardiac output is also low. In unobstructed TAPVC with an unobstructed
ASD, the clinical presentation is usually similar to that of a large ASD with some degree of cyanosis. Pulmonary hypertension can occur as a late
consequence. On echocardiography, each of the four pulmonary veins is identified. Evidence for obstruction is obtained through velocity flow mapping
of the individual pulmonary veins and their coalescing path to the right atrium. Pulmonary hypertension is always present when pulmonary venous
return is obstructed. Cardiac catheterization is not usually indicated, unless uncertainty exists over the site of pulmonary venous drainage.
Surgery
In obstructed TAPVC, surgery is an emergency. Without surgery, mortality is 100% in the first year of life. Because of severe pulmonary venous
obstruction, mechanical ventilation is necessary and should be instituted quickly. Prostaglandin E1 (PGE1 ) use is controversial. An open PDA may
worsen systemic cyanosis, but it may improve systemic cardiac output if the ASD is restrictive.
For supracardiac TAPVC, through a median sternotomy and CPB with or without DHCA, an anastomosis is fashioned between the retropericardial
pulmonary venous confluence and the left atrium. This may be performed from the posterior aspect working through the oblique pericardial sinus or
utilizing the techniques of Schumacher and Tucker ( Fig. 58–14A ).[23] The ASD is closed as well.
For the intracardiac type repair requires the huge coronary sinus to be unroofed into the left atrium. The ASD and coronary sinus ostium are closed
primarily or patched separately.
For the infracardiac type the pulmonary venous confluence and left atrium are anastomosed in a side-to-side fashion (see Fig. 58–14B ). The descending
vertical vein is
1823
Figure 58-14 A, Repair of supracardiac TAPVC through a superior approach. B, Repair of infracardiac TAPVC. Elevating the apex of the heart to the right side exposes the left
atrium and pulmonary confluence. Anastomosis is created as shown. (From Lupinetti FM, Kulik TJ, Beekman RH, et al: Correction of total anomalous pulmonary venous connection
in infancy. J Thorac Cardiovasc Surg 106:880, 1993.)
divided. The current overall mortality is quite low for obstructed TAPVC, around 5%. This correlates directly with preoperative morbidity.
Postoperative pulmonary venous obstruction occurs in 5% to 10% of patients. This is associated with a poor outcome if it involves the individual veins.
[24]
Lesions Resulting in Decreased Pulmonary Blood Flow
These lesions reduce pulmonary blood flow by obstruction at, below, or above the pulmonary valve. The obstruction may be at a single level (e.g.,
pulmonary valve stenosis) or may be a more complex, multilevel obstruction, such as TOF.
Tetralogy of Fallot
TOF is a conotruncal defect resulting from anterior malalignment of the infundibular septum. This single morphologic defect gives rise to the four
components of TOF: the VSD, aortic valve override, and narrowing of the right ventricular outflow tract resulting in secondary right ventricular
hypertrophy ( Fig. 58–15 ). Complex forms of TOF include TOF with pulmonary atresia with or without major aortopulmonary collateral arteries,
absent pulmonary valve, and CAVC defects.[25]
The right ventricular outflow tract obstruction (RVOTO) may be at subpulmonary level, pulmonary valve level, main pulmonary artery level, or
pulmonary artery bifurcation level or may involve branch pulmonary arteries. In some cases, obstruction is present at all levels. A right-sided aortic arch
occurs in 25% (associated with chromosome 22q11 microdeletion); particularly in the presence of anomalous origin of the left subclavian artery.
Preoperative physiology depends on the degree of RVOTO. Patients with minimal obstruction present with a left-to-right shunt owing to the VSD.
These
Figure 58-15 Anatomy of tetralogy of Fallot. A cutaway of the right ventricular free wall reveals the four components: ventricular septal defect, aortic override, hypertrophied right
ventricular muscle, and infundibular stenosis, with small hypoplastic pulmonary annulus. (From Spray TL, Wernovsky G: Right ventricular outflow tract obstruction. In Chang AC,
Lee FL, Wernovsky G, Wessel DL [eds]: Pediatric Cardiac Intensive Care. Baltimore, Williams & Wilkins, 1998.)
patients have pulmonary overcirculation; this is called acyanotic TOF. These patients present with congestive heart failure. At the other end of the
spectrum, severe obstruction to pulmonary blood flow causes profound cyanosis.
Indications for Intervention
The nature of the RVOTO dictates management. In cyanotic TOF, hypercyanotic episodes may occur with agitation or irritability. If profound, blood
pressure falls with an altered level of consciousness; this is a classic TOF spell. A single spell is an indication for surgery. Ideally, referral should
precede the spell. Conservative management is knee-to-chest positioning, administration of supplemental oxygen, sedation, volume expansion, and
other measures that increase cardiac preload and systemic resistance. β Blockade is helpful. The chest radiograph may be classic in terms of a “boot-
shaped” heart. Echocardiography is diagnostic, and associated anomalies can be excluded. Cardiac catheterization is indicated before repair of TOF with
previous palliation and under those circumstances in which the presence of aortic pulmonary collaterals and pulmonary artery branching abnormalities
are suspected. Timing is controversial regarding management of asymptomatic TOF. In asymptomatic patients, elective repair has been advocated from
the neonatal period up until 1 year of age. In symptomatic or cyanotic patients, depending on institutional preferences, complete repair can be performed
as a single-stage procedure or as a two-stage approach, with initial systemic-to-pulmonary artery shunting.[26] [27]
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Surgical Intervention
Palliation
A systemic-to-pulmonary artery shunt is indicated in those patients in whom the risk of complete repair is considered to be higher than the cumulative
risk of two-stage repair for a given institution. Creation of a systemic-to-pulmonary artery shunt can be carried out either from the midline or from a
thoracotomy. Methods include a classic Blalock-Taussig shunt or a modified Blalock-Taussig shunt utilizing an interposition Gore-Tex tube graft.
Creation of a shunt on the left side is more difficult to take down at the time of complete repair. Most institutions will currently perform a right-sided
shunt through a right thoracotomy or from the midline, creating a central-to-right pulmonary artery shunt.[27]
Complete Repair
Complete repair involves closure of the VSD, preservation of the conducting system, and relief of the RVOTO. A median sternotomy is utilized with
CPB. Two approaches are used. The transventricular repair with a right ventriculotomy in the infundibulum allows exposure of the VSD and a patch
closure of the infundibular incision. Alternatively, the VSD and subpulmonary obstruction can be approached from a transatrial direction.[28] Muscle
resection is carried out to relieve the RVOTO. Assessment of the pulmonary annulus, utilizing predicted mean-normal diameters of the pulmonary valve
annulus corrected for body surface area, provide some guidance for enlarging the pulmonary annulus (transannular patching) ( Fig. 58–16 ).[29] The
transventricular repair is less popular today with increasing use of the transatrial-transpulmonary repair. In severe multilevel obstruction and hypoplasia,
a conduit connection of the right ventricle to the pulmonary arteries is preferred in some centers. Distal pulmonary arteries and branch pulmonary artery
stenosis are dealt with at the time of surgery, utilizing autologous pericardial patch enlargement.
Today, the mortality risk for uncomplicated TOF repair should approach 0%. Specific postoperative problems are a residual or previously undiagnosed
VSD. Residual RVOTO may be progressive and may require repeat resection. Acute right ventricular dysfunction is quite frequent after TOF repair
utilizing a transventricular approach but usually recovers. Residual VSDs, residual RVOTO, and long-standing pulmonary and tricuspid valve
regurgitation all contribute to right ventricular dysfunction. The risk of reoperation for various reasons within 2 to 5 years is 3% to 5%. Other forms of
complex TOF (e.g., absent pulmonary valve and CAVC) are managed as follows:
TOF with absent pulmonary valve is usually associated with massive enlargement of the main and branch pulmonary arteries. There is a characteristic
“to-and-fro” murmur. The airway is abnormal, with areas of tracheomalacia or malformed cartilage. Three basic symptom groups are identified. The
first group is neonates in extremis with immediate postnatal respiratory distress. The second group comprises older infants who develop significant
airway compromise more remotely from birth. The third group experiences no significant airway problems. The neonatal group presents early with
marked respiratory distress, cyanosis, and air trapping owing to tracheobronchial compression. The hypoxemia is largely
Figure 58-16 Complete repair of tetralogy of Fallot. A, Enlargement of the right ventricle to main pulmonary artery (PA) connection with a transannular incision if necessary. Ao,
aorta. B, Resection of muscle from the outflow tract and identification of edges of the ventricular septal defect (VSD). MPA, main pulmonary artery. C, Patch closure of the VSD. D,
Placement of a transannular patch if required.
due to impaired ventilation because it is unusual to have severe RVOTO. Early repair of this subgroup is necessary, with anterior and posterior
reduction of the pulmonary arteries and complete TOF repair, but results may be disappointing because of the intrinsic small and large airway disease.[30]
Older infants are repaired when airway obstruction becomes evident and usually do well. The third group is electively repaired by 1 year of age.
The combination of TOF with CAVC has resulted in a very widespread incidence of reported surgical and postoperative mortality. The management of
the RVOTO is as been described for TOF, but it is important to avoid narrowing of the left ventricular outflow tract at the time of patch placement for
the VSD. Right-sided AV valve regurgitation is of more concern when pulmonary valve incompetence is created by a transannular patch.[31]
Another subgroup that should be included briefly is the group of “extreme tetralogy” or TOF with pulmonary atresia and duct-dependent pulmonary
blood flow. Neonatal surgical intervention is required, either shunt or complete repair. A further subgroup of TOF with pulmonary atresia has
hypoplastic or absent central pulmonary arteries and multiple aortopulmonary collateral arteries
1825
(MAPCAs). In this variant, the intracardiac anatomy is that of TOF. However, the heterogeneity of the pulmonary artery anatomy complicates the
condition. Essentially three patterns of pulmonary supply are recognized:
Absent central pulmonary arteries with MAPCAs; small central pulmonary arteries and MAPCAs; and diminutive central pulmonary arteries usually
with significant arborization defects and MAPCAs. The approach is to provide a stable source of pulmonary blood flow and attempt to recruit the
MAPCAs into the central pulmonary artery system (unifocalization). Complete or biventricular repair is achieved when enough unobstructed segments
have been unifocalized to allow VSD closure and placement of a conduit between the right ventricle and the reconstructed pulmonary arteries, with a
postrepair right ventricular pressure less than 70 mm Hg. The optimal choice of surgery is controversial, with many groups still preferring a staged
approach,[32] whereas others prefer a single-stage, midline complete unifocalization and an attempt at VSD closure.[33]
Pulmonary Atresia and Intact Ventricular Septum
Pulmonary atresia and intact ventricular septum (PAIVS) is a heterogeneous group of lesions. The spectrum ranges from patients with small right
ventricles and small tricuspid valves with associated coronary artery abnormalities placing them at high risk for survival. At the other end of the
spectrum, patients have nearly normal-sized right ventricles with a well-developed infundibulum and absent coronary anomalies. These patients have a
favorable prognosis.
Associated with atresia of the pulmonary valve is an intact ventricular septum and variable hypoplasia of the right ventricle and tricuspid valve. The
main pulmonary artery is present, somewhat smaller than normal, and pulmonary blood flow is supplied by a PDA. The tricuspid valve is variably
hypoplastic and usually morphologically abnormal. Right ventricular cavity-to-coronary artery connections are almost always seen in patients with a
particularly small right ventricle and tricuspid valve and a tiny or absent infundibulum. The physiology is similar to that of other forms of functional
single ventricle with a duct-dependent pulmonary circuit. Depending on the degree of sinusoid and fistula formation, part or all of the coronary circuit
may exhibit so-called right ventricular dependency.
Diagnosis can be made on echocardiography. Ductal patency can be confirmed and the degree of right ventricular hypertension estimated by the
gradient across the regurgitant tricuspid valve. Abnormal flow patterns into the right ventricle are suggestive of coronary artery fistula. The presence
and size of the infundibulum, inflow, and the trabeculated portion of the right ventricle are determined. Cardiac catheterization is indicated for an
assessment of coronary artery anatomy when sinusoids and fistula formation are suspected,[34] or this investigation may be deferred until before planned
CPB operations. PAIVS associated with Ebstein’s anomaly and a large right ventricle constitutes a separate subgroup warranting a separate therapeutic
approach.
Management
This condition necessitates a staged approach toward either a univentricular or a biventricular repair. Decision making is based initially on the size of
the infundibulum.[35] In patients with an absent infundibulum and significant coronary-to-right ventricle fistulas in whom biventricular repair is unlikely,
the first stage would be a palliative modified Blalock-Taussig shunt with or without ligation of the PDA in the neonatal period. If the PDA remains open
without PGE1 , the PDA should be ligated at the time of shunting. With favorable anatomy, relief of the RVOTO by means of a surgical valvotomy, a
systemicto-pulmonary artery shunt (left subclavian to main pulmonary artery), and PDA ligation through a left thoracotomy is an option. Alternatively,
if the right ventricle is less hypoplastic, the pulmonary valve may be opened in the catheterization laboratory and the duct kept open with PGE1 for 2 to
4 weeks. If saturations are inadequate with trial duct closure, a shunt is added surgically.
Depending on the response to the initial procedures, either a Fontan-type track is chosen or a biventricular repair is aimed for. If the RVOTO can be
adequately relieved and the tricuspid valve and right ventricle grow to adequate size, the shunt can be taken down surgically and the ASD closed. About
80% of patients with a well-formed infundibulum will eventually achieve biventricular repair. The remainder may benefit from 1½ ventricle repair (ASD
closure and a superior vena cava-to-right pulmonary artery shunt [bidirectional Glenn]).[36] The Fontan operation is indicated in those cases with severe
right ventricular hypoplasia and a right ventricle-dependent coronary circuit. Surgical mortality depends on the anatomic variant of PAIVS; some of
these patients will require subsequent cardiac transplantation.[37]
Pulmonary Valve Stenosis
Isolated pulmonary valve stenosis usually occurs with a nearly normal-sized, hypertrophied right ventricle and a normal tricuspid valve, but some have
significant right ventricular hypoplasia. The distal pulmonary artery anatomy is also usually normal. The morphology of the pulmonary valve is variable
and can be morphologically abnormal with a unicuspid, bicuspid, or tricuspid valve with commissural fusion. In these patients, a biventricular outcome
can be expected. Balloon valvuloplasty has become the initial procedure of choice. In some patients with severely dysplastic pulmonary valves, surgical
valvotomy may be required. If properly managed, these patients have a low mortality
Other Abnormalities of the Conotruncus
Transposition of the great arteries (TGA) accounts for 5% to 7% of all congenital cardiac malformations. Congenitally corrected TGA is a condition in
which, in addition to ventricular arterial discordance, AV discordance is present.[38]
Transposition of the Great Arteries
TGA is defined as an aorta arising from the morphologic right ventricle and the pulmonary artery arising from the
1826
morphologic left ventricle. Associated abnormalities include VSD (40%), coarctation or interrupted aortic arch (10%), left ventricular outflow tract
obstruction (5% to 10%), and abnormal coronary artery branching patents in one third of patients.[39] Classification of TGA is into simple and complex
TGA.
Simple Transposition
The interventricular septum is intact or almost intact. Profound cyanosis presents soon after birth, worsened by closure of the PDA. Early survival
depends on the size of the ASD. Reopening of a closing ductus arteriosus with PGE1 infusion enhances mixing at the atrial level. The dominant
physiologic abnormality in TGA is reduced oxygenation with increased right and left ventricular volume load.
Complex TGA
The physiology is essentially unchanged from that of simple transposition, aside from the fact that a VSD or VSDs allow mixing at an additional level,
with a tendency to higher systemic saturations than occurs in simple TGA and adding congestive heart failure to the symptoms.
Diagnosis
In the neonatal period, diagnosis is made with echocardiography. Formal catheterization is rarely needed. If balloon atrial septostomy is required, this
can be done in the catheter laboratory, or preferably in the intensive care unit under echocardiographic guidance. Cardiac catheterization may be
requested in complex TGA in the absence of adequate echocardiographic detail or in delayed diagnosis to measure pulmonary resistance.
Surgical Intervention
The current “gold standard” is the arterial switch operation. Previously, atrial level repair with either a Senning or a Mustard procedure was used ( Fig.
58–17 ). The atrial level repair is no longer the procedure of choice owing to associated atrial arrhythmias, baffle obstruction, and late deterioration of
the morphologic right ventricular function in the systemic circuit.[40] Fundamental to the arterial switch operation is the fact that the left ventricle must be
able to handle the systemic workload. In simple TGA, closure of the PDA in the postnatal period decreases PAPs, which causes the left ventricle to
involute or decondition. Currently, experience indicates that the left ventricle remains adequately prepared for at least 1 month after closure of the PDA.
[41] Patients with a large VSD will have a higher pressure in the left ventricle.
Surgery can be undertaken when the infant is a little older but, in the absence of a pulmonary artery band, should be achieved by 3 months of age. The
arterial switch is performed on CPB through a median sternotomy. In simple TGA, circulatory arrest is avoided or utilized only for ASD closure. The
great vessels are transected, and the orifices and course of the coronary arteries are inspected. The coronary arteries are reimplanted into the neoaorta.
The posteriorly located pulmonary artery bifurcation is brought anterior to the aorta (Lecompte maneuver) and aortic reconstruction is completed.
Pulmonary artery reconstruction of the excised coronary artery buttons is with autologous pericardium. Continuity between the right ventricle and the
pulmonary arteries is reestablished ( Fig. 58–18 ). Any associated defects are repaired at the time of arterial switch.
Results
In some centers, mortality for simple TGA approaches 0%, whereas for TGA with VSD it is reported at between 3% and 5%. Factors shown to increase
the mortality in some series include intramural course of the left coronary artery, a retropulmonary course of the left coronary artery, multiple VSDs,
and hypoplasia of a ventricle.[42]
Figure 58-17 The Mustard operation. A, Interior of the right atrium is shown after a longitudinal right atriotomy is performed. The interatrial septum is excised along the dotted lines.
The coronary sinus is cut back toward the left atrial side. B, A baffle is used to divert the venal caval blood to the left atrium and across the mitral valve. Completion of the right atrial
suture line will allow systemic venous blood to enter the right atrium, cross the tricuspid valve, and exit into the transposed aorta. (From Trusler GA, Freedom RM: Transposition of
the great arteries: The Mustard procedure. In Sabiston DC Jr, Spencer FC [eds]: Gibbons Surgery of the Chest. Philadelphia, WB Saunders, 1983, p 1138.)
1827
Figure 58-18 The arterial switch procedure for transposition of the great arteries. A, The external anatomy is shown, with sites of transection of the two great vessels delineated by the
dotted lines. B, The aorta and main pulmonary arteries are transected and the coronary buttons have been removed from the native aortic root. C, The coronary buttons are transferred
to the neoaorta. D, The coronary button reimplantation sites are repaired with a pericardial patch, and the neopulmonary artery is anastomosed to the distal main pulmonary artery. The
distal pulmonary artery has been moved anterior to the ascending aorta (Lecompte maneuver). (From Wernovsky G, Jonas RA: Other conotruncal lesions. In Chang CA, Hanley FL,
Wernovsky G, Wessell DL [eds]: Pediatric Cardiac Intensive Care. Baltimore, Williams & Wilkins, 1998.)
Postoperatively, reevaluation for supravalvular, aortic, and pulmonary stenosis should be followed with echocardiography. Long-term follow-up of
patients has so far been notable for the low incidence of complications or need for reoperation in many series.[42] Late coronary artery obstruction has
been reported in some series.
Congenitally Corrected Transposition (ccTGA)
In this rare condition there is atrioventricular and ventriculoarterial discordance or DORV at the transposition end of the DORV spectrum. Saturated and
desaturated blood streams through this kind of heart to the appropriate destination but the morphologic RV is in the systemic circuit and is prone to a
high incidence of Ebsteinoid malformation of the tricuspid valve (systemic AV valve). The systemic RV tends to fail with time in much the same way
that the systemic RV tends to fail after atrial repair (Mustard or Senning) for TGA. Similarly in ccTGA the incidence and earliness of systemic RV
failure is related to associated lesions, and particularly the presence of a large VSD that has been previously patched. In addition the prevalence of an
Ebsteinoid type malformation of the tricuspid valve ensures a high incidence of early or progressive systemic AV valve regurgitation in ccTGA
compared with atrially repaired TGA. Spontaneous or surgical heart block occurs, adding to the burden of the morphologic RV.
In theoretical terms, a combined atrial repair (Mustard or Senning) and arterial switch in patients without significant pulmonary stenosis, or a combined
atrial repair and Rastelli operation for those with significant pulmonary stenosis, will extract the morphologic RV from the systemic circuit and decrease
the back pressure on a defective tricuspid valve. This has been called the “double switch operation.” However, this is a long operation with at least
significant theoretic hazards in terms of myocardial protection and ability to achieve a nonobstructed atrial repair particularly in the commonly found
configuration of a ventricular apex in a position discordant with the atrial situs. In this situation the morphologic RA lies behind the ventricular mass
and has a much smaller anteroposterior width of the free wall. Interestingly, this form of cardiac positional discordance appears to present more
frequently in the presence of severe pulmonary stenosis or atresia. In addition, the tricuspid valve appears less likely to be deformed compared with
when the apex of the heart is pointing in the appropriate direction for the type of atrial situs and is also less likely to be incompetent when significant PS
or pulmonary atresia is present.
There are two types of ccTGA. The most common is with atrial situs solitus and an L-loop of the ventricles (ccTGA SLL), and much more rarely (2% to
3% of a ccTGA) there is atrial situs inversus with a D-loop of the ventricles (ccTGA IDD). When the pulmonary valve comes largely off the RV
(DORV) there is nearly always severe pulmonary stenosis or atresia and the aorta is further from the VSD, making the Rastelli part of the double switch
more difficult, particularly if the VSD is somewhat restrictive. The usual VSD enlargement for a Rastelli by enlarging superiorly is not possible without
creating complete heart block in a ccTGA (SLL), because of the superiorly placed AV mode and conducting bundle. In ccTGA (IDD) the conducting
system is inferior and superior VSD enlargement is feasible.
If patients with ccTGA present with RV and TV failure with an intact interventricular septum (either congenitally or after previous VSD closure),
without LV outflow obstruction, the morphologic LV has involuted and is thin walled. Before achieving a double switch, the LV requires retraining by
pulmonary artery banding to a point where it is operating comfortably at near systemic pressure and with good function, before double switching is
feasible. It is clearly easier to retrain a morphologic LV in patients younger than 12 to 14 years of age than in late adolescence or adulthood, particularly
when full retraining must be achieved from an LV starting pressure that is of a normal pulmonary artery systolic pressure. The double switch after LV
retraining appears more hazardous and is more likely to be associated with late LV failure particularly in older patients. LV failure either during
retraining or after the double switch is an indication to consider heart transplantation at the appropriate clinical timing.
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Despite the magnitude of the double switch (0 to 8%) operations, the 30-day mortality has been acceptable particularly in the three centers with the
largest current experience (the author’s personal series,[43] Birmingham Children’s Hospital, UK,[44] and Tokyo Women’s Hospital[45] ). Intermediate-term
results also appear encouraging, but long-term results are not yet available.
Double-Outlet Right Ventricle
DORV is a conotruncal malformation in which both great arteries arise or mostly arise from the right ventricle. DORV is a spectrum of abnormalities
with variable physiology and surgical options.
By one definition, all of one and more than 50% of the other great artery arise from the right ventricle. Four types of DORV can be described based on
the relationship of the VSD to the great vessels ( Fig. 58–19 ): (1) subaortic VSD with or without pulmonary stenosis; (2) subpulmonary VSD with or
without subaortic stenosis (SAS) and associated arch obstruction; (3) doubly committed VSD; and (4) remote or noncommitted VSD. Associated
abnormalities occur frequently, including right or left ventricular hypoplasia, straddling or common AV valves, mitral or tricuspid valve stenosis or
Figure 58-19 The four types of VSD in double-outlet right ventricle (DORV). A view of the right-sided ventricular septum. CS, conal septum. A, Subaortic VSD. B, Subpulmonary
VSD. C, Doubly committed VSD. D, Remote or noncommitted VSD. (From Thompson WR, Nichols DG, Ungerleider RM: Double outlet right ventricle and double outlet left
ventricle. In Nichols DG, Cameron DE, Greeley WJ, et al [eds]: Critical Heart Disease in Infants and Children. St. Louis, Mosby, 1995.)
atresia, multiple VSDs, and an association with heterotaxy.[46] The physiology will depend on the nature of the VSD, the state of the AV valves, the
degree of pulmonary stenosis, and the degree of subaortic obstruction and arch obstruction.
Surgery
For subaortic VSD, biventricular repair involves closure of the VSD to baffle the left ventricular outflow to the aorta and relief of the pulmonary
outflow tract obstruction, which may or may not involve right ventricle-to-pulmonary artery conduit placement.[47] The subpulmonary VSD type of
DORV is physiologically similar to TGA. SAS and aortic arch obstruction often complicate this subtype. In the absence of significant pulmonary or
subpulmonary stenosis, surgical repair involves closure of the VSD to baffle the left ventricular outflow to the pulmonary root, an arterial switch
operation, and correction of associated arch obstruction and SAS. Surgical mortality for this subset is higher, around 10%. For DORV with a doubly
committed VSD or a remote VSD, biventricular repair is more difficult. Intraventricular baffling is frequently complex, and intracardiac obstruction
may result, particularly in the smaller heart. Staged management should be considered. Frequently, pulmonary artery banding or a palliative systemic-
to-pulmonary artery shunt allows somatic growth and a reassessment of the feasibility of a biventricular repair. If septation is possible but the right
ventricle is small, a one and one-half ventricle repair may be considered. If septation of the heart is impossible or very difficult, it may be safer to
perform a Fontan type of repair.
Left Ventricular Outflow Tract Obstruction

Left ventricular outflow tract obstruction (LVOTO) can occur at any level from the subaortic area to the descending aorta. These lesions may be isolated
or combined; and when combined with mitral and left ventricular hypoplasia, the eponym Shone’s syndrome is attached. Significant hypoplasia of the
left ventricle, mitral valve, and aortic valve may preclude a biventricular repair. Various degrees of left ventricular hypertrophy accompany LVOTO.
In severe LVOTO, endocardial fibroelastosis may be present at birth, further impairing contractile function and significantly affecting short- and long-
term survival. Neonates with critical or severe LVOTO present in a low cardiac output state and with multiple organ dysfunction. Adequate systemic
output requires an open duct, and PGE1 infusion is needed for resuscitation and stabilization. Older patients have time for the left ventricle to
hypertrophy. Concentric hypertrophy, if severe enough, can lead to subendocardial ischemia, which will present as an abnormal exercise response. If the
condition is untreated, left ventricular failure or sudden death supervenes. Surgery is directed at establishing unobstructed systemic blood flow, and a
wide variety of procedures are variously indicated: from the conservative LVOTO resection, through the radical Konno procedure, which includes
aortic valve replacement, to the Norwood-type procedure for overall uncorrectable left heart inadequacy.
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Aortic Stenosis
Valvular Aortic Stenosis
The degree of stenosis varies, and symptoms are usually seen only when the stenosis is severe. In symptomatic neonates, the valve leaflets are often
markedly thickened and nodular. The valve may be tricuspid, bicuspid, or unicuspid, and this has a bearing on the anticipated effectiveness of the
different forms of intervention. In older children, gross nodular myxomatous changes are less frequently seen. Multiple additional levels of LVOTO
may accompany valvular stenosis.
The echocardiogram is usually diagnostic. Neonates and infants present with congestive heart failure or circulatory collapse. Older infants present with
signs of congestive heart failure or a murmur. Surgery in the neonate can be briefly delayed for resuscitation with PGE1 , inotropes, and positive-
pressure ventilation. Urgent relief of the obstruction should be considered. In older infants, a measured gradient of greater than 50 mm Hg, a positive
exercise test, or symptoms (even in the presence of normal left ventricular function) are indications for intervention.
Surgery
Balloon dilation is considered by some centers as a valid alternative to surgical valvotomy. Surgical valvotomy is undertaken under CPB. The aortic
valve is incised through an aortotomy ( Fig. 58–20 ). Patients with severe multiple left heart obstructions may be better served with a Norwood-type
procedure.[48] In older patients, aortic valvuloplasty is undertaken. Aortic valve replacement is the less preferable option. Replacement may be with a
bioprosthetic, mechanical valve, or a homograft. Another option is the Ross procedure, which involves translocation
Figure 58-20 Close-up of the aortic valve demonstrates a surgical valvotomy. A, The valve is bicuspid with a prominent raphe in the anterior valve leaflet. B, The orifice is enlarged
by incising the fused commissure between the two leaflets. (From Chang AC, Burke RP: Left ventricular outflow tract obstruction. In Chang AC, Hanley FL, Wernovsky G, Wessell
DL [eds]: Pediatric Cardiac Intensive Care. Baltimore, Williams & Wilkins, 1998.)
of the excised, native pulmonary valve into the aortic position with coronary artery relocation. The right ventricular outflow tract is then reconstructed
utilizing a pulmonary homograft ( Fig. 58–21 ). The Ross operation has risks, and those reported include coronary artery insufficiency, aortic
insufficiency, ventricular dysfunction, RVOTO, and LVOTO. Additionally, the conduit in the pulmonary position will have to be replaced in time.[49]
For patients with severe complex LVOTO, aortoventriculoplasty or a Konno procedure is an option. An incision is made into the aorta, and a right
ventriculotomy is extended into the septum. The aortic annulus is enlarged with a prosthetic patch. A patch is used to close the right ventriculotomy
separately. A modification of this procedure includes the Ross-Konno operation.[50]
Results
In expert hands, there is little difference in mortality between balloon valvuloplasty and surgical valvotomy.[51] Surgical valvotomy provides the
opportunity of a more precise valvotomy and débridement of thickened nodular leaflets. Mortality for the Ross-Konno operation in selected centers can
be less than 5% but is higher in neonates and infants.[50]
Subaortic Stenosis
SAS is divided into three groups: discrete membranous, fibromuscular tunnel type, and hypertrophic type. Discrete membranous SAS consists of a
fibrous ring located below the level of the aortic valve. The aortic valve is often distorted, owing to abnormal flow patterns, and aortic insufficiency is
common. So-called discrete membranous SAS is nearly always associated with a muscular abnormality of the LVOTO, and membrane removal should
be combined with muscle wedge excision. The fibromuscular tunnel type is less common. The hypertrophic type is a dynamic outflow tract obstruction
with hypertrophy of the underlying interventricular septum (idiopathic hypertrophic SAS). The characteristics of this are asymmetrical septal
hypertrophy and the presence of systolic anterior motion of the anterior leaflet of the mitral valve. A posteriorly malaligned VSD may contribute
postoperatively to a degree of subaortic obstruction. Associated lesions include malalignment VSD defects, coarctation of the aorta, and CAVC.
Physiology
These lesions have in common a pressure overload on the left ventricle associated with progressive left ventricular hypertrophy and eventual
dysfunction. Additionally, with SAS, the turbulence created below the valve leaflets causes thickening and distortion of the aortic valve with an
increased risk of aortic regurgitation. Echocardiography can identify the anatomic site of obstruction as well as the degree of obstruction. Cardiac
catheterization is rarely indicated. Serial exercise testing is useful for borderline cases.
Surgery
Indications for surgical intervention include a gradient of more than 25 mm Hg, the presence of underlying aortic insufficiency, and coexisting lesions.
Surgery removes the LVOTO and preserves the aortic valve ( Fig. 58–22 ). In Figure 58–22B the authors would recommend that the muscular wedge
incision is made more to the left than shown in this illustration to avoid complete heart
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Figure 58-21 The Ross procedure. A, The anatomy is shown with the lines of transection of the pulmonary autograft and the diseased aortic valve root. B, The pulmonary autograft is
removed and is placed into the aortic root with coronary artery transfer. C, A pulmonary allograft is placed to reestablish right ventricular-to-pulmonary artery continuity. (From
Kouchoukos NT, Davila-Roman VT, Spray TL, et al: Replacement of the aortic root with a pulmonary autograft in children and young adults with aortic valve disease. N Engl J Med
330:1, 1994. Copyright 1994 Massachusetts Medical Society. All rights reserved.)
Figure 58-22 A, Excision of discrete subaortic stenosis. The aorta is opened obliquely, and the aortic valve leaflets are retracted to expose the subaortic membrane. The membrane is
excised circumferentially along the indicated line. B, This is usually combined with a muscle resection. (From de Leval M: Surgery of the left ventricular outflow tract. In Stark J, de
Leval M [eds]: Surgery for Congenital Heart Defects, 2nd ed. Philadelphia, WB Saunders, 1994.)
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block. Aortic insufficiency may be addressed by repair, but replacement may be necessary. For the tunnel-type SAS, a more radical procedure has to be
undertaken; this would be a Konno or a modified Ross-Konno procedure. Current mortality is less than 5% for uncomplicated SAS. With a
fibromuscular tunnel and other extensive surgeries, the mortality rate is higher. Recurrence and reoperation for SAS vary between 5% and 10%, with
recurrence rates higher in the younger patient with severe stenosis.[52]
Supravalvular Aortic Stenosis
Supravalvular is rare and consists of a localized or diffuse narrowing from the level of the sinotubular junction. It is often associated with Williams’
syndrome. A narrowing occurs above the aortic valve and the sinuses of Valsalva associated with a generalized vessel wall thickening. The aortic valve
leaflets may or may not be abnormal. The coronary orifices may be obstructed by the thickened ring of tissue at the sinotubular junction.[53] Two types of
supravalvular stenosis are described. Branch vessels from the ascending or descending aorta may be stenotic or hypoplastic. Associated lesions are
pulmonary stenosis (valvular, supravalvular, or peripheral), aortic valve stenosis, and coarctation of the aorta. The pathophysiology is that of pressure
overload and progressive left ventricular hypertrophy and eventual dysfunction.
Diagnosis
Echocardiography is usually diagnostic. In association with Williams’ syndrome, right-sided obstruction should be excluded. Cardiac catheterization is
probably indicated to identify coronary artery origin narrowing, other systemic artery origin stenosis, and peripheral pulmonary artery stenosis.
Surgery
Indications for surgery are a gradient of more than 50 mm Hg in an asymptomatic patient or positive exercise testing. Coronary artery stenosis is an
indication for surgery. Through a median sternotomy and under CPB, patch angioplasty of the ascending aorta has been satisfactory ( Fig. 58–23 ).
Mortality for surgery is lower for the discrete type of supravalvular aortic stenosis (<5%). With the diffuse form, the risk of recurrence and re-
intervention is quite high.[53]
Aortic Arch Interruption
Aortic arch interruption is a rare lesion with loss of continuity between the ascending and the descending aorta. Blood flowing to the descending aorta is
maintained through a large ductus arteriosus. Interrupted arch is rarely isolated, and in 80% of cases there is an associated large, malalignment VSD.
Other associated abnormalities include a bicuspid aortic valve, LVOTO, DiGeorge’s syndrome, truncus arteriosus, single ventricle, TGA, DORV, and
aorticopulmonary window.
Aortic arch interruption is classified into the three types of Celoria and Patton, types A, B, and C. Type A (interruption beyond the left subclavian
artery) occurs in approximately 25% of patients. Type B (interruption between the left carotid and the subclavian arteries) is the most common form,
occurring in approximately 70% of patients. Type C (interruption
Figure 58-23 Surgical repair of discrete supravalvular aortic stenosis. A, The autotomy is performed extending into both the noncoronary and the right coronary sinuses. B, After the
supravalvular ridge and obstructive tissue are excised, a Y-shaped, pantaloon-shaped patch of pericardium is sutured in place. (From Van Son JA, Danielson GK, Puga FJ, et al:
Supravalvular aortic stenosis: Long term results of surgical treatment. J Thorac Cardiovasc Surg 107:103, 1994.)
between the innominate and the left carotid arteries) is the rarest, occurring in less than 5% of patients ( Fig. 58–24 ). Interrupted aortic arch is thought
to occur as a result of disappearance of a normally persisting connection between the left fourth and the left sixth aortic arches. Most infants with
interrupted aortic arch present within the first few days of life. Reduced systemic blood flow to the lower extremities causes acidosis, renal failure,
hepatic ischemia, and necrotizing enterocolitis. Additionally, the pulmonary circulation is flooded as the PVR drops. These patients develop rapid
congestive heart failure. Pulse oximetry may reveal a differential between left upper body, right upper body, and lower body saturations. PGE1 allows
reopening of the duct, providing a period for recovery before undertaking complete repair. Ventilatory management is used to limit pulmonary blood
flow. The DiGeorge syndrome or variant occurs in 15% to 30% of infants with interrupted aortic arch. Hypocalcemia can be problematic, and blood
products should be irradiated. Echocardiography is diagnostic.
Surgery
In the current era, a single-stage complete repair is preferable to a staged approach. Utilizing CPB and DHCA, resection of all ductal tissue followed by
anastomosis between the separated aortic segments is performed. The VSD and other defects are repaired on CPB to limit the period of DHCA. The
VSD is closed, and other
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Figure 58-24 The types of interrupted aortic arch. A, The interruption is at the aortic isthmus between the left subclavian artery and the ductus arteriosus. B, The interruption is at the
distal aortic arch between the left carotid and the left subclavian arteries. C, The interruption is located at the proximal aortic arch between the innominate and the left carotid arteries.
(From Chang AC, Starnes VA: Interrupted aortic arch. In Chang AC, Hanley FL, Wernovsky G, Wessell DL [eds]: Pediatric Cardiac Intensive Care. Baltimore, Williams & Wilkins,
1998.)
defects are treated accordingly. Surgical management of interrupted aortic arch continues to have a relatively high mortality rate owing to associated
defects. Reported mortality rates vary between 10% and 38%. Risk factors for mortality are low birth weight, interrupted aortic arch type B, and SAS.[54]
Coarctation of the Aorta
Coarctation of the aorta is a congenital narrowing of the thoracic aorta, usually occurring distal to the left subclavian artery, at the point of insertion of
the ductus arteriosus. Coarctation represents 5% to 8% of all cases of congenital heart disease. It is associated with other congenital heart defects,
specifically PDA, VSD, bicuspid aortic valve, subaortic obstruction, and mitral valve abnormalities.
The site of coarctation is always juxtaductal, with or without associated arch or isthmic hypoplasia. Two theories for the development of coarctation are
the flow theory and the ductal sling theory. Coarctation of the aorta occurs in two groups of patients: infants with associated cardiac anomalies and
those with isolated severe coarctation of the aorta, with the blood flow to the lower extremities dependent on the ductus arteriosus. Patients with severe
coarctation and duct-dependent descending aortic flow present with cardiovascular collapse at the time of spontaneous ductal closure. PGE1 opens and
maintains the patency of the ductus arteriosus, preserving distal organ perfusion. Surgical intervention can be delayed until stabilization of the child has
been achieved. Older patients are often asymptomatic or present with claudication on exercise or with upper body hypertension. Alterations in renal
function, baroreceptor function, and the renin-angiotensin axis contribute to the proximal systemic hypertension. Later in life, these patients may
develop aortic aneurysms proximal or distal to the coarctation, aortic dissection, cerebral aneurysm rupture, and increased atherosclerotic disease.
Physical findings of absent femoral pulses and poor distal perfusion are highly suggestive of the diagnosis in an infant. Echocardiography is diagnostic
in most instances. This study can document the branching pattern of the head and neck vessels and determine the aortic arch and isthmus size. In the
older child who is asymptomatic, upper extremity hypertension and a differential between upper and lower body pressures should be diagnostic. The
echocardiogram in most instances will confirm the diagnosis. Cardiac catheterization may be required for associated cardiac anomalies. Other
modalities that may be useful include computed tomography (CT) and MRI. In the older patient, diagnosis is an indication for elective intervention.
Surgery
The surgical approach is through a left posterolateral thoracotomy. The exception is with important associated intracardiac anomalies, when the defect is
repaired simultaneously through the midline. Surgical options include resection and end-to-end anastomosis ( Fig. 58–25 ), prosthetic patch aortoplasty,
and subclavian flap aortoplasty. In the current era, the re-coarctation rate for end-to-end anastomosis is less than previously reported. The re-coarctation
rate in neonates is about 10%. Prosthetic patch aortoplasty was introduced owing to the high rate of re-coarctation with the earlier end-to-end technique.
Aneurysm formation has been reported in 5% to 30% of these patients. Subclavian flap aortoplasty utilizes the subclavian artery as an onlay graft.
Variations of this technique include a reverse subclavian flap for repair of a coarctation proximal to the left subclavian artery. In older children, left arm
ischemia after ligation of the subclavian artery can occur and sacrifice of the left subclavian artery may affect long-term growth and function of the
limb. Prosthetic interposition graft placement has been used but has disadvantages in a growing child.
Potential complications during and after coarctation repair include hemorrhage, recurrent laryngeal nerve injury, Horner’s syndrome, paraplegia, stroke,
aneurysm formation, and recoarctation. Postoperatively, paradoxic
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Figure 58-25 Repair of discrete coarctation by end-to-end anastomosis. A, The lines of resection of the coarctation are shown, as is the relationship of the vagus and recurrent
laryngeal nerves. B, Completion of the anterior suture line. Note the clamp positioning preserving upper body flow. (From Castaneda AR, Jonas RA, Mayer JE, Hanley FL: Aortic
coarctation. In Cardiac Surgery of the Neonate and Infant. Philadelphia, WB Saunders, 1994.)
hypertension is a common occurrence. It relates to the release of pressure on the baroceptors in the carotid arteries and aortic arch after removing the
obstruction, as well as dissection around the sympathetic plexus of the aorta. This has to be carefully controlled postoperatively because reperfusion of
the mesenteric arteries at a high pressure is potentially hazardous. Children are particularly prone to the development of mesenteric ischemia with severe
abdominal pain, distention, and the development of an ileus. Hypertension usually resolves within 2 to 4 weeks postoperatively.
Paraplegia is a feared complication of aortic adult coarctation surgery, with an incidence of up to 1.5%. The incidence in neonates and children is closer
to 0.2%. Paraplegia correlates with the length of aortic crossclamping. Prevention of paraplegia requires the shortest possible cross-clamp time,
moderate hypothermia intraoperatively (34°C to 35°C), a high-enough proximal blood pressure, and the avoidance of acute blood loss or acidosis.
Aneurysms are related to all types of coarctation repair but especially prosthetic patch aortoplasty. Risk of aneurysms is greater over the age of 15 years
or in patients undergoing operation for re-coarctation. Reoperation is required for a postoperative peak systolic pressure gradient over 20 mm Hg across
the repair.[55] In recurrent coarctation, balloon angioplasty is the procedure of choice; initial success rates are high, with a low incidence of
complications.[56]
Unseptatable Hearts and the Fontan Principle
When it is estimated that one of the ventricles is inadequate for supporting total cardiac output, attempts at septation become too risky. A large number
of diverse pathologic processes fall into this category. In unseptatable hearts, the aim of palliation is to ensure adequate, but not excessive, pulmonary
blood flow, unobstructed systemic outflow from the ventricular mass, and unobstructed pulmonary venous return to the ventricular mass. If the early
and subsequent palliative procedures are well conceived and executed, each patient will then fulfill criteria controlling eligibility for a Fontan-type
repair. The Fontan principle requires that providing the total resistance to blood flow through the lungs and into the ventricular cavity is nearly normal;
the systemic venous return can be connected directly to the pulmonary arteries without an intervening pump. This will separate oxygenated and
nonoxygenated blood. Systemic venous blood will then flow continuously through the lungs with a mean pressure of between 8 and 14 mm Hg. The
Fontan procedure has many modifications to separate the systemic and pulmonary circuits and achieve full oxygen saturation of systemic arterial blood.
An often used preliminary procedure before completing the Fontan connection is the bidirectional cavopulmonary shunt (superior vena cava connected
to pulmonary artery) or bidirectional Glenn shunt ( Fig. 58–26 ). This intermediate procedure reduces volume load on the ventricle. The initial selection
criteria by Fontan were stringent,[57] and these principles remain largely valid. Improved understanding of Fontan physiology has allowed modifications
to the selection of patients and the classification of risk.[58] Complications of the Fontan procedure are frequent in the postoperative period: increased
incidence of atrial arrhythmias, pleural effusions, and, with bad Fontan physiology, ascites with protein-losing enteropathy and progressive ventricular
dysfunction. Currently, the theoretical hemodynamic advantages of the lateral tunnel Fontan procedure ( Fig. 58–27 ) are being evaluated.[59]
Tricuspid Atresia
Tricuspid atresia is characterized by the absence of a communication between the right atrium and the right ventricle. Associated with this anomaly are
an ASD, enlargement of the mitral valve and left ventricle, and a varying degree of right ventricular hypoplasia. Tricuspid atresia is a relatively common
cyanotic heart lesion, occurring in 0.3% to 3.7% of patients with congenital heart disease.
Anatomy and Physiology
Tricuspid atresia has been classified into three types. In type 1 (70% of patients), the great arteries are in concordance with the ventricles. Type 2 (20%
of patients) comprises hearts with TGA. Type 3 (<10%) includes hearts with AV discordance and TGA.
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Figure 58-26 The cavopulmonary shunt or bidirectional Glenn shunt. An end-to-side anastomosis of the superior vena cava (SVC) to the right pulmonary artery (RPA) is performed.
The proximal end of the SVC is divided at the cavoatrial junction. IVC, inferior vena cava; LPA, left pulmonary artery; RA, right atrium. (From Bridges ND, Jonas RA, Mayer JE, et
al: Bidirectional cavopulmonary anastomosis as interim palliation for high-risk Fontan candidates: Early results. Circulation 82[Suppl IV]:IV-170, 1990. Copyright 1990, American
Heart Association.)
Physiology depends on the degree of obstruction to pulmonary blood flow. As a rule, patients with obstruction to pulmonary blood flow have
unobstructed systemic blood flow. In contrast, patients with unobstructed pulmonary blood flow tend to have some obstruction to systemic flow.
Diagnosis and Management
Echocardiography is diagnostic of the cardiac morphology. Cardiac catheterization is required for assessment of PVR and to assess the anatomy of the
pulmonary arteries in patients subjected to either pulmonary artery banding or previous shunt placement.
Surgery
The ultimate goal for these patients is to achieve anatomy and physiology favorable for an eventual Fontan circuit. Patients with inadequate pulmonary
blood flow will require shunting, and care is taken to avoid overshunting. With unobstructed pulmonary blood flow, a pulmonary artery band is applied
early in life. Atrial septectomy can be performed at the same time, but in general, neonates will have already been subjected to balloon atrial
septostomy. Survival and mortality rates reflect the complexity of the lesion, the effectiveness of initial palliation, and correct decision making at the
time
Figure 58-27 Modification of the Fontan operation—the lateral tunnel Fontan with total cavopulmonary anastomosis. A, Polytetrafluoroethylene patch is placed in the right atrium,
creating a uniform tunnel between the superior and the inferior vena cava. The main pulmonary artery is transected. The bidirectional Glenn is then performed (if not already present).
B, Completed total cavopulmonary connection. (From Stein DG, Laks H, Drinkwater DC, et al: Results of total cavopulmonary connection in the treatment of patients with a
functional single ventricle. J Thorac Cardiovasc Surg 102:280, 1991.)
of assessment for completing the Fontan circuit. Most centers report an early mortality of around 5% for tricuspid atresia. The actuarial survival after
the Fontan operation deviates significantly from that of normal people. Failing Fontans are managed by residual lesions or by heart transplantation.
Hypoplastic Left Heart Syndrome
Hypoplastic left heart syndrome (HLHS) encompasses a constellation of features: severe aortic valve hypoplasia or atresia, hypoplasia of the ascending
aorta, stenosis or atresia of the mitral valve, and hypoplasia or atresia of the left ventricle. Associated noncardiac abnormalities are frequent and affect
survival. HLHS accounts for 25% of cardiac mortality in the first week of life, with an incidence of 7% of all cardiac anomalies. Norwood[60] was the
first to describe an operative method for successful palliation. These principles have led to an option for managing other forms of single-ventricle,
complex lesions with LVOTO. The staged operative strategy for long-term palliation involves creation of an unobstructed outlet to the systemic circuit
and adequate pulmonary blood flow, followed a few months later by a bidirectional cavopulmonary shunt and, finally, completion of the Fontan.
There are variations in the degree of stenosis or atresia of the aortic and mitral valves. The ascending aorta ranges from 1 to 2 mm to near normal in size
( Fig. 58–28 ). In neonates, the duct must be kept open with PGE1 infusion. As PVR falls, in the setting of parallel circulations, available cardiac output
preferentially goes to the lungs. This leads to progressive acidosis from reduced systemic perfusion. The key to managing these patients is to balance the
pulmonary and systemic
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Figure 58-28 Anatomy of the hypoplastic left heart syndrome. The tiny ascending aorta is seen, arising from a markedly hypoplastic left ventricle. The ductus arteriosus is large,
providing forward flow to the systemic circuit. The right ventricle is hypertrophied, and the pulmonary artery is enlarged. (From Wernovsky G, Bove EL: Single ventricle lesions. In
Chang AC, Hanley FL, Wernovsky G, Wessell DL [eds]: Pediatric Cardiac Intensive Care. Baltimore, Williams & Wilkins, 1998.)
blood flows. This is achieved by controlling ventilation to adjust the PVR by increasing the hematocrit and pharmacologically by manipulating the
systemic vascular resistance and PVR.[61]
Diagnosis
Echocardiography is diagnostic and cardiac catheterization is not usually indicated, unless associated anomalies require further delineation and
clarification.
Surgery
First-stage palliation is accomplished with the Norwood procedure or a modification. Important to achieving survival is aggressive preoperative
management and optimizing all organ systems before surgery. This is achieved by balancing the systemic and pulmonary blood flow ratios. Surgical
attainment of the Norwood operation usually involves a period of circulatory arrest, although, recently, almost complete avoidance of cerebral
circulatory arrest has been achieved by regional perfusion through the upper end of a preplaced PTFE shunt. A modification of the Norwood operation
has been described,[62] without using homograft material for arch reconstruction but using all native tissue ( Fig. 58–29 ). A stable source of pulmonary
blood supply is provided by the creation of a shunt. More recently, Sano[63] has described placement of a small RV to distal MPA PTFE conduit instead
of systemic shunt. Theoretically, this poses an advantage in that there is no diastolic steal from the aorta, compared with the use of systemic shunt. This
new approach is rapidly gaining acceptance. Postoperative management involves balancing the pulmonary and systemic circulation again. Provided
Figure 58-29 The Norwood procedure for first-stage palliation of the hypoplastic left heart syndrome. A, The main pulmonary artery (MPA) is divided proximal to the bifurcation, the
ductus arteriosus is ligated and divided, and the aortic arch is opened from the level of the transected MPA to a point distal to the ductal insertion in the descending aorta. B, A
segment of homograft is cut to an appropriate size and shape. This is sutured into place, creating an unobstructed outflow from the right ventricle to the pulmonary artery and aorta. C,
Polytetrafluoroethylene tube graft is placed from the innominate artery to the right pulmonary artery. The atrial septectomy is done while the patient is under circulatory arrest as well.
(From Castaneda AR, Jonas RA, Mayer JE, Hanley FL: Hypoplastic left heart syndrome. In Cardiac Surgery of the Neonate and Infant. Philadelphia, WB Saunders, 1994.)
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aortic arch and pulmonary artery growth is satisfactory, the second stage is undertaken when the child outgrows the shunt. The ventricle is unloaded by
a bidirectional cavopulmonary shunt, and this is later converted to a lateral tunnel Fontan or a modification thereof at 1½ to 5 years of age. Some centers
prefer to utilize primary heart transplantation for HLHS.[64] This approach is hindered by the lack of donor availability.
Results
Current reviews indicate that survival after surgical intervention for both transplantation and staged palliation is approximately 67% at 1 month and
52% at 12 months.[64] An advantage of staged palliation with use of the Fontan is that transplantation may be avoided for a number of years.

OTHER ANOMALIES
Coronary Artery Anomalies
Anomalies occur as a result of anomalous origin, termination, courses, and aneurysm formation. Of these variables, only anomalous left coronary artery
rising from the pulmonary artery (ALCAPA) and coronary artery fistulas are discussed. An ALCAPA is a rare lesion often lethal in early infancy.
Untreated, the mortality approaches 90%.
Anomalous Left Coronary Artery Rising from the Pulmonary Artery
Developmentally, failure of the normal connection of the left coronary artery bud to the aorta results in an abnormal connection to the pulmonary artery.
The abnormal origin can be situated in the main pulmonary artery or proximal branches. Associated abnormalities are rare but important to recognize
because lowering of the PAP by PDA ligation or closure of a VSD can be fatal if the ALCAPA is not noted. In utero, with equal pulmonary arterial and
aortic pressures, satisfactory perfusion of the ALCAPA can occur. After birth, the PAP falls and left coronary artery perfusion decreases. Ischemia
causes impaired ventricular function and myocardial infarcts and leads to left ventricular dilation. Papillary muscle dysfunction causes mitral
regurgitation. Early coronary collateral development may prevent ongoing infarction.
ALCAPA should be suspected in any infant with mitral regurgitation, ventricular dysfunction, or dilated cardiomyopathy. The syndrome of angina with
feeding in infants was described by Bland and colleagues,[65] with sudden death and angina precipitated by feeding. Sudden death has been described in
older children. Infants present with a low cardiac output and systemic heart failure. The electrocardiogram may reflect ischemic changes. The
echocardiogram is usually diagnostic, but because this diagnosis is often confused with dilated cardiomyopathy there is an argument in favor of
catheterizing all patients with dilated cardiomyopathy in whom the coronary artery anatomy cannot be clearly defined on echocardiography. Secondary
findings of dilated cardiac chambers and segmental wall motion
Figure 58-30 Direct reimplantation of the anomalous left coronary artery arising from the pulmonary artery (ALCAPA). A, Excision of the ALCAPA from the pulmonary artery
(PA). AO, aorta. B, Aortic reimplantation of the coronary ostium into the aorta. C, Reconstruction of the PA with autologous pericardium. (From Vouhe PR, Tamisier D, Sidi D, et al:
Anomalous left coronary artery from the pulmonary artery: Results of isolated aortic reimplantation. Ann Thorac Surg 54:621, 1992. Reprinted with permission from the Society of
Thoracic Surgeons.)
abnormalities together with mitral regurgitation should prompt a search for an ALCAPA. Diagnosis of an ALCAPA is an indication for intervention.
Surgery
A degree of ventricular dysfunction is usually present. Preoperative inotropic support and optimization of hemodynamics may be required before
surgical intervention. Severe cardiomyopathy may rarely necessitate cardiac transplantation. Current experience indicates that creation of a dual
coronary system is safe and reproducible and offers the best opportunity for recovery of function.[65] Operative considerations include optimal
myocardial protection and prevention of left heart distention. Direct reimplantation of the ALCAPA into the ascending aorta is currently the procedure
of choice ( Fig. 58–30 ). Sometimes, limited mobility of the coronary artery will preclude reimplantation, and a surgically created aorta-pulmonary
artery-coronary artery tunnel is created: the Takeuchi procedure.[66] Ligation of the ALCAPA is not recommended.
Postoperative management is directed toward maintaining adequate coronary perfusion and cardiac output. Mechanical support of the heart may be
required temporarily. Mitral regurgitation usually improves, and valve replacement is rarely necessary. Current intervention has a low operative
mortality. Risks for nonsurvival relate to preoperative ventricular dysfunction and cardiogenic shock. The Takeuchi repair is associated with tunnel
complications such as obstruction, leak, aortic valve damage, and RVOTO in the long term.
Coronary Arteriovenous Fistula and Aneurysms
Isolated coronary artery fistula is rarer than ALCAPA. Aneurysms are associated with Kawasaki’s disease.
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Drainage of coronary artery fistula is reported to terminate more commonly in the right side of the heart or pulmonary artery than in the left side of the
heart. A shunt from the high-pressure coronary artery system into a low-pressure cardiac chamber may result in coronary steal and some degree of
cardiac volume overload.
Presentation depends on the amount of functional compromise produced by the ischemia and volume overload. Echocardiography may be able to
delineate the anomaly, but coronary angiography is diagnostic. Details of coronary anatomy are essential for determining intervention. Interventional
catheterization is useful for the obliteration of fistulas and terminal aneurysms.[67]
Surgery
If the lesion is not amenable to transcatheter intervention, surgery is indicated. The options include suture ligation without bypass, CPB, and
aneurysmectomy with closure of the fistula. Early and late mortality are low. Risk factors for death and ventricular dysfunction relate to coronary artery
insufficiency and infarction after fistula ligation or aneurysmectomy.
Vascular Rings and Pulmonary Artery Sling
Vascular rings and pulmonary artery sling are abnormalities of the aortic arch and its branches, compressing the trachea and or esophagus. The ring may
be either complete or partial. A pulmonary artery sling occurs when the left pulmonary artery arises from the right pulmonary artery, passing leftward
between the trachea and the esophagus. The trachea may be compressed, the cartilage may be soft, or there may be intrinsic stenosis of the trachea in the
form of complete cartilage rings.
Anatomy
Categorization of the defects is useful for description:
Complete Vascular Rings
Double arch: equal arches or left or right arch dominant
Right arch: left ligamentum arteriosus from anomalous left subclavian artery
Right arch: mirror image branching, with left ligamentum from descending aorta
Partial Vascular Rings
Left arch: aberrant right subclavian artery
Left arch: innominate artery compression
Pulmonary Artery Slings
Understanding of aortic arch formation has been enhanced by the hypothetical model of paired segmental structures and a double aortic arch in the
embryo.[68] The final configuration of the aortic arch and its branching pattern depends on the regression and preservation of specific segments ( Fig. 58–
31 ). The double aortic arch is the most common form of complete ring. Two arches arise from the ascending aorta, forming a true ring. The left arch is
usually smaller. The right arch-left ligamentum complex is formed from persistence of the right fourth arch and regression of the left fourth arch. The
anomalously arising left subclavian artery is often associated with a diverticulum at its base (Kommerell). In partial rings, the most common form is an
aberrant right subclavian artery arising distal to the left subclavian artery with a left arch. The right subclavian artery passes behind the esophagus from
left to right. Innominate artery compression arises from a more posterior and leftward origin of the innominate artery from a left arch, leading to anterior
compression of the trachea.
Symptoms reflect the degree of tracheal and esophageal compression, as well as the presence of coexistent tracheomalacia or stenosis from complete
rings. Upper respiratory symptoms predominate, with a characteristic brassy cough, recurrent respiratory infections, failure to thrive, and, sometimes,
esophageal motility problems. In children, documentation of a ring is an indication for surgery. Older patients are often asymptomatic. Initially,
diagnosis is made by a high index of suspicion and the barium swallow as the first investigation. Nowadays, echocardiography can document an
abnormal head and neck vessel branching pattern, excluding intracardiac abnormalities. MRI provides complete anatomic detail.
Surgery
Most vascular rings are accessible through a left posterolateral thoracotomy (the exception is a left arch with right-sided ligamentum). Division of the
ring and, in the case of double arch, preservation of the dominant arch is performed. Preservation of the recurrent laryngeal nerve is of importance.
Pulmonary artery slings are approached through the midline, and currently the use of CPB facilitates tracheal reconstruction and relocation of the right
pulmonary artery ( Fig. 58–32 ).[69] Repair can be achieved with low risk. Symptoms may take months to resolve, with slow resolution of the underlying
tracheomalacia.
Ebstein’s Anomaly of the Tricuspid Valve
Ebstein’s anomaly of the tricuspid valve is a rare defect in which the tricuspid valve attachments are displaced into the right ventricle to varying
degrees. Ebstein’s anomaly comprises a spectrum of abnormalities involving a degree of displacement of the tricuspid valve, variable right ventricular
size, and variable pulmonary outflow obstruction. Associated abnormalities are an ASD, pulmonary atresia, and congenitally corrected transposition.
The tricuspid valve’s posterior and septal leaflets are variably displaced to the apex of the right ventricle. This results in an atrialized portion of the right
ventricle. The anterior leaflet remains large and sail-like. The major hemodynamic issue is tricuspid incompetence with decreased pulmonary blood
flow and, if an ASD is present, right-to-left shunting causing cyanosis. Long-standing tricuspid incompetence leads to volume overload of an abnormal
right ventricle. Variable pulmonary outflow tract obstruction will limit effective pulmonary blood flow. If adequate pulmonary blood flow requires
continued ductal patency, then the need for neonatal intervention is almost certain.
Diagnosis and Intervention
The more severe forms of Ebstein’s anomaly present with cyanosis in infancy. Ill neonates tend to have a severe form of the disease, with a grossly
inefficient right ventricle compounded by the high pulmonary resistance of the neonate or by pulmonary
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Figure 58-31 The process of resorption and fusion of the paired first to the sixth branchial arches with paired dorsal aortas results in the formation of the normal aorta, aortic arch, and
pulmonary artery. The appearance of the aorta at various embryo crown-rump lengths is shown. (From Castaneda AR, Jonas RA, Mayer JE, Hanley FL: Vascular rings, slings, and
tracheal anomalies. In Cardiac Surgery of the Neonate and Infant. Philadelphia, WB Saunders, 1994.)
valve atresia. The mortality rate in this group is high. Older patients present in heart failure and may have cyanosis. Supraventricular arrhythmias and
the preexcitation syndrome (Wolff-Parkinson-White) are associated with Ebstein’s anomaly. Echocardiography is diagnostic. Critically ill neonates
have poor survival rates, and surgery is indicated only after stabilization with PGE1 and controlled ventilation. In the older patient, cyanosis and heart
failure are indications to intervene, although recently earlier intervention in the asymptomatic patient, before excessive RV dilation, is being more
actively pursued.
Surgery
In critically ill neonates, after stabilization, palliation with a systemic-to-pulmonary artery shunt may be required. The Starnes operation has allowed
salvage in previously hopeless cases. This operation consists of patch closure of the tricuspid orifice, atrial septectomy, and a systemic-to-pulmonary
artery shunt.[70] This approach commits the child to a Fontan-type repair in the future. In patients with less severe forms of this disease, options include
tricuspid valve repair using several ingenious methods ( Fig. 58–33 )[71] and tricuspid valve replacement. Surgery should be performed when heart size
increase is documented.
Results
Neonates have poor survival rates after palliative surgery. Older patients have a reported operative mortality of 0% to 25%.
Mitral Valve Anomalies
Most abnormalities of the mitral valve are associated with other complex lesions, for example, Shone’s complex. More commonly, mitral disease in the
pediatric population will be inflammatory in nature, that is, rheumatic disease or infective endocarditis. It may also be associated with collagen vascular
disease and Marfan’s syndrome.
Mitral Stenosis
Mitral stenosis is caused by obstruction at a supravalvular, valvular, or subvalvular level, singly or in combination. Supravalvular stenosis is due to a
ring of fibrous tissue above the annulus of the mitral valve or attached to the proximal leaflets. Valvular stenosis involves the leaflets, with commissural
fusion occurring with or without hypoplasia of the valve ring. Hypoplasia of the mitral valve is often associated with left ventricular hypoplasia.
Frequently, the leaflets and subvalvular apparatus are dysplastic as well. Fusion of the leaflets can lead to an accessory orifice and produce mitral
stenosis at a pure valvular level (so-called double-orifice mitral valve). Three types of subvalvular stenosis have been recognized: parachute mitral
valve, hammock valve, and absence of one or both papillary muscles.
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Figure 58-32 Method for the management of a pulmonary artery sling with associated tracheal stenosis, using cardiopulmonary bypass. A, Tracheal resection of the involved segment.
B, Anterior translocation of the left pulmonary artery after transection of the trachea. C, Direct anastomosis of the trachea. (From Castaneda AR, Jonas RA, Mayer JE, Hanley FL:
Vascular rings, slings, and tracheal anomalies. In Cardiac Surgery of the Neonate and Infant. Philadelphia, WB Saunders, 1994.)
Figure 58-33 Repair of Ebstein’s malformation using the Carpentier method. A, The anterior and posterior leaflets of the tricuspid valve are detached from the annulus. B, The atrium
is plicated, reducing the annular diameter. The detached leaflets are reattached to the annulus. (From Ebstein’s anomaly. In Castaneda AR, Jonas RA, Mayer JE, Hanley FL [eds]:
Cardiac Surgery of the Neonate and Infant. Philadelphia, WB Saunders, 1994.)
Mitral regurgitation is a result of secondary annular dilation, congenital isolated clefts of the valve, and prolapse of the leaflets from abnormal chordae
or papillary muscle insertion.
Echocardiography is diagnostic. Intervention includes balloon valvuloplasty, particularly for selected forms of rheumatic mitral stenosis, and surgical
intervention. Intervention is timed to avoid irreversible sequelae related to either chronic volume overload or pulmonary hypertension. Surgical
intervention in children is aimed at preserving the mitral valve. Valvuloplasty techniques have a valuable place in children. Prosthetic valves are the
least desirable option. Bioprosthetic or tissue valves should be avoided in children. Supra-annular placement of the prosthesis may be necessary. Repeat
replacement is ensured.[72]
Thoracic Transplantation in Congenital Cardiac Disease
The philosophy of transplantation is to maximize survival and improve quality of life. Transplantation is in itself not curative and carries time-related
attrition rates from acute and chronic rejection, infection, and other complications associated with long-term immunosuppression. Pretransplant
evaluation and contraindications to transplant are identical to those of the adult population. Additionally, the need for providing a stable home
environment and reliable medication administration (immunosuppression) becomes crucial.
Heart Transplantation
Pediatric heart transplant candidates fall into two categories: primary or secondary cardiomyopathy and congenital heart disease not amenable to
standard surgery. In the pediatric age group, the specific risk factor for heart transplantation is an elevated PVR. There is a significantly
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increased risk of mortality when indexed PVR exceeds 6 Wood units/m2 , under which circumstances heart-lung transplantation or heterotopic
transplantation is a consideration. Approximately 10% of all patients born with congenital heart disease are unable to have a reasonable anatomic
surgical repair. In some forms of complex congenital heart disease, palliative surgery is performed as a bridge to cardiac transplantation. The prototype
of this group is HLHS, in which the Norwood procedure is currently offered in a limited number of centers, and the collective short- and long-term
results have been marginal. Transplantation has become a reasonable alternative, but lack of donor availability has resulted in attrition rates of up to
40% before transplantation. Another group are those who have undergone surgical repair but have developed an irreversible abnormality or myocardial
dysfunction. Examples in this group are patients with a failed Fontan operation and complex congenital heart disease with severe AV valve
regurgitation and RV dysfunction following the Norwood procedure or the atrial switch procedure (Mustard or Senning).
Preoperative management is aimed at optimizing the patient for transplantation. Under some circumstances and depending on the size of the child,
implantable bridge to transplant devices may be appropriate. In smaller children, particularly infants younger than 6 months of age, extracorporeal
membrane oxygenation is used as a bridge for transplantation. In this group of patients, the time constraints are imposed by the complications related to
longer duration of extracorporeal support. Anatomic considerations with regard to aortic arch hypoplasia, heterotaxy syndromes, and varying degrees of
previous palliative procedures on pulmonary artery and interatrial anatomy pose different surgical challenges for this group. In the infant group, donor
size greater than 300% of the recipient can be successfully used; in other pediatric recipients, the body weight of the donor can be 50% to 250% greater
than that of the recipient.
As a general rule, a donor weighing more than 25% less than the recipient is not acceptable. The transplanted heart grows proportionately, maintaining a
cardiac output sufficient to sustain normal growth. It is preferable to use an oversized donor heart or a donor heart with a conditioned right ventricle for
preexisting, elevated PVR. Heart transplantation is performed utilizing an orthotopic heart transplant technique or variations as imposed by the
congenital anomalies.[73]
Lung and Heart-Lung Transplantation
The cumulative experience with pediatric lung and heart-lung transplantation has increased since the mid-1990s. Potential candidates for lung
transplantation are those with end-stage pulmonary vascular disease or bronchopulmonary pathology. Bronchopulmonary pathology includes those
patients with cystic fibrosis and severe bronchopulmonary dysplasia. Patients with repairable congenital heart lesions who have Eisenmenger’s
syndrome with long-standing, irreversible cardiomyopathy are candidates for heart-lung transplantation. Lung transplantation (cadaveric and living-
related) is another option for pediatric patients.[74] Decreased right ventricular function in a setting of pulmonary hypertension is not a contraindication to
isolated lung transplantation.
Single-lung transplantation can generally be performed through a thoracotomy without CPB. Double-lung transplantation exposure is provided with a
transverse submammary, clamshell incision. A median sternotomy can also be used. Heart-lung transplantation exposure is through either a midline
sternotomy or a clamshell incision. Postoperative management in transplantation follows the principles of postcardiac surgery care. Variable organ
preservation and prolonged ischemia, together with reperfusion injury, may cause a delay in return of function of the transplanted organs.
Special aspects of post-transplant care involve the prevention of graft rejection through immunosuppression. Standard triple therapy is usually used
(corticosteroids, cyclosporine, and azathioprine). Some centers favor a corticosteroid-free regimen for chronic immunosuppression, if feasible. Newer
immunosuppressives (mycophenolate and tacrolimus [FK-506]) are also used in the pediatric group. Immunosuppression may result in infectious
complications that are often more severe and may sometimes be fatal in the pediatric population.[75]
Results
Heart transplantation for children has a 30-day perioperative mortality of 15% to 20% with 1- and 5-year actuarial survival rates of 75% to 80% and
60% to 75%, respectively. Pediatric heart and lung transplantation provides 1- and 5-year survival rates of 60% and 40%, respectively. The 24-month
survival rate for double-lung transplantation is approximately 60%.[75] The leading cause of early death in these patients is infection, whereas
bronchiolitis obliterans accounts for many late deaths in lung and heart-lung transplantation. Post-transplant lymphoproliferative disease is a malignancy
affecting approximately 10% of pediatric transplant recipients. This is associated with infection with the Ebstein-Barr virus.[76] In heart transplantation,
graft coronary artery disease is considered to be a consequence of chronic rejection. Retransplantation is the only option for patients with significant
coronary disease and myocardial dysfunction after transplantation.
Other complications include hypertension, renal failure, hyperlipidemia, and postoperative cytomegalovirus infection.

Selected References
Castaneda AR, Jonas RA, Mayer JE, Hanley FL (eds): Cardiac Surgery of the Neonate and Infant. Philadelphia, WB Saunders, 1994.
This book details an experience of the Children’s Hospital in Boston over a 20-year period. Congenital cardiac defects with special emphasis on neonatal and infant
applications are discussed in detail. The heart defects themselves are very well covered, and the strength of this particular reference is in the general considerations, which
cover in detail cardiopulmonary bypass, myocardial preservation, and perioperative management of the infant and neonate with congenital heart disease.
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Kirkland JW, Barratt-Boyes BG: Cardiac Surgery, 2nd ed. New York, Churchill Livingstone, 1993.
This remarkable book is the standard reference for cardiac surgeons. It catalogs the experiences of two well-known experts in the field of congenital heart surgery. The
material is well organized, well presented, and exquisitely illustrated. This reference provides an in-depth review of all aspects of congenital cardiac surgery.
Stark J, de Leval M: Surgery for Congenital Heart Defects, 2nd ed. Philadelphia, WB Saunders, 1994.
This book is a readable, well-illustrated, and in-depth synopsis of congenital cardiac disease. Chapters are written by experts in the various fields. A perspective on the
management of all congenital lesions is presented.

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Chapter 59 - Surgical Treatment of Coronary Artery Disease
Phillip C. Camp Jr. M.D.

Robert M. Mentzer Jr. M.D.
CORONARY ARTERY ANATOMY
The coronary arteries originate at the root of the aorta, behind the left and right cusps of the aortic valve. They provide the blood supply to the
myocardium via the main epicardial conductance vessels and enter the myocardium by penetrating vessels called resistance arteries. These vessels then
branch into a plexus of capillaries that are essentially contiguous with every myocyte (intercapillary distance at rest is 17 µm). The left main coronary
artery (LMCA) rises from the left coronary sinus; it averages 2 cm in length, and varies from 1 to 4 cm. After coursing between the pulmonary artery
and the left atrial appendage, it bifurcates into two major branches, the left anterior descending coronary artery (LAD) and the left circumflex coronary
artery (LCA). In many instances, the vessel trifurcates; this occurs when the ramus medianus vessel originates between the anterior descending and the
circumflex arteries. Occasionally, the LMCA is absent, and the LAD and LCA arise from common or separate ostia. Less commonly, a single coronary
vessel arises from a common orifice and provides all cardiac blood flow ( Fig. 59–1 ).
In general, the LAD supplies the anterior and left lateral portions of the left ventricle. The LAD proceeds distally behind the pulmonary trunk into the
anterior intraventricular sulcus and provides a number of anterior perforating branches to the anterior interventricular septum. In most cases, the LAD
wraps around the apex of the heart and forms an anastomosis with the posterior descending coronary artery (PDA), a branch of the right coronary artery
(RCA). As the LAD follows the interventricular groove, it may give rise to one or more branches that course diagonally over the left anterior ventricular
free wall. The first diagonal branch and the first septal perforator are usually the largest vessels arising from the LAD, and both the septals and the
diagonals become smaller as the vessel progresses distally.
The LCA originates from the LMCA and follows a course posteriorly under the left atrial appendage and along the left atrioventricular (AV) groove. In
most cases, the circumflex terminates as an obtuse marginal branch. It can, however, be the primary source of blood flow to the PDA. One to four
obtuse marginal branches of varying size emerge from the main circumflex artery and course along the lateral and posterolateral aspects of the left
ventricle. The branches that arise most distally are often referred to as posterolateral branches of the circumflex artery. These branches course parallel
to the PDA but provide no perforating branches into the intraventricular septum. In 10% of patients, the circumflex artery supplies the posterior
descending and the AV nodal arteries as it courses along the posterior intraventricular sulcus. This
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Figure 59-1 Left main coronary artery (A), left anterior descending coronary artery (B), left circumflex coronary artery (C); and obtuse marginal vessels (D). (Courtesy of David
Booth, MD, Division of Cardiology, University of Kentucky, 2003.)
pattern of circulation is referred to as a left dominant circulation (see Fig. 59–1 ).
The RCA supplies most of the right ventricle, as well as the posterior part of the left ventricle. The RCA emerges from its ostium in the right coronary
sinus, passes deep in the right AV groove, and then proceeds to course over the anterior surface of the heart. At the superior end of the acute margin of
the heart, the RCA turns posteriorly toward the crux and usually bifurcates into the PDA and the right posterolateral artery. The RCA also supplies
multiple right ventricular branches (acute marginals) as wells as branches to the AV node, although the latter may also arise from the left circumflex
artery. In approximately 90% of patients, the RCA passes through the AV sulcus to the posterior interventricular sulcus and becomes the PDA. This
pattern of circulation is referred to as a right dominant system. Occasionally, the PDA arises from both the RCA and the LCA, and the circulation is
considered to be codominant ( Fig. 59–2 ). The sinoatrial node artery arises from the proximal RCA in 50% of patients, and many other small atrial
branches arise from the RCA, but they are rarely of significance. Other prominent branches arising from the RCA include the acute marginal artery and
anterior ventricular branches. Although the source of the PDA is often used clinically to define dominance of circulation in the heart, anatomists define
it based on where the sinoatrial node artery arises. In 90% of patients, the RCA bifurcates into the posterior descending and the right posterolateral
arteries. The AV node artery arises from the RCA in approximately 90% of patients.
Figure 59-2 Right coronary artery (A) and posterior descending artery (B). (Courtesy of David Booth, MD, Division of Cardiology, University of Kentucky, 2003.)
The incidence of coronary artery anomalies is approximately 1%, and these congenital anomalies may or may not be clinically significant.
Hemodynamically significant anomalies include coronary fistulas or origin of the coronary artery from the pulmonary artery. Both may result in
abnormal coronary perfusion. The most common congenital variation encountered during angiography is the origin of the circumflex artery from the
RCA or the right coronary sinus, which occurs in approximately 0.5% of patients. Anomalous origin of the anterior descending artery from the right
sinus of Valsalva or from the RCA is another common anomaly and is associated with tetralogy of Fallot.
A network of veins drains the coronary circulation, and the venous circulation can be divided into three systems: the coronary sinus and its tributaries,
the anterior right ventricular veins, and the thebesian veins. Occlusive disease is uncommon in the venous system.
The coronary sinus predominantly drains the left ventricle and receives 85% of coronary venous blood. It lies within the posterior AV groove and
empties into the right atrium. The anterior right ventricular veins travel across the right ventricular surface to the right AV groove, where they enter
directly into the right atrium or form the small cardiac vein, which enters into the right atrium directly or joins the coronary sinus just proximal to its
orifice. The thebesian veins are small venous tributaries that drain directly into the cardiac chambers and exit primarily into the right atrium and right
ventricle.

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CORONARY CIRCULATION AND REGULATION OF BLOOD FLOW
Coronary Blood Flow
The coronary arteries deliver oxygen and other metabolic substrates to the myocardium and simultaneously remove carbon dioxide and metabolic
degradation products through the process of transcapillary exchange. Relative to most other organ systems, the myocardium has a high rate of energy
utilization. Normal coronary blood flow averages 225 mL/min or 0.7 to 0.9 mL/g of myocardium per minute and delivers 0.1 mL of oxygen per gram
per minute to the myocardium. Oxygen extraction in the coronary capillary bed averages 75% under normal conditions and has the capacity to increase
to 100% during stress. In response to strenuous exercise, the healthy heart can increase myocardial blood flow fourfold to sevenfold.
Factors Influencing Coronary Vascular Resistance
Metabolic
Local myocardial metabolism is the primary regulator of coronary blood flow ( Fig. 59–3 ). There is a strong correlation between myocardial metabolic
activity and the magnitude of coronary blood flow changes ( Fig. 59–4 ). The mechanism by which increased myocardial metabolism promotes
coronary blood flow has not yet been clearly elucidated. It is hypothesized that the decrease in oxygen supply to oxygen demand triggers release of a
vasodilator substance from the myocardium, which in turn, initiates relaxation of the coronary resistance vessels. This results in increased delivery of
oxygen-rich blood. An example of this is the phenomenon of reactive hyperemia. When blood flow is transiently stopped by the occlusion of a vessel in
the beating heart, blood flow immediately exceeds the normal baseline flow when the occlusion is removed. Blood flow returns to the baseline level
over a period of time proportional to the duration of the occlusion. Several metabolic factors that have been implicated as the mediator of reactive
hyperemia include CO2 , decreased O2 tension, hydrogen ions, lactate, potassium ions, and adenosine. Of these, adenosine is one of the strongest
candidates. In the setting of ischemia or increased metabolic activity, adenosine, a potent vasodilator and degradation product of adenosine triphosphate,
is produced, accumulates in the interstitial space, and releases the vascular smooth muscle. This results in vasomotor relaxation, coronary vasodilation,
and increased blood flow. Although adenosine is a leading candidate, it may be only part of the process, since adenosine receptor antagonists do not
completely block reactive hyperemia. Another factor that may play an important role is nitric oxide (NO). In the absence of the endothelium, a source of
NO production, coronary arteries do not autoregulate.
Physical
Aortic pressure is a key factor responsible for myocardial perfusion. The coronary vasculature can compensate and maintain normal coronary perfusion
pressures between systolic pressures of 60 and 180 mm Hg via the process of autoregulation. This is a process whereby baroreceptors promote local
vasodilation or vasoconstriction through alterations in coronary diameter, so that coronary blood flow is maintained at a constant level. Extravascular
compression of the coronaries during systole is another factor that plays an important role in the regulation of blood flow. During systole, the
intracavitary pressures generated within the left ventricular wall exceeds intracoronary pressure and nutrient flow is impeded. This may result in the
transient reversal in the direction of blood flow in the epicardial vessels (see Fig. 59–3 ). The heart rate
Figure 59-3 Schematic representation of factors that increase (+) or decrease (–) coronary vascular resistance. (From Berne RM, Levy MN [eds]: Physiology, 4th ed. St. Louis, Mosby,
1998, p 483.)
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Figure 59-4 Relationship between myocardial oxygen consumption and coronary blood flow during a variety of interventions that increased or decreased myocardial metabolic rate.
(From Berne RM, Levy MN [eds]: Physiology, 4th ed. St. Louis, Mosby, 1998, p 482.)
also affects coronary artery blood flow. Tachycardia increases the proportion of the cardiac cycle in systole and results in the restriction of blood flow.
In general, this is compensated by coronary vasodilation that occurs as a result of an increase in metabolic activity. Bradycardia prolongs diastole, and
thus coronary flow and nutrient delivery is increased (see Fig. 59–3 ).
Neural and Neurohumoral
Stimulation of the cardiac sympathetic nerves indirectly increases coronary blood flow as a result of increased metabolic activity secondary to
augmented myocardial contractility and tachycardia. Although α- and β-adrenergic receptors do exist in coronary vessels, the α receptors are more
prominent in the epicardial vessels, and the β receptors are more prominent in the intramuscular vessels. Although both vasodilation and
vasoconstriction can occur with activation of the receptors, these effects play a less important role than metabolic factors (see Fig. 59–3 ).
Parasympathetic stimulation has only a slight vasodilatory effect on the coronary arteries and is not a significant contributor to the regulation of normal
coronary blood flow.

MECHANICS OF PUMP FUNCTION
In the normal heart, an increase in intraventricular volume during diastole leads to an increase in the force of contraction. The association between end-
diastolic volume and systolic pressure, known as the Frank-Starling relationship, is under the influences of hormonal and neuronal stimulation. For
example, an increase in circulating catecholamines may result in more forceful contractions (inotropy), a rapid heartbeat (chronotropy), and more
efficient relaxation (lusitropy). Ventricular performance is also determined, in part, by changes in preload and afterload. Preload varies as a result of
changes in intravascular volume caused by alterations in systemic venous capacitance, pulmonary vascular capacitance, and ventricular compliance.
Preload is a term that describes the intraventricular pressure immediately prior to contraction and is commonly referred to as the filling pressure.
Afterload refers to the amount of pressure developed during ventricular systole that is required to eject blood against the pressure of the receiving vessel,
the aorta, or pulmonary artery. The greater the afterload is, the greater the energy requirements and consumption of oxygen. Afterload is commonly
measured by dividing the difference between the mean aortic pressure and central venous pressure by the cardiac output. The compliance of the
ventricle is determined by changes in volume and pressure. The right ventricle is more compliant than the left ventricle and as a consequence may serve
as a volume reservoir.
An understanding of the mechanics of normal and abnormal ventricular contraction is facilitated by a graphic depiction of the relationship between
ventricular pressure and volume during a single cardiac cycle. This depiction, called a pressure-volume loop, is shown for a normal heart in Figure 59–
5 . Diastolic filling begins at point A and continues to point C. During the initial rapid filling from the atria, there is a slight fall in ventricular pressure,
which demarks progressive ventricular relaxation. Once active relaxation of the ventricle ceases, point B, the pressure slightly increases as a result of
passive ventricular filling.
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Figure 59-5 Pressure-volume loop during a single cardiac cycle. A variety of pathologic conditions bring about a shift in the pressure-volume loop to quadrants, here identified as
quadrants I to V. A to C, diastolic filling; C to D, isovolumetric contraction; D to F, ventricular ejection; F to A, isovolumetric relaxation. (Courtesy of the Division of Cardiothoracic
Surgery, University of Kentucky, 2003.)
Just prior to completion of filling, point C, an atrial contraction adds additional volume, the atrial kick. During isovolumetric contraction, from point C
to D, the ventricular pressure rapidly increases with no change in ventricular volume. When the pressure in the ventricle exceeds the pressure in the
aorta, the aortic valve opens, point D, and the rapid phase of ejection begins. Between point D and E, ventricular pressure increases while ventricular
volume declines. Between points E and F, both pressure and volume decline until ejection is complete and the aortic valve closes, point F. Once the
aortic valve is closed, a phase of isovolumetric relaxation occurs, point F to A, which is characterized by a rapid fall in pressure with no change in
ventricular volume. When the ventricular pressure falls below the atrial pressure, the mitral valve opens, point A, and ventricular filling begins
completing the cardiac cycle.
Pressure-volume loops are useful in understanding various physiologic and pathophysiologic conditions. The shape of the pressure-volume loop during
systole is determined by the contractility of the heart and the afterload against which the ventricle is ejecting. In the setting of adrenergic stimulation, the
pressure-volume loop is shifted to the left (see Fig. 59–5 , quadrant II). Assuming all other variables remain constant, the positive inotropic and
lusitropic effects of adrenergic stimulation promote an improved ejection fraction (EF) that is associated with a lower end-diastolic filling pressure. An
increase in afterload, as might be seen with hypertension or with aortic valve stenosis, is associated with a shift of the loop upward and to the right (see
Fig. 59–5 , quadrant III). If the stroke volume remains constant, the EF decreases slightly. In the setting of myocardial ischemia, there is a reduction in
myocardial contractility and ventricular compliance. If the stroke volume is maintained, the pressure-volume loop shifts to the right (see Fig. 59–5 ,
quadrant IV). This results in an acute decline in the EF and an increase in filling pressure. Myocardial fibrosis secondary to chronic ischemia and
infarction can lead to decreased ventricular compliance. If systolic function is preserved, the stroke volume can be maintained if the filling pressures are
increased. In this setting the pressure-volume loop is shifted upward. As ventricular function begins to deteriorate and the ventricle dilates, the pressure-
volume loop also shifts to the right and higher filling pressures are required to maintain cardiac output (see Fig. 59–5 , quadrant IV).

CORONARY ARTERY DISEASE
Pathogenesis
Coronary artery atherosclerosis is a progressive disease that begins early in life. Epicardial vessels are the most susceptible, intramyocardial arteries the
least. Initially, the internal elastic membrane undergoes rupture, degeneration, and regeneration. This is accompanied by a deposition of
mucopolysaccharides and proliferation of endothelial cells and fibroblasts. Later, growth lesions appear in the form of small deposits of lipoid material
visible beneath the intima. This ultimately progresses to plaque formation and obstruction of the arterial lumen. In the final stages of the disease,
patients become symptomatic or die from a myocardial infarct as a result of marked narrowing or closure of the vessel lumen, plaque rupture, or
coronary artery thrombosis.
Role of Inflammation
Although several mechanisms and many risk factors for disease development have been implicated, it appears that the primary causes of atherosclerotic
coronary artery disease (CAD) are endothelial injury induced by an inflammatory wall response and lipid deposition. There is evidence that an
inflammatory response is involved in all stages of the disease, from early lipid deposition to plaque formation, plaque rupture, and coronary artery
thrombosis. Early after initiation of an atherogenic diet in animals, endothelial cells begin to express selected adhesion molecules, such as the vascular
cell adhesion molecule-1, that bind various classes of leukocytes, monocytes, and T lymphocytes. Once the leukocytes adhere to the endothelium,
chemoattractant molecules promote transmigration, and they penetrate the intima where they participate in and perpetuate a local inflammatory
response. The monocytes express scavenger receptors for modified lipoproteins, which allow them to ingest lipids. These modified
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lipids induce the expression of numerous adhesion molecules, chemokines, proinflammatory cytokines, and other mediators of inflammation in
macrophages and vascular wall cells. The activated macrophages also release mitogens and chemoattractants, such as macrophage colony–stimulating
factor, and monocyte chemoattractant protein-1. These molecules promote and perpetuate ongoing mobilization of monocytes into the evolving plaque.
Tissue signals likewise stimulate T cells to elaborate inflammatory cytokines, such as interferon-γ and tumor necrosis factor-β, which further stimulate
the inflammatory process.[1] Activated leukocytes also release fibrogenic mediators, which promote elaboration by local cells of a dense extracellular
matrix. In addition to promoting the initiation of the atheroma, the inflammation precipitates the evolution of acute thrombotic complications. For
instance, activated macrophages secrete proteolytic enzymes that degrade the collagen that lends strength to the plaque’s protective fibrous cap. This in
turn renders the cap thin, weak and prone to rupture.
Plaque Rupture
Several studies have shown that 70% to 80% of coronary thrombi occur where the fibrous cap of an atherosclerotic plaque has fissured or ruptured.
Subsequent extension of the thrombus into the plaque with propagation of the thrombus downstream leads to an acute coronary event. According to the
current paradigm, rupture of the fibrous cap leads to exposure of thrombogenic components of the plaque with subsequent activation of the platelets and
coagulation pathways that result in thrombus formation and acute luminal compromise ( Figs. 59–6 and 59–7 ).[2] Although the exact mechanism
responsible for plaque rupture is unknown, the five features of vulnerable or high-risk plaques that are disruption prone are (1) a large, eccentric, soft
lipid core; (2) a thin, fibrous cap; (3) inflammation within the cap and adventitia; (4) increased plaque neovascularity; and (5) evidence of outward or
positive vessel remodeling. Thinner fibrous caps are at a higher risk for rupture. This is probably due to an imbalance between the synthesis and
degradation of the extracellular matrix in the fibrous cap that results in an overall decrease in the collagen and matrix components ( Fig. 59–8 ).
Increased matrix breakdown may be due to matrix degradation as a result of metalloproteinase expressed by inflammatory cells within the plaque.
Reduced production of extracellular matrix is likely to contribute as well. Although plaque rupture can lead to thrombosis and manifest as an acute
coronary syndrome (ACS), not all ruptures are symptomatic. The thrombotic response to plaque rupture is likely regulated by the thrombogenicity of
the plaque’s components. Tissue factor, secreted by activate macrophages and found in high concentrations within the lipid core of the plaque, is one of
the most potent thrombogenic stimuli in both the intrinsic and extrinsic pathways. Spontaneous rupture of a vulnerable plaque may occur spontaneously
or as a result of specific triggers such as extreme physical activity, severe emotional distress, exposure to drugs, cold exposure, and acute infections.
There are also circadian components to the onset of ACSs.[3] [4] [5]
Lipid Metabolism
Epidemiologic evidence suggests that coronary artery atherosclerosis is closely linked to lipid metabolism, specifically cholesterol. Numerous studies
have demonstrated that hydroxymethylglutaryl coenzyme-A reductase inhibitor (statin) therapy, aimed at lowering lipids, has resulted in a significant
reduction in mortality.[6] In one observational study of patients who received statin therapy and were known to have CAD, statin treatment was
associated with improved survival in all age groups.[7]
Figure 59-6 Anatomy of the atherosclerotic plaque. After the leukocytes have accumulated in the lesion, they often undergo death, sometimes by apoptosis, which can lead to a lipid
core covered by a fibrous cap. (From Lipids Online—www.lipidsonline.org.)
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Figure 59-7 Thrombosis of a disrupted atheroma: weakening of the fibrous cap. Most coronary syndromes are caused by thrombosis of a disrupted atheroma, which can result from
weakening of the fibrous cap and enhanced thrombogenicity of the lipid core. (Courtesy of Michael J. Davies, MD. Source: Lipids Online—www.lipidsonline.org.)
There was a 30% adjusted risk reduction in mortality for those younger than 65 years of age, 44% for patients aged 65 to 79, and a 50% reduction in
patients who were older than 79 years old. The greatest survival benefit was derived in those patients with the highest quartile of high-sensitivity C-
reactive protein (hs-CRP), a biomarker of inflammation and CAD.[8] Animal and human studies have demonstrated that statin therapy also modifies the
lipid composition within plaques by lowering the amount of low-density lipoprotein (LDL) cholesterol and by stabilizing the plaque through a variety of
mechanisms, including reduction in macrophage accumulation, collagen degradation, reduction in smooth muscle cell protease expression, and decrease
in tissue factor expression.[9] Thus, more aggressive statin use after coronary artery bypass grafting (CABG) surgery may be indicated.
Fixed Coronary Obstructions
More than 90% of patients with symptomatic ischemic heart disease have advanced coronary atherosclerosis due to a fixed obstruction. Atherosclerotic
plaques of the coronary arteries are either concentric (25%) or eccentric (75%). Eccentric lesions compromise only a portion of the lumen and, through
vascular remodeling, the arterial lumen may remain patent until late in the disease process. The impact of an arterial stenosis on coronary blood flow
can be appreciated in the context of Poiseuille’s law. The volume of a homogeneous fluid passing per unit time through a tube is directly proportional to
the pressure difference between its ends and to the fourth power of its internal radius, and inversely proportional to its length and to the viscosity of the
fluid. Clinically, reductions in luminal diameter up to 60% have minimal impact on flow. Once the cross-sectional area of the vessel decreases by 75%
or more, however, coronary blood flow is significantly compromised. Clinically, this often coincides with the onset of exertional angina. With a 90%
reduction in luminal diameter, resistance is 256 times greater than a 60% stenosis, and coronary flow may be inadequate at rest.

ISCHEMIA AND MYOCARDIAL CELL INJURY
Myocardial ischemia can lead to reversible and/or irreversible injury. Ischemia of 15 to 20 minutes’ duration is associated with postischemic myocardial
dysfunction that lasts from hours to days despite the restoration of normal coronary blood flow. This reversible injury is referred to as myocardial
stunning. The mechanisms underlying stunning are complex but in general appear to be related to intracellular calcium overload and oxidative stress
induced by reactive oxygen species (ROS) released at the time of reperfusion. Although intracellular calcium may
Figure 59-8 Matrix metabolism and integrity of the plaque’s fibrous cap. This figure depicts the current understanding of the dynamics of the plaque’s stability and thrombogenicity.
The inflammatory cells can send molecular messages to the smooth muscle cells (interferon-γ) that inhibit the ability of this cell type to synthesize new collagen to strengthen the
plaque’s fibrous cap. In addition, the inflammatory cells can release proteolytic enzymes capable of degrading collagen and other structurally important constituents of the plaque’s
fibrous cap. Thus, when there is inflammation in the intima, the collagen responsible for the integrity of the plaque’s fibrous cap is under double attack, subject to both decreased
synthesis and increased degradation. This sets the stage for plaque disruption. The inflammatory cells also are responsible for signaling and producing increased quantities of tissue
factor, a potent procoagulant deemed responsible for thrombosis of ruptured plaques. (From Libby P: Molecular bases of the acute coronary syndromes. Circulation 91:2844–2850,
1995. Source: Lipids Online—www.lipidsonline.org.)
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return to normal levels early during reperfusion, transient increases can activate a variety of proteases, including protein kinase C, whose activation and
subsequent action on contractile proteins can lead to myofibrillar injury. The incidence of myocardial stunning in patients after CABG surgery ranges
from 20% to 80%, depending on its definition and/or clinical manifestation. For example, as many as 75% of patients after CABG surgery may be
administered some form of inotropic support in the immediate postoperative period to maintain a satisfactory cardiac index, mixed venous saturation,
blood pressure, and/or urinary output. In most patients, this is not associated with specific adverse outcomes. In the patient with severe preoperative
myocardial dysfunction and limited cardiac reserve, however, myocardial stunning may result in a more profound reduction in cardiac output. These
patients may require intensive inotropic support postoperatively and insertion of an intra-aortic balloon pump (IABP). In these patients the mortality rate
secondary to stunning may be as high as 10% to 15%.
Reversible contractile dysfunction that matches a reduction in resting coronary artery blood flow is termed hibernating myocardium. It is characterized
by a balanced reduction in myocardial contractility and oxygen consumption and is typically found in patients with severe CAD who present with stable
or unstable angina (UA), myocardial infarction, or congestive heart failure. By definition, it is reversible on restoration of normal coronary blood flow.
Human tissue biopsies obtained from hibernating myocardium have shown a loss of myofibrils, glycogen accumulation, and interstitial fibrosis.
Whether the contractile dysfunction in the hibernating myocardium is due to a reduction in coronary blood flow or a reduction in coronary reserve is
unclear. It is also controversial whether chronic contractile dysfunction is caused by repetitive episodes of myocardial stunning or is an adaptive
mechanism to chronic myocardial ischemia. Characteristics that differentiate these two phenomena are summarized in Table 59–1 .
Myocardial infarction represents cell death and necrosis. It is an irreversible injury that is associated with ischemia lasting more than 20 minutes. Since
a gradient of ischemia may exist within the myocardium, not all cells are equally at risk for injury. Cells around the periphery of an ischemic zone and
adjacent to the oxygen-rich blood in the ventricular chamber may remain viable. In the absence of adequate collateral flow, sustained ischemia often
results in a transmural infarction within 6 to 12 hours. Cell death leads to an inflammatory process that involves the migration of polymorphonuclear
leukocytes into the ischemic area and removal of necrotic tissue by macrophages over days. This is followed by a fibroblastic response and
neovascularization. Because the myocytes are incapable of regeneration, the infarcted tissue is ultimately replaced with noncontractile fibrous tissue.
Another cause of cardiomyocyte cell loss associated with ischemia is apoptosis, or programmed cell death. This phenomenon is a noninflammatory
process that occurs as a result of reperfusion and may be associated with early and delayed cardiac muscle dysfunction. Some of the proposed
mechanisms underlying apoptosis are the same mechanisms proposed for myocardial stunning, hibernation, and infarction, namely, generation of
intracellular ROS, and/or intracellular calcium overload. Apoptosis has been observed in humans with hibernating myocardium, acute myocardial
infarction (AMI), and
TABLE 59-1 -- Characteristics of Reversible Postischemic Myocardial Dysfunction
Stunning Hibernation
Dysfunctional myocardium with normal or near-normal blood flow Dysfunctional myocardium with reduced blood flow
Contractile abnormality reversible with time Contractile abnormality reversible on reperfusion
Absence of irreversible damage Absence of irreversible damage
Perfusion imaging (PET scan) normal or increased Perfusion imaging (PET scan) increased
Contractile function decreased Contractile function decreased
No metabolic deterioration during inotropic stimulation Recruitment of inotropic reserve at the expense of metabolic recovery
Disruption of myofibrillar structure in canine model Disruption of myofibrillar structure in canine model
Heart does not adapt to chronic underperfusion Heart adapts to chronic underperfusion
Steady-state between perfusion and contraction not achieved Steady-state between perfusion and contraction can be reached
Perfusion-contraction mismatching lasts from hours to days to months Perfusion-contraction matching can be maintained for prolonged periods
Lack of evidence for dedifferentiation process in myocytes Evidence for dedifferentiation process in myocytes
PET, positron-emission tomography.
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chronic heart failure. The characteristics of apoptotic death are morphologically and biochemically different from cell death secondary to cell necrosis.
With necrosis, cell death is associated with swelling and rupture of the sarcolemmal, mitochondrial, and nuclear membranes, and nuclear chromatin
clumping, and the dead cells are removed via an inflammatory process. In contrast, the apoptotic cell shrinks, the nucleus condenses and breaks into
nucleosomes and DNA fragments and the cells are phagocytized. There is some evidence that not all apoptotic cells are committed to cell death in the
early stages of the process. Apoptosis represents one aspect of a continuum of ischemia. Its contribution to myocardial dysfunction after ischemia may
be more relevant to the process of postischemic ventricular remodeling.
Finally, there is preclinical and circumstantial evidence in humans that an ischemic adaptive phenomenon exists in the human heart. This phenomenon
is known as ischemic preconditioning (IPC). It is associated with a reduction in infarct size, apoptosis, and reperfusionassociated arrhythmias. IPC
occurs when the heart is exposed to brief periods of sublethal ischemia prior to a period of prolonged ischemia. The underlying mechanism(s) is unclear
but most likely involves activation of cell surface receptors and intracellular transduction signaling pathways. If the phenomenon can be mimicked
pharmacologically, this could lead to the development of pharmacologic agents that are effective in increasing the heart’s tolerance to ischemia.

CLINICAL MANIFESTATIONS AND DIAGNOSIS OF CORONARY ARTERY DISEASE
Clinical Presentation
One of the most typical manifestations of CAD is angina pectoris, a discomfort or sensation of heaviness, tightening, squeezing, or constricting in the
chest. This discomfort is often retrosternal or left precordial and may radiate from the chest. It is also characterized as discomfort in the jaw, shoulder,
back, or arm. Patients often experience a disagreeable feeling similar to that of indigestion and/or experience shortness of breath. It often presents while
the patient is exercising, eating, or under emotional duress and usually subsides with rest. Patients with UA typically have pain at rest or with minimal
exertion that lasts more than 20 minutes. It is often severe in nature, new in onset (within 1 month), and occurs in a crescendo pattern. Angina is not,
however, always present with myocardial ischemia. As many as 15% of patients with significant CAD do not present with angina. This silent ischemia
occurs most frequently in patients with diabetes mellitus and is detected during electrocardiographic (ECG) and/or echocardiographic monitoring during
stress testing. In patients at risk for the disease, angina pectoris is graded according to a variety of classification systems, such as the New York Heart
Association Functional Classification, and Canadian Cardiovascular Society Classification System (CCSCS). The latter is more specific for patients
with angina pectoris. Using the CCSCS schema, class I patients are asymptomatic, class II patients experience slight limitation of ordinary activity,
class III patients experience marked limitation of activity with ordinary physical activity, and class IV patients are unable to undertake any activity
without discomfort. Classifications allow for the evaluation of the patient’s condition followed over time and the assessment of therapeutic
interventions.
In contrast to angina pectoris, a myocardial infarction often presents as crushing chest pain that may be associated with nausea, diaphoresis, anxiety, and
dyspnea. Symptoms also include dizziness, fatigue, and vomiting. The pain or associated paresthesias often radiate to the neck and/or jaw and down the
arm. Heart rate and blood pressure may be initially normal, but both increase in response to the duration and severity of pain.
Physical Examination
It is possible for a patient to have extensive CAD and the physical examination to be unremarkable. Pertinent physical findings are more frequently
associated with manifestations of atherosclerosis in general. The patient’s mental status can vary from normal, to anxious, to confused. Eye examination
may reveal a copper-wire sign, retinal hematoma or thrombosis secondary to vascular occlusive disease, and hypertension. The presence of neck bruits
and thrills may reflect significant underlying carotid artery disease. Abnormal neck vein pulsations may be seen in patients with second- or third-degree
heart block. The pulse may be weak or thready and suggest ectopic or premature ventricular beats. Often a precordial ectopic impulse may be palpated
along the left lower sternal border, demarking enlargement of the left ventricle due to increase chamber compliance and bulging. A third heart sound
can be noted with elevated left ventricular filling pressures. A fourth heart sound is commonly heard in patients with acute and chronic CAD, and heart
murmurs may reflect ischemic papillary muscles and mitral valve insufficiency, aortic stenosis/insufficiency, and ventricular septal rupture. In patients
with more advanced ischemic heart disease, auscultation of the chest may reveal rales, and examination of the abdomen may reveal hepatomegaly, right
upper abdominal quadrant tenderness, ascites, and marked peripheral and presacral edema.
Laboratory Studies
Patients suspected of having CAD should undergo appropriate blood testing, including a lipid profile (cholesterol, triglycerides, LDL, high-density
lipoprotein [HDL]) and perhaps an hs-CRP level. Elevated serum cholesterol level is associated with an elevated risk of coronary heart disease. A 10%
increase in serum cholesterol is associated with a 20% to 30% increase in heart disease. Clear benefits have been shown for dietary and drug regimens
that lower serum cholesterol, and statins have been shown to reduce fatal and nonfatal coronary heart disease and slow the progression of bypass graft
plaque progression.
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There are also several markers of inflammation that are useful at predicting the development of coronary heart disease. These include hs-CRP, the
adhesion molecule ICAM-1, and cytokines such as interleukin (IL)-6 and tumor necrosis factor. hs-CRP adds to the predictive value of total and HDL
cholesterol in determining risk of future myocardial infarction. Whether these markers can be used to better identify patients at risk and target
therapeutic intervention remains to be determined.
Diagnostic Studies
There are numerous methods and technologies that are available to detect the presence of hemodynamically significant coronary artery stenoses and to
assess cardiac function and myocardial viability. The information ultimately determines whether a patient should be treated medically, with
percutaneous transluminal coronary angioplasty (PTCA), percutaneous coronary intervention (PCI), or with CABG surgery ( Fig. 59–9 ). Many of these
studies can be performed even if the patient is unable to exercise. The strengths and weaknesses of each method are shown in Box 59–1 .
Chest Radiograph
The chest radiograph is helpful in identifying causes of chest discomfort or pain other than that due to CAD. With
Figure 59-9 A strategy for evaluating patients with suspected coronary artery disease (CAD) and unable to exercise. CABG, coronary artery bypass graft. (Courtesy of the Division of
Cardiothoracic Surgery, University of Kentucky, 2003.)
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Box 59-1. Strengths and Limitations of Diagnostic Techniques *
DETECTING CORONARY ARTERY DISEASE AND ASSESSING

PROGNOSIS
Exercise ECG
Strengths: Low cost; short duration; functional status evaluated; high

sensitivity in three-vessel or left main coronary artery disease; prognostic
(e.g., ischemia at low workload)
Limitations: Suboptimal sensitivity; low detection rate of one-vessel

disease; nondiagnostic with abnormal baseline ECG; poor specificity in
premenopausal women; must achieve ≥ 85% of maximum heart rate for
accuracy
Exercise/Pharmacologic SPECT Perfusion Imaging
Strengths: Simultaneous evaluation of perfusion and function (gated

SPECT); higher sensitivity and specificity than exercise ECG; high
specificity with 99m Tc; can be performed in most patients; added
prognostic value; comparable accuracy with pharmacologic stress;
viability and ischemia simultaneously assessed; quantitative image
analysis
Limitations: Suboptimal specificity with 201 Tl; long procedure time with
99m Tc; higher cost than exercise ECG; radiation exposure; poor-quality
images in obese patients
Exercise/Pharmacologic Stress Echocardiography
Strengths: Higher sensitivity and specificity than exercise ECG; added

prognostic value; comparable value with dobutamine stress; short
examination time; identification of structural cardiac abnormalities;
simultaneous evaluation of perfusion with contrast agents; relatively
lower cost; no radiation
Limitations: Decreased sensitivity for detection of one-vessel disease or

mild stenosis with postexercise imaging; inability to image all of the left
ventricle in some patients; highly operator dependent; no quantitative
image analysis; poor acoustic window in some patients (e.g., chronic
obstructive lung disease); infarct zone ischemia less well detected
ASSESSMENT OF MYOCARDIAL VIABILITY
SPECT Imaging
Strengths: High sensitivity for predicting improved function after

revascularization; quantitative objective criteria (e.g., >60% segmental
uptake); LVEF quantitated on 99m Tcsestamibi or 99m Tc-tetrofosmin
imaging; predictive of clinical outcomes
Limitations: Reduced resolution and sensitivity compared to PET; less

quantitative than PET; areas of attenuation (e.g., inferior wall on 99m Tc-
sestamibi scans) misconstrued as nonviability; cannot differentiate
endocardial from epicardial viability; no absolute measurement of blood
flow; lower specificity than dobutamine echocardiography for predicting
improved function after revascularization
PET Imaging
Strengths: Simultaneous assessment of perfusion and metabolism; more

sensitive than other techniques; good specificity; no attenuation problems;
absolute blood flow can be measured; predictive of outcomes
Limitations: Lower specificity than dobutamine echocardiography or

MRI; cannot separate endocardial from epicardial viability; high cost and
highly sophisticated technology; limited availability
Dobutamine Echocardiography
Strengths: Higher specificity than nuclear techniques; viability assessed at

low doses and ischemia at higher doses; evaluation of mitral regurgitation
on baseline echocardiography; predictive of outcomes; widely available;
lower cost than dobutamine MRI
Limitations: Poor windows in 30% of patients; lower sensitivity than

nuclear techniques; myocardium with poor flow may not show increased
during stimulation; reliance on visual assessment of wall thickening
Contrast Echocardiography
Strengths: Microcirculatory integrity evaluated as well as systolic

thickening; better estimation of extent of viability than functional
assessment alone; precise delineation of area of necrosis; resolution of
endocardial vs. epicardial perfusion; viability assessed in presence of total
coronary occlusion
Limitations: Difficult windows in 30% of patients; attenuation problems;

scant clinical data available
Dobutamine MRI
Strengths: Evaluate inotropic reserve in endocardium with tagging;

measurement of wall thickness more accurate than with TTE; better
image quality than echocardiography for contractile reserve; simultaneous
assessment of perfusion using contrast enhancement; good sensitivity and
specificity for viability
Limitations: Higher cost than echocardiography; limited availability; less

sensitive than nuclear techniques but may be more specific; imaging
information not available in real time; patients with pacemakers or
implantable cardioverter defibrillators cannot be imaged
ECG, electrocardiogram; LVEF, left ventricular ejection fraction; MRI,

magnetic resonance imaging; PET, positron-emission tomography;
SPECT, single-photon emission computed tomography; TTE,
transesophageal echocardiography.
* Adapted from Braunwald E, Zipes DP, Libby P (eds): Heart Disease: A Textbook of
Cardiovascular Medicine, 6th ed. Philadelphia, WB Saunders, 2001, pp 435, 437.
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advanced CAD there may be evidence of cardiomegaly, pulmonary edema, or pleural effusions, which are indicative of heart failure. Evidence of
calcification in the coronary arteries, aortic or mitral valves, or aorta is also consistent with presumptive evidence of generalized atherosclerosis.
Electrocardiogram
A 12-lead resting ECG should be obtained in patients suspected of having CAD and in those with episodes of chest discomfort or angina pectoris. The
ECG is evaluated for evidence of left ventricular hypertrophy, ST-segment depression or elevation, ectopic beats, or Q waves. In addition, arrhythmias
(atrial fibrillation or ventricular tachycardia) and conduction defects (left anterior fascicular block, right bundle branch block, left bundle branch block)
are suggestive of CAD and myocardial infarction. Persistent ST-segment elevation or an evolving Q wave is consistent with myocardial injury and
ongoing ischemia. Fifty percent of patients have normal ECGs despite the existence of significant CAD, and 50% of ECGs obtained during chest pain
are normal at rest. An exercise stress ECG is helpful in determining the extent of CAD and prognosis. Exercise protocols, typically using a treadmill or
bicycle, increase myocardial oxygen demand to elicit an ischemic threshold. A positive exercise ECG may show progressive flattening of the ST-
segment or ST-segment depression as exercise progresses. During the recovery phase, ST depression may persist, with down-sloping segments and T-
wave inversion. Additional findings associated with an adverse prognosis and presence of multivessel occlusive disease include duration of symptom-
limited exercise less than 6 metabolic equivalents, failure to increase systolic blood pressure higher than 120 mm Hg, or appearance of ventricular
arrhythmias. For detection of CAD, the sensitivity and specificity of an exercise ECG approaches 70% and 80%, respectively. The accuracy of exercise
ECG testing is dependent on a patient achieving 85% to 90% of their age-predicted maximum exercise (see Box 59–1 ).
Echocardiography
Surface and transesophageal echocardiography (TEE) use reflected acoustic waves for cardiac imaging. Common indications for a resting
echocardiogram include heart murmurs and suggested diagnoses such as aortic stenosis or insufficiency, hypertrophic cardiomyopathy, mitral valve
stenosis or regurgitation, and congestive heart failure. Rest echocardiography can also reveal regional wall motion abnormalities, ventricular dilation,
and wall thinning. The sensitivity and specificity of echocardiography can be enhanced with the administration of intravenous dobutamine in
incremental doses and is helpful in differentiating stunned, hibernating, and infarcted myocardium. A reduced inotropic response and evidence of new
wall motion abnormalities are also indicative of myocardial ischemia (see Box 59–1 ).
Single-Photon Emission Computed Tomographic Imaging
Exercise or pharmacologic stress 201 Tl or 99m Tc-sestamibi single-photon emission computed tomographic (SPECT) imaging has a sensitivity for
detecting CAD of 85% to 96% and, when gated with ECG, has a specificity of 90%. Compared to exercise ECG, both techniques are more accurate.
They are particularly useful in patients with left ventricular hypertrophy and/or conduction abnormalities and for patients unable to achieve 85% of their
maximum predicted exercise response. In conjunction with ECG gating, 201 Tl SPECT imaging also provides useful data on regional wall thickening,
global left ventricular EF, and myocardial perfusion. For the patient who cannot exercise, administration of vasodilators (adenosine, dipyridamole) or
inotropes (dobutamine) allows similar data acquisition with comparable sensitivity and specificity (see Fig. 59–9 ).
Positron-Emission Tomography
Positron-emission tomography (PET) scanning is a useful technique for assessing myocardial viability and metabolism and evaluating myocardial blood
flow. Since myocardial extraction of glucose is elevated in ischemic myocytes, the glucose analog radiotracer 18 F-2-fluoro-2-deoxyglucose (FDG) can
be used to image the heart; PET is reported to be superior to thallium SPECT, 99m Tc perfusion, and stress-dobutamine echocardiography in the
evaluation of myocardial viability.[10] The positive predictive value (PPV) and negative predictive value (NPV) approach 95%. Perfusion of
nonmetabolic tracers through the myocardium can identify two basic patterns: normal/uniform perfusion and underperfused myocardium. The
combination of abnormal perfusion with a positive PET study indicates viable myocardium in an area of coronary artery stenosis. In addition, FDG PET
is a highly accurate predictor of improvement in regional wall motion and global left ventricular EF after myocardial revascularization. For patients with
abnormal perfusion and greater than 75% of normal PET uptake, the PPV value of left ventricular recovery is 65% to 90%. Uptake less than 75%
generally indicates unlikely recovery of left ventricular function (NPV, 75% to 95%) after revascularization. Limitations to PET include cost,
availability of cardiac PET, and inability to interpret the study in diabetic patients who have significant insulin resistance (see Box 59–1 ).
Magnetic Resonance Imaging (MRI)—Gadolinium MRI
Due to the radiation exposure of SPECT scanning, and the limited availability of cardiac PET imaging, myocardial first-pass magnetic resonance
imaging (MRI) perfusion is a good alternative for evaluating the myocardial viability. One prospective study of 31 patients with confirmed CAD and
reduced left ventricular function (EF < 0.35) showed that MRI had a sensitivity and specificity of 86% and 94%, respectively, when PET was used as
the standard for identifying segments of myocardium with matched flow/metabolism defects.[11] Quantitative assessment of
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infarct mass also correlated well with PET. In another study, 48 patients with CAD were prospectively evaluated with gadolinium-enhanced multislice
hybrid echo-planar pulse-sequence MRI versus PET, and compared to 18 normal patients.[12] Receiver-operator characteristic analysis of CAD (as
defined by PET) had a sensitivity and specificity of 91% and 94%, respectively. Compared to quantitative coronary angiography, MRI detected lesions
greater than 50% with a specificity and sensitivity of 87% and 85%, respectively. These findings suggest that cardiac MRI is an excellent alternative
diagnostic method that can be used to determine the presence and extent of CAD and myocardial viability (see Box 59–1 ).
ECG-Gated Multidetector Spiral Computed Tomography/Electron-Beam Computed Tomography
ECG-gated multidetector spiral computed tomography (CT) is a potential tool for functional and ischemic cardiac imaging. It can identify regional
myocardial wall thinning and the presence of mural thrombus. Contrast-enhanced electron-beam CT (EBCT) is used to detect hemodynamically
significant lesions, although 30% to 40% of patients cannot be imaged due to unacceptable cardiac motion artifact. There is variation in efficacy for
specific coronary arteries, with an overall sensitivity and specificity of 90% and 80%, respectively. EBCT quantification of coronary artery calcium has
been reported to predict abnormal SPECT findings and correlate with existing asymptomatic myocardial ischemia in clinically high-risk patients. This
suggests that EBCT may be a good screening test for CAD.
Cardiac Catheterization
Anatomy of the coronary arteries, ascending aorta, aortic and mitral valves, and left ventricle can be readily evaluated at the time of left heart
catheterization. High-quality coronary angiography is essential for the identification of CAD and the assessment of its extent and severity. Cardiac
catheterization that includes ventriculogram also permits assessment of systolic and diastolic function, diagnosis of intracardiac shunts, differentiation
of myocardial restriction from pericardial constriction, and assessment of valve dysfunction. Right heart catheterization is used to measure central
venous, right atrial, right ventricular, pulmonary artery, and pulmonary wedge pressures, as well as cardiac output. It can also be used to evaluate the
presence of intracardiac shunts, assess arrhythmias, and initiate temporary cardiac pacing.

CORONARY ARTERY BYPASS SURGERY: TECHNICAL ASPECTS
Cardiopulmonary Bypass
The basic components of an extracorporeal heart pump circuit consist of one or more venous cannulae, a venous reservoir that collects blood by gravity,
an oxygenator and heat exchanger, a perfusion pump, a blood filter in the arterial line, and an arterial cannula ( Fig. 59–10 ). The cardiopulmonary
bypass (CPB) machine is constructed from a variety of biocompatible materials that can include polycarbonate, polyvinyl chloride, Teflon,
polyethylene, stainless steel, titanium, silicone rubber, and polyurethane. The blood conduits are designed to minimize turbulence, cavitation, changes in
blood flow velocity, and the volume of nonblood solutions necessary to prime the pump and tubing. The circuitry has multiple access ports or sites to
obtain blood samples for laboratory studies and the infusion of blood, blood products, crystalloids, and/or drugs.
Supplemental components include a cardiotomy suction system to collect undiluted or “clean” blood from open cardiac chambers and the surgical field.
This blood is filtered, de-aired, and returned to the bypass pump. Diluted field blood and blood that has been exposed to potentially harmful elements
(e.g., inflammatory cytokines, fat) are collected via a separate system device that concentrates washed red blood cells before returning them directly to
the patient. A cardioplegia infusion device consists of a separate pump, reservoir, and heat exchanger. It is used to deliver a cold potassium-enriched
blood or crystalloid solutions into the coronary circulation to protect the heart during ischemic arrest. Approximately 2 L of solution are required to
prime the heart pump for adults. The priming solution consists of a balanced salt solution and often a starch solution. Homologous blood is not usually
added unless the patient is anemic (i.e., the hematocrit is <25 mL/dL). Use of CPB requires suppression of the clotting cascade with heparin since the
components of the bypass pump and the surgical wound are powerful stimuli for thrombus formation. Systemic heparinization may, however, result in
increased blood loss and the requirements for homologous blood and blood product transfusions. Heparin can also induce transient hypotension as a
result of an allergic reaction.
The oxygen consumption of a patient on CPB at normal temperatures averages 80 to 125 mL/min/m2 , similar to the anesthetized adult not on bypass.[13]
Although a pump flow rate of 2.2 L/min/m2 meets the metabolic needs of most patients and avoids acidosis, a flow rate of 2.5 L/min/m2 ensures
perfusion of the microcirculation and adds a margin of safety. If hypothermia is employed, the flow rate can be reduced to less than 2.2 L/min/m2 . This
is because the mean oxygen consumption of the body decreases by 50% for every 10°C decrease in body temperature.[14] Below 28°C, a flow rate of 1.6
L/min/m2 may be safe for as long as 2 hours. Significant disadvantages of using systemic hypothermia to accommodate lower flow rates include the
extra time required to rewarm the patient and associated alterations that occur in the reactivity of blood elements, particularly platelets. The latter may
result in a greater propensity for bleeding once the patient has been rewarmed. During CPB, the systemic blood pressure is maintained by adjusting the
speed of the roller pump, manipulating the patient’s intravascular volume, and adjusting the peripheral vascular resistance by infusing vasodilators such
as nitroprusside or nitroglycerin or vasoconstrictors like ephedrine. In general, the mean normothermic blood pressure should be maintained between 50
and 70 mm Hg. The perfusion pressure may
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Figure 59-10 Components of cardiopulmonary bypass (CPB) system: A indicates the venous reservoir and blood filter; B indicates the membrane oxygenator; and C indicates the heat
exchange coil. D shows the following components: (1) CPB control console, (2) roller pump for infusing oxygenated blood, (3) cardioplegia, and (4) controlling suction catheters. E is
the cardioplegia reservoir and heat exchanger. (Courtesy of the Division of Cardiothoracic Surgery, University of Kentucky, 2003.)
be maintained 10 to 15 mm Hg higher if a patient is known to have significant obstructive intracranial or carotid artery disease.
Myocardial Protection Techniques
With the advent of CABG surgery, it was evident that some patients experienced varying degrees of myocardial injury and necrosis despite adequate
myocardial revascularization. Many of these patients died of heart failure or experienced prolonged periods of low cardiac output. Ultimately, it was
determined that this necrosis occurred as a result of ischemic damage sustained during the time of aortic cross-clamping and ischemic arrest. As a result,
a number of methodologies and techniques have evolved over the past 50 years to prevent this complication ( Table 59–2 ). The cornerstone, however,
is the use of systemic hypothermia and the infusion of cold hyperkalemic crystalloid or blood solutions directly into the proximal ascending aorta after
placement of the aortic cross-clamp.[15] The latter results in diastolic arrest of the heart, a marked reduction in myocardial oxygen consumption, and a
quiescent operative field.
Currently, there are a number of different ways to deliver cardioplegic solutions ( Table 59–3 ). One technique involves a balanced approach; that is, the
cardioplegic solution is administered first antegrade via the proximal ascending aorta and then retrograde via a coronary sinus catheter inserted through
a pursestring suture placed in the right atrium. The extensive collateralization among the coronary veins and arteries and the paucity of valves within the
coronary vein system ensures a relatively homogeneous distribution of cardioplegia when the retrograde approach is used. Patients with high-grade
proximal lesions, especially those with suboptimal collateral
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TABLE 59-2 -- Innovations in the Field of Myocardial Protection

Name Year Innovation
Bigelow WG 1950 Studied the application of hypothermia to cardiac surgery in canines
Melrose DG & Bentall HH 1955 Introduced the concept of reversible chemical cardiac arrest in canines
Lillehei CW 1956 Detailed a method for delivering hypothermic crystalloid cardioplegia by cannulating
coronary arteries
Gerbode F & Melrose DG 1958 Used potassium citrate to induce cardiac arrest in humans
Bretschneider HJ 1964 Developed a sodium-poor, calcium-free, procaine-containing solution to arrest the
heart
Sondergaard KT 1964 Adopted Bretschneider’s cardioplegic solution and was one of the first to routinely use
it for myocardial protection in clinical practice
Gay WA & Ebert PA 1973 Credited with revival of potassium-induced cardioplegia; demonstrated that potassium
solution could arrest a canine heart for 60 minutes without cellular damage
Hearse DJ 1975 Emphasized preischemic infusions to negate ischemic injuries in rats; this formula
became known as St. Thomas solution No. 1
Braimbridge MV 1975 One of the first to use St. Thomas solution No. 1 clinically
Buckberg GD 1979 Introduced the use of blood as the vehicle for infusing potassium into coronary arteries
Akins CW 1984 Utilized technique of hypothermic fibrillatory arrest for coronary revascularization
without cardioplegia
Lichenstein SV & Salemo TA 1991 Introduced warm-blood cardioplegia
TABLE 59-3 -- Methods and Delivery of Cardioplegic Solutions

Infusion Types Infusion Temperatures Infusion Intervals
Antegrade Tepid Continuous
Retrograde Warm Intermittent
Combined retrograde/antegrade Cold
vessels, may benefit from the application of both techniques. Following the initial administration of cardioplegia, additional doses are usually
administered every 15 to 20 minutes. The temperature of the myocardium can be continuously monitored with an intracardiac probe. For patients with
significant ventricular hypertrophy, and those without obvious adequate collateral vessels, shorter intervals between infusions may be required.
Conduits for Coronary Artery Bypass Grafting
The internal thoracic arteries (ITAs) (left and/or right) are the preferred conduits since their patency rates exceed 90% at 10 years.[16] The left ITA is
generally used to graft the LAD, and reversed saphenous vein segments are used to graft the remaining vessels. The right ITA pedicle can be used to
graft the RCA; if it is of sufficient length, it can be used to graft the PDA or branches of the LCA. The advantage of using these conduits must be
weighed against the potential risks in specific subsets of patients. For example, the diabetic patient may be at increased risk of infections. In these
patients bilateral ITA mobilization has been associated with a 14-fold increase in the risk of sternal wound infections. Since there is some evidence that
there may be a survival benefit associated with using only arterial grafts,[17] the radial artery is often used in conjunction with ITA grafts to revascularize
the heart. Use of arterial grafts only has the added advantages of eliminating the need for lower extremity incisions and the risk of leg wound infections.
[18] Prior to making a forearm incision, an Allen’s test is performed, and the palmar arch is evaluated using ultrasound to confirm the presence of
adequate collateral circulation in the hand. The radial artery is then procured using a no-touch technique. Prior to its removal, the adequacy of arterial
flow to the respective hand can be assessed by direct compression of the proximal end of the vessel. Systemic vasodilators, such as nitroglycerin, are
frequently used during the dissection to minimize vasospasm. The free graft is then stored in a solution containing heparin and papaverine. Another
pedicled arterial conduit that can be used is the gastroepiploic artery. This conduit is more appropriate for vessels in the inferior and lateral portions of
the left ventricle. Limitations associated with the use of this graft include its predilection for vasospasm, twisting, kinking, and vulnerability to technical
error at the anastomotic site due to its thin arterial wall. In general, the gastroepiploic artery is reserved for patients with limited conduit options.
The most commonly used conduit is the greater saphenous vein. Whether the right or left leg is chosen depends on a variety of factors such as evidence
of previous saphenous vein stripping, venous stasis disease, arterial vascular
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insufficiency, presence of nonhealing wounds, varicose veins, or history of superficial thrombophlebitis. The adequacy of the vein can be assessed
preoperatively using Doppler ultrasound. This technique can also be used to map the anatomic location of the vessel to minimize the extent of the lower
extremity incision. Techniques that are used to procure the saphenous vein include a single long incision over the vein, multiple small incisions with
bridges of intact skin, and endoscopic dissection. In general, the ideal saphenous vein should have a diameter of 3.5 mm, no varicosities, or areas of
stricture. The bridged or endoscopic technique minimizes the length of the skin incision and is associated with lower infection rates and less
postoperative pain. Stretching or manipulation of the vessel is minimized to avoid endothelial injury and thrombosis. Side branches are clipped or
ligated to avoid bleeding complications in the postoperative period. The leg incisions are closed in layers to eliminate dead space and avoid hematoma
formation and decrease the risk of infection. The vein conduit is then stored in heparinized saline or blood until it is needed. Vein graft patency rates
have been reported to be 88% early after grafting, 81% at 1 year, 75% at 5 years, and 50% at 15 years. Venous graft occlusion rate is approximately 2%
per year.[19] If the saphenous vein is inadequate or unavailable, the lesser saphenous vein can be used.
Anesthesia for Myocardial Revascularization
Major advances in cardiac anesthesia in the past 5 years primarily reflect improvements in techniques and methodologies. For example, high-dose
narcotic anesthesia, which was routinely used a decade ago, has evolved into a method of balanced anesthesia. This involves the judicious use of
shorter-acting narcotics, such as remifentanil, supplemented by safer volatile agents, such as sevoflurane, and/or short-acting intravenous agents, such as
propofol. The use of short-acting agents has resulted in less ventilatory support time, shorter intensive care unit (ICU) stay, and a decrease in hospital
length of stay. There is also an increase in the use of supplemental techniques, such as regional, epidural, and paraspinal blocks to reduce the use of
systemic agents and improve analgesic control and postoperative pulmonary function. Maintaining control of the mean arterial blood pressure to
preserve the cerebral perfusion pressure minimizes postoperative neuropsychometric and neurocognitive dysfunction. Also, real-time cerebral bispectral
index monitoring, although somewhat controversial, can be used to predict anesthetic depth and avoid excess narcotic anesthesia. Adequate oxygen
delivery is ensured by maintaining a hematocrit 25% or higher during and after CPB. Finally, there is evidence that tight glycemic control in diabetic
patients undergoing CABG surgery may improve survival and decrease recurrent ischemic events. Perioperative serum glucose levels should be
maintained between 100 and 150 mg/dL.
The use of intraoperative TEE represents another major advance in cardiac anesthesia. It is particularly useful in assessing myocardial function before
and after CPB. Perioperative myocardial ischemia manifest by new regional wall motion abnormalities can often be detected prior to ischemic changes
in the ECG and elevation in pulmonary artery pressures. TEE can also be used to determine the presence of intracavitary air and the ventricular response
to increasing intravascular volume. This information can be helpful in deciding the optimum time to wean the patient from CPB.
With off-pump CABG (OPCAB) surgery, there is, in general, a greater requirement for increased monitoring and circulatory support, particularly
during cardiac manipulation and periods of isolated coronary occlusion. This includes a more dynamic administration of inotropic and chronotropic
agents, intravascular volume loading, and the administration of antiarrhythmic medications. This has enabled myocardial revascularization to be
performed safely without CPB and is responsible, in part, for the wider application of OPCAB surgery.
The Operation
Preparation for CABG surgery includes the administration of preoperative antibiotics (e.g., cefuroxime 1.5 g intravenously or vancomycin 1 g
intravenously for a patient with penicillin allergy) at least 30 minutes prior to making the skin incision. After the appropriate hemodynamic monitoring
lines have been placed and a Foley catheter inserted, the patient is positioned in a frog-leg position and padded appropriately to minimize pressure
points. The patient is prepared and draped, and a median sternotomy incision is performed ( Fig. 59–11 ). Typically, the left half of the sternum is
retracted and elevated to expose the ITA. Once identified and pulsatility verified, the endothoracic fascia is opened medial to the artery. Minimal
traction and a no-touch technique are employed to protect the vessel. The use of a headlight and magnification provided by surgical loupes allows
precise dissection. A radiofrequency device or electrocautery can be used to mobilize the pedicle and identify the arterial and venous branches. The
advantage of a radiofrequency device is the lack of transmitted thermal energy with its associated risk of heat-induced vascular injury. Side branches are
clipped on the arterial side, and the other side is clipped or cauterized. The pedicle is mobilized from the subclavian artery and vein beneath the
manubrium to the bifurcation of the superior epigastric and musculophrenic branches distally at the level of the diaphragm. Following anticoagulation
with heparin, the ITA is divided at the distal bifurcation and flow is measured. A free flow rate greater than 60 mL/min is desirable. The vessel is then
gently occluded distally with a clamp, and the pedicle is inoculated with a stream of papaverine solution to promote arterial dilation and prevent
vascular spasm. The distal end of the vessel is prepared for grafting at this time or deferred to just prior to performing the anastomosis. If the diameter
of the ITA appears adequate but the vessel lacks adequate inflow or pulsatility, the pedicle can be used as a free graft. In this case, the pedicle is divided
at the level of the subclavian artery and stored in a papaverine solution until needed. Another surgical
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Figure 59-11 Coronary artery bypass procedures are performed through a median sternotomy (left inset). The divided sternum is lifted by controlled retraction that provides exposure
but must not be so excessive as to fracture the sternum or ribs. The section proceeds proximally and distally until adequate length is obtained for the intended graft and usually
terminates at the bifurcation of the internal mammary artery (right inset). Heparin is then administered systemically before the internal mammary artery is occluded. The internal
mammary artery is prepared for grafting after transection. (From Jones RH: Coronary artery bypass grafts. In Sabiston DC Jr [ed]: Atlas of Cardiothoracic Surgery. Philadelphia,
WB Saunders, 1995.)
team can procure the greater saphenous vein or radial artery simultaneously.
Next, the patient is systemically heparinized (300 units/ kg) with a target activated clotting time (ACT) greater than 400 seconds. The aorta is examined
to detect areas of calcification and determine the site of cannulation and cross-clamping. Epiaortic echocardiography can be used to identify calcific
plaques and help plan the site of cannulation to minimize disruption of atherosclerotic plaques and reduce the risk of embolization and stroke. The
ascending aorta is then cannulated proximal to the innominate artery using double-pursestring sutures placed anterolaterally. When the cannula is
introduced into the aorta, it is important that the tip is directed distally. The aortic cannula is then secured in place by tightening the pursestring sutures
with Rumel tourniquets; these are then secured to the side of the cannula. The open end of the cannula is back-flushed to de-air and remove any
atherosclerotic debris in the cannula. It is then attached to the arterial perfusion line from the CPB machine. The proximal end of the aortic cannula is
then secured to the wound edges. Venous cannulation is performed by introducing a cannula into the right atrium through a single pursestring suture in
the right atrial appendage. If a dual-stage venous drainage cannula is used, the tip is directed into the inferior vena cava. The venous pursestring sutures
are tightened using a Rumel tourniquet and then secured to the venous cannula. The open end is then interfaced with the venous tubing from the bypass
pump. If retrograde cardioplegia is to be administered, a pursestring suture is placed near the AV groove, the atrium is incised, and a retrograde
cardioplegia cannula is introduced into the coronary sinus.
After heparinization, the patient is placed on CPB and cooled to a core temperature of 30°C to 32°C. During cooling, a cardioplegic cannula can be
inserted into the aorta through a separate pursestring suture. It is sufficiently distanced from the aortic cannula to allow room for the aorta to be cross-
clamped. In addition to infusing cardioplegic solutions, the cannula can be used to decompress the left ventricle. Once acceptable pump flows (2.2
L/min/m2 ) have been achieved, and the mean blood pressure has been stabilized (50 to 70 mm Hg), the aorta is cross-clamped and cold cardioplegic
solution is infused.
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The heart is also cooled topically using a saline slush solution. The phrenic nerve can be protected by covering it with an insulating pad. If both
antegrade and retrograde cardioplegia are to be used, two thirds of the solution is administered antegrade, with the remainder given via the retrograde
cannula. Antegrade and/or retrograde cold oxygenated blood cardioplegia is then administered intermittently, usually at 15- to 20-minute intervals, to
ensure adequate myocardial protection during the period of ischemic arrest.
Distal Anastomoses
The target vessels can be identified and the sites of the distal anastomoses determined either prior to cross-clamping the aorta or afterward. The
advantage of the former is that the coronary arteries are distended and appropriate graft length is easier to assess. The ideal anastomotic site is readily
accessible and free of atherosclerotic disease and has a diameter of at least 1.5 mm.
After the anastomotic site has been selected, a Beaver blade is used to expose the anterior aspect of the coronary artery, and a small, sharp, pointed lance
is used to puncture the vessel; the arteriotomy is then extended by using fine coronary scissors to create a 3- to 7-mm opening that is scaled to match the
luminal diameter of the conduit. Care must be taken not to injure the posterior wall. An endarterectomy of the coronary artery is avoided, if possible,
because this is associated with a higher graft/native artery thrombosis rate.
When a reversed saphenous vein segment graft is used, the anastomosis is performed with either interrupted sutures or a continuous running 7–0 or 8–0
polypropylene suture. With the latter, care is taken to avoid pursestringing the suture line and narrowing the anastomotic site. A 1.0- or 1.5-mm vessel
probe is often used to evaluate the patency of the anastomosis. The graft is then pressurized with heparinized blood or cardioplegic solution to evaluate
the anastomosis for hemostasis or evidence of stricture. If multiple vessels are to be grafted, a single conduit (e.g., saphenous vein) can be used by
performing multiple side-to-side anastomoses (sequential grafting) to conserve graft length.
When using the ITA, the distal end of the vessel is beveled at a sharp angle and then the incision is extended using fine iris scissors ( Fig. 59–12 ). The
arteriotomy of the native vessel is sized to match the opening of the ITA. The end-to-side anastomosis can be completed using a continuous running 8–0
polypropylene suture ( Fig. 59–13 ). On completion of the anastomosis, both sides of the pedicle are sutured to the epicardium to minimize tension on
the anastomosis and/or twisting of the graft when the patient is weaned from CPB and the lungs are ventilated.
Proximal Anastomoses
If the proximal anastomoses are completed after the distal anastomoses have been completed, this can be done either while the aortic cross-clamp is still
in place or after it has been removed and replaced with a partial occlusion clamp. The latter allows the heart to be perfused and rewarmed. The
advantage of the single cross-clamp technique is that it minimizes the number of times the aorta is manipulated and theoretically reduces the risk of
plaque disruption and embolization. With either approach, an aortic punch is used to excise buttons of aortic tissue and create the sites for the proximal
graft anastomoses. The proximal opening of the conduits are then spatulated to create a hood, and each anastomosis is completed using a running 6–0 or
7–0 polypropylene suture. The same technique can be used if the proximal aortic anastomoses are performed prior to placing the patient on CPB using a
partial aortic occlusion clamp. After completing the anastomoses, small bulldog clamps are placed on each graft and the patient is placed in a head-
down position. Pump flow is transiently reduced as the aortic cross-clamp is
Figure 59-12 The technique of anastomosis between the left internal mammary artery and the left anterior descending coronary artery illustrates the general principles used to
construct all proximal and distal anastomoses. The graft is opened longitudinally to match or exceed the length of the coronary arteriotomy. This opening prevents kinking at the site
of the anastomosis of the internal mammary artery and aorta to the saphenous vein. This opening is not necessary at the distal vein anastomotic site, but a slight bevel cut of the distal
vein helps prevent kinking of the saphenous vein to the coronary artery anastomosis. (From Jones RH: Coronary artery bypass grafts. In Sabiston DC Jr [ed]: Atlas of Cardiothoracic
Surgery. Philadelphia, WB Saunders, 1995.)
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Figure 59-13 The anastomosis begins midway along the side of the graft so that the final knot will not be at the most distal or proximal portion of the anastomosis, thereby decreasing
the chances of technical error that would impede graft flow. The polypropylene suture permits a portion of the anastomosis to be completed before the two vessels are joined. (From
Jones RH: Coronary artery bypass grafts. In Sabiston DC Jr [ed]: Atlas of Cardiothoracic Surgery. Philadelphia, WB Saunders, 1995.)
removed and the heart is reperfused. After the grafts have filled with blood, small punctures are made in the veins using a 25-gauge needle for de-airing.
The bulldog clamps are then removed, and the heart is perfused via both the grafts and the native vessels. All anastomotic sites are re-examined and
bleeding sites are oversewn.
Termination of Cardiopulmonary Artery Bypass
Systemic rewarming is usually initiated after completion of the last distal anastomosis. Blood that has accumulated in the pleural spaces is evacuated
and reprocessed using a Cell Saver for later reinfusion. After removing the aortic cross-clamp, the heart usually starts to beat spontaneously within a
short time. Although a normal sinus rhythm may develop, it frequently deteriorates into ventricular fibrillation and requires cardioversion using internal
defibrillating paddles. If the heart rate is less than 70 beats/min, temporary atrial and ventricular pacing wires can be attached to the surface of the heart,
usually the right atrium or right ventricle, and pacing is commenced at approximately 90 beats/min. Once the patient has been adequately rewarmed
(˜36.5°C), normal sinus rhythm has been restored, and ventilation has been re-established, then the patient is weaned from CPB by gradually reducing
the pump flow rates to zero while maintaining adequate intravascular volume via transfusion. It is often necessary to manipulate the contractile state of
the heart and the peripheral vascular resistance (afterload) by infusing inotropic agents such as dobutamine and vasodilators or vasoconstrictors such as
nitroglycerin and ephedrine, respectively. Once the patient has been stabilized off CPB, protamine is administered to reverse the heparin-induced
anticoagulation. The aortic and venous cannulae are removed and pursestring sutures tied. Pleural and mediastinal chest tubes are inserted (along with
temporary epicardial atrial and ventricular pacing wires if not placed earlier) and after hemostasis has been achieved, the sternum is closed using large-
caliber stainless steel wire in simple and/or figure-of-eight patterns. The presternal fascia and area around the xiphoid is then approximated with Vicryl
sutures and the remaining tissue closed in layers. The skin can be approximated with a running subcuticular monofilament suture and Steri-Strips or
staples. Dry sterile dressings are placed over all incisions, lines, wires, and drains and the patient is transferred to the surgical ICU.
Postoperative Care
Postoperative care begins with the transport of the patient to the cardiac surgery ICU.[20] A directed physical examination should be performed on
arrival. This includes the assessment of level of consciousness, respiratory sounds, peripheral pulses, and body temperature. Mediastinal chest tube
drainage should be recorded and assessed hourly. Initial ventilator settings should be set to match those in the operating room. Alternatively, the
ventilator can be set at a tidal volume of 12 mL/kg, an intermittent mandatory ventilation rate of 8 to 10 breaths/min, a fraction of inspired oxygen of
60% and a 5 cm H2 O positive end-expiratory pressure to provide an adequate margin of safety. A portable chest radiograph is obtained to confirm the
position of the endotracheal tube and identify a pneumothorax, atelectasis, pulmonary edema, or pleural effusions. Initial laboratory studies should
include hemoglobin, hematocrit, electrolytes, blood urea nitrogen, creatinine, platelet count, prothrombin time, partial thromboplastin time, and arterial
blood gases. With respect to continuous monitoring devices, the patient should have an ECG with the ability to assess ST-T wave abnormalities, an
arterial line to measure arterial blood pressure, a line to measure central venous pressure, pulse oximetry, capnography, and a core temperature. In select
patients, pulmonary artery pressures and cardiac output are monitored continuously using a Swan-Ganz catheter.
The primary considerations during the first 12 hours after the operation should be the maintenance of adequate blood pressure, cardiac output,
correction of coagulation defects, correction of ionized hypocalcemia, stabilization of intravascular volume, and normalization of the peripheral vascular
resistance. This often involves the administration of crystalloid solutions, blood or blood products, inotropic agents, calcium, and vasodilators and/or
vasoconstrictors.
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In the immediate postoperative period it is desirable to avoid marked elevations in blood pressure (mean arterial pressure > 100 mm Hg). Significant
hypertension results in increased myocardial oxygen consumption and tension on arterial suture lines. In patients with significant preexisting left
ventricular dysfunction, this can also lead to a reduction in the cardiac index. The hypertension may be due to hypoxemia, hypercarbia, hypothermia
with shivering, and inadequate sedation and analgesia. It can be treated by clearing the endotracheal tube of secretions, adjusting ventilator settings,
warming the patient with forced hot-air blankets, administering analgesics, and infusing vasodilators. Since low cardiac output occurs frequently after
CPB, a Swan-Ganz catheter can be used to assess the adequacy of systemic perfusion by measuring the cardiac index and monitoring mixed venous
oxygenation. In general, the aim is to maintain a cardiac index of 2.2 L/min/m2 and a mixed venous oxygenation of 60%. The most common causes of
low cardiac output are myocardial ischemia, hypovolemia, abnormal heart rate/rhythm, myocardial dysfunction, and cardiac tamponade. Myocardial
ischemia can range from asymptomatic ST-segment changes to infarction with profound hypotension. The former may be due to coronary artery spasm,
ITA hypoperfusion, or early graft occlusion. Coronary artery spasm occurs in about 1% of patients, and it can be treated with intravenous nitroglycerin
or calcium-channel blockade. In the meantime, α-adrenergic agents may be required to support the blood pressure. If the ischemia is severe and the
patient does not respond to pharmacologic support, the patient may require emergent coronary angiography or reoperation and/or regrafting. With
hypovolemia, there is a variety of opinion as to which resuscitation fluid should be used postoperatively. Generally, lactated Ringer’s solution is
appropriate but can be supplemented with up to 1.5 L of hydroxy-ethyl starch (Hetastarch) or blood products. The latter depends on whether the patient
is anemic or there is evidence of active bleeding.
In the immediate postoperative period, most patients are tachycardic. If they have been treated prior to surgery with β blockers, they may be bradycardic
(<60 beats/min). In general, the desirable rhythm and heart rate is sinus and a rate between 70 and 100 beats/min. A normal sinus rhythm ensures AV
synchrony and maintenance of the atrial kick, which can contribute up to 25% of the cardiac output. If the patient is bradycardic or has evidence of heart
block, atrial or AV pacing via temporary pacing wires should be initiated to increase the rate and/or restore synchrony. Arrhythmias are common and
may result from abnormal electrolytes, acidosis, high circulating catecholamine levels, and myocardial ischemia. Supraventricular tachyarrhythmias
associated with hemodynamic instability should be treated immediately with cardioversion. Hemodynamically stable patients with re-entrant
supraventricular tachyarrhythmias should be treated with adenosine in incremental doses. Atrial flutter can be treated using overdrive pacing if the rapid
atrial rate can be captured and controlled. Atrial fibrillation responds to intravenous digoxin, procainamide, diltiazem, β blockers, amiodarone, and
cardioversion. The hemodynamically unstable patient with ventricular arrhythmias needs immediate defibrillation and an intravenous bolus infusion of
lidocaine followed by a continuous infusion. Other alternative agents include procainamide and bretylium.
Myocardial dysfunction is also common in patients after CPB and may be secondary to myocardial stunning (a transient reversible injury) or myocardial
necrosis (infarction). In these patients, if the heart rate, ventricular preload, rhythm, and afterload have been optimized and they still demonstrate a low
cardiac output, they will most likely benefit from inotropic support. This support can be divided into catecholamine (epinephrine, norepinephrine,
dobutamine, and dopamine) and noncatecholamine (milrinone) agents. Epinephrine is an effective drug because of its α- and β-adrenergic properties. At
low doses, epinephrine stimulates peripheral β2 receptors and promotes mild vasodilation. At higher doses, α stimulation causes vasoconstriction and
tachycardia. Norepinephrine has a more pronounced effect on the peripheral α receptors, with resultant vasoconstriction. These drugs are generally
administered at doses between 0.01 and 0.10 µg/kg/min and adjusted according to the patient’s response. Dobutamine and dopamine have comparable
effects on cardiac output and are similar to epinephrine and norepinephrine but, in general, are less arrhythmogenic. Dobutamine is also a vasodilator
and is used to reduce both left ventricular preload and afterload. Milrinone is a phosphodiesterase inhibitor and slows the degradation of cyclic
adenosine monophosphate (cAMP). Its mechanism of action complements that of catecholamines, which stimulate cAMP. This drug is often used in
combination with other inotropic agents to achieve a synergistic outcome.
Intra-aortic Balloon Pump.
For patients who demonstrate profound myocardial dysfunction, and are unresponsive to volume resuscitation, intense pharmacologic therapy treatment
with an IABP may be indicated. Intra-aortic balloon pumping improves mean blood pressures and coronary artery perfusion and decreases cardiac
work/oxygen demand. If the IABP was used to control chest pain preoperatively, it is often removed within 24 hours after the operation. However, if
used preoperatively for hemodynamic instability, weaning from the IABP may be more difficult and require more time.
Tamponade.
In the postoperative period, pericardial tamponade is due to formation of pericardial clot and compression of the heart. The condition should be
suspected if the patient exhibits evidence of low cardiac output and hypotension, fails to respond to intravenous fluid infusions, and requires increasing
levels of inotropic support. Often there has been a marked decline in mediastinal chest tube drainage prior to the onset of signs of tamponade. The
diagnosis can be made on the basis of widening of the mediastinum on chest radiograph and evidence of a pericardial effusion by echocardiography.
Surface echocardiography is helpful but may be compromised due to the presence of wound dressings and chest tubes. TEE is more reliable but may not
be immediately available. If a Swan-Ganz catheter is in place and right and left heart pressures are monitored, the central venous pressure and
1865
pulmonary capillary wedge pressure are usually elevated and equal. Once the diagnosis is made, the patient should be returned to the operating room for
evacuation of the clot and relief of the compression. If the patient’s condition is rapidly deteriorating, a simple subxiphoid approach can be performed at
the bedside and result in a dramatic improvement in hemodynamics.[21]
Postoperative Bleeding.
The combination of heparinization, hypothermia, CPB, and protamine reversal is associated with increased risk of bleeding after CABG surgery.
Bleeding after CABG surgery requiring transfusion and/or reoperation to stop the bleeding is associated with a significant increase in morbidity and
mortality. Bleeding, more than 500 mL in the first hour or persistent bleeding more than 200 mL an hour for 4 hours, are indications for mediastinal
exploration. Exploration is also indicated if a large hemothorax is identified on chest radiograph or pericardial tamponade occurs. In 20% of the cases, a
specific site of bleeding can be identified. The typical sources of surgical bleeding include the cannulation sites, the proximal/distal anastomoses, and
branches of the ITAs and the vein grafts. Most of the time, however, a specific bleeding site is not identified, and it is related to inadequate heparin
neutralization; qualitative or quantitative platelet dysfunction; fibrinolysis; and deficiencies in factors V, VIII, XIII, and fibrinogen and plasminogen. In
the setting of suspected nonsurgical postoperative bleeding, one approach is to obtain an ACT and administer protamine to return the ACT to baseline.
[22] In general, red blood cells are administered if the patient is actively bleeding and/or anemic (hematocrit ≤25%). Platelets are administered if there is
evidence of thrombocytopenia (<50,000/mm3 ) or platelet dysfunction (abnormal thromboelastography). Cryoprecipitate can be used to treat low
fibrinogen levels (<100 mg/dL). Desmopressin,[23] epsilon aminocaproic acid,[24] and aprotonin [25] can also be used, although these are generally
restricted to high-risk patients.
Extubation.
It is desirable to initiate the process of ventilator weaning as soon as the patient awakens, is hemodynamically stable with minimal chest tube drainage,
and can maintain a satisfactory spontaneous tidal volume and respiratory rate. In general, the cardiac index should be 2.2 L/min/m2 or higher and the
mean arterial pressure should exceed 70 mm Hg. The patient should be comfortable on continuous positive airway pressure support with minimal
secretions and have a spontaneous respiratory rate of 20 or more breaths/min. The ability to maintain an arterial pH greater than 7.35 while the
intermediate mandatory ventilation rate is reduced to zero is a reliable test. After extubation the patient needs to be encouraged to breathe deeply and
use incentive spirometry. Suboptimal postoperative pulmonary function may require additional therapy, including the use of bronchodilators,
mucolytics, and chest physical therapy. After extubation it is important to provide the patient with sufficient pain relief to minimize emotional distress,
poor coughing, and the reluctance to begin ambulation. Unrelieved pain can also be a source of tachycardia, hypertension, and myocardial ischemia.
Prior to leaving the ICU, unnecessary lines and catheters should be removed. Removal of temporary atrial and ventricular pacing wires is often deferred
to the third postoperative day.[26] [27] [28]

INDICATIONS FOR CABG SURGERY AND OUTCOMES AFTER REVASCULARIZATION
The aim of medical treatment for patients with symptomatic CAD is to reduce the heart’s demand for oxygen by slowing the heart rate, decreasing
myocardial contractility, and reducing systemic vascular resistance. The objective of interventional therapy is to increase the supply of oxygen and
nutrients by dilating or bypassing the coronary artery obstructions. Although medical therapy can be effective, in many cases the optimal treatment is
angioplasty with or without stents and/or CABG surgery. Since interventional therapy is not without risk, it is important to understand the relative
survival benefits and risks associated with them. The survival benefit of CABG surgery patients was initially studied in patients with chronic stable
angina.
Chronic Stable Angina
CABG Surgery Versus Medical Management
In the 1970s and 1980s several prospective, randomized clinical trials evaluated the survival benefit of CABG surgery in patients with chronic stable
angina ( Table 59–4 ). These studies showed significant benefit and resulted in the widespread application of CABG surgery for the treatment of
patients with CAD. They also helped to identify specific categories of patients with angina who were most likely to benefit from CABG surgery,
namely patients with LMCA disease; one-, two-, or three-vessel disease with proximal LAD involvement; and three-vessel disease with impaired left
ventricular function.[29] [30] These observations were confirmed in a meta-analysis of these studies (see Table 59–4 ) ( Fig. 59–14 ). These trials were
conducted in an era in which fewer than 50% of the patients were treated with β blockers, fewer than 40% were administered aspirin (ASA), and fewer
than 10% of the patients received an ITA graft. Also, calcium-channel blockers, angiotensin-converting enzyme inhibitors, and lipid-lowering agents
were not available. Women were excluded in all but one of the trials, and only patients younger than 65 years of age were studied. Nevertheless, as a
direct result of these clinical studies, CABG surgery is now accepted as an appropriate therapeutic modality for the treatment of specific subsets of
patients with chronic stable angina ( Table 59–5 ).
PTCA Versus Medical Management
In the 1980s PTCA was introduced as an alternative to CABG surgery. The first successful PTCA was performed by Gruntzig and colleagues.[31]
Initially the procedure was performed in patients with one-vessel disease and isolated lesions. With improvements in catheter technology, increasing
experience, and the advent of stent-based
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TABLE 59-4 -- Foundation Studies of Patients with Stable Ischemic Heart Disease Used to Evaluate CABG Surgery Outcomes *
Study Reference Source Conclusions
Veterans Administration Coronary Artery Bypass N Engl J Med 311:1333– Survival advantage was associated with CABG surgery when
Surgery Cooperative Study Group: Eleven-year 1339, 1984 CAD included LM and/or 3VD with impaired LV function.
survival in the Veterans Administration randomized
trial of coronary bypass surgery for stable angina.
Varnauskas E, European Coronary Surgery Study N Engl J Med 319:332–337, CABG surgery was associated with increased survival in
Group: Twelve-year follow-up of survival in the 1988 patients with LM disease, 3VD, decreased LV function, and
randomized European Coronary Surgery Study. proximal LAD stenosis 12 years from randomization. Peak
advantage was observed at 5 years.
Coronary Artery Surgery Study (CASS) principal Circulation 68:939–950, 1983 CABG surgery and nonoperative management was associated
investigators and associates: CASS: A randomized with similar survival rates. Surgery could be safely deferred
trial of coronary bypass surgery. until onset of symptoms.
Norris RM, Agnew TM, Brandt PWT, et al: Circulation 63:785–792, 1981 CABG surgery conferred survival advantage in patients with
Coronary surgery after recurrent myocardial LM disease and/or 3VD with decreased LV function.
infarction: Progress of a trial comparing surgical with
nonsurgical management for asymptomatic patients
with advanced coronary disease.
Mathur VS, Guinn GA: Prospective randomized Cardiovasc Clin 8:131–144, CABG surgery was associated with superior improvement in
study of the surgical therapy of stable angina. 1977 symptoms and quality of life.
Kloster FE, Kremkau EL, Ritzman LW, et al: N Engl J Med 300:149–157, CABG surgery resulted in greater functional improvement and
Coronary bypass for stable angina. 1979 less UA compared to medical therapy, no difference in death or
myocardial infarction after 3 years.
LM, left main; 3VD, three-vessel disease; CAD, coronary artery disease; LAD, left anterior descending artery; CABG, coronary artery bypass grafting;
UA, unstable angina, LV, left ventricular.
* Used by Yusuf and associates[30] in meta-analysis.
Figure 59-14 Cumulative total mortality for 12 years after coronary artery bypass grafting (CABG) surgery versus noninterventional management for patients with chronic stable
angina. (Adapted from Yusuf S, Zucker D, Peduzzi P, et al: Effect of coronary artery bypass graft surgery on survival: Overview of 10-year results from randomised trials by the
Coronary Artery Bypass Graft Surgery Trialists Collaboration. Lancet 344:563–570, 1994.)
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TABLE 59-5 -- Indications for CABG Surgery Alone in Patients with Stable Angina, Unstable Angina, and Acute Myocardial Infarction
Class *
Condition I IIa IIb III
Asymptomatic or 1. LMCA stenosis ≥ 60% Proximal LAD stenosis with 1VD or 2VD not None
mild angina 1VD or 2VD † involving the
proximal LAD ‡
2. LMCA equivalent: proximal
LAD and LCA stenoses >
70%
3. 3VD (survival benefit >
with abnormal LV function:
EF < 0.50)
Chronic stable 1. LMCA stenosis ≥ 60% 1. Proximal LAD stenosis with None 1. 1VD or 2VD without significant
angina 1VD † proximal LAD stenosis, in patientswho
have mild symptoms that are unlikely due
to myocardial ischemia or have not
received an adequate trial of medical
therapy and (1) have only a small area of
viable myocardium or (2) no demonstrable
ischemia on noninvasive testing
2. LMCA equivalent: proximal 2. 1VD or 2VD without 2. Borderline stenoses (50–60%) other than
LAD and LCA stenoses > significant proximal LAD in the LCMA, no demonstrable ischemia
70% stenosis, moderate area of on noninvasive testing
viable myocardium, and
demonstrable ischemia on
noninvasive testing
3. 3VD (survival benefit > 3. <50% coronary stenosis
with abnormal LV function:
EF < 0.50)
4. 2VD with significant
proximal LAD stenosis: either
EF < 0.50 or ischemia on
noninvasive testing
5. 1VD or 2VD without
significant proximal LAD
stenosis, but with myocardium
and high-risk criteria on
noninvasive testing
6. Disabling angina despite
maximal medical therapy
(acceptable-risk patient)
UA/NSTEMI 1. LMCA stenosis ≥ 60% Proximal LAD stenosis with 1VD or 2VD not None
1VD or 2VD † involving the
proximal LAD ‡
2. LMCA equivalent: proximal
LAD and LCA stenoses >
70%
3. Ongoing ischemia
unresponsive to maximal
nonsurgical therapy
STEMI/AMI None Ongoing ischemia/infarction 1. Progressive LV Primary reperfusion late (>12 hr) in
unresponsive to maximal pump failure with evolving STEMI without ongoing ischemia
nonsurgical therapy coronary stenosis
compromising
viable myocardium
outside the initial
infarct area
2. Primary
reperfusion in the
early hours (≤6–12
hr) of an evolving
STEMI
§ Becomes class I if arrhythmia is resuscitated sudden cardiac death or sustained ventricular tachycardia.
1VD, one-vessel disease; 2VD, two-vessel disease; 3VD, three-vessel disease; LMCA, left main coronary artery; LAD, left anterior descending
coronary artery; LCA, left coronary artery; LV, left ventricular; EF, ejection fraction; STEMI/AMI, ST-elevation myocardial infarction/acute
myocardial infarction; UA/NSTEMI, unstable angina/non–ST-elevation myocardial infarction.
Adapted from Eagle KA, Guyton RA, Davidoff R, et al: ACC/AHA guidelines for coronary artery bypass graft surgery: Executive summary and
recommendations. Circulation 100:1464–1480, 1999.
* Class I: Conditions for which there is evidence and/or general agreement that a given procedure/treatment is useful and effective.
Class IIa: Weight of evidence/opinion is in favor of usefulness/efficacy.
Class IIb: Usefulness/efficacy is less well established by evidence/opinion.
Class III: Conditions for which there is evidence and/or general agreement that the procedure/treatment is not useful/effective and in some cases may be harmful.
† Becomes class I if extensive ischemia documented by noninvasive study and/or an LVEF < 0.50.
‡ If there is a large area of viable myocardium and high-risk criteria on noninvasive testing, becomes class I.
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interventions, angioplasty is now performed in patients with multivessel disease. Although the short-term symptomatic success rate for PTCA
approaches 85% to 90%, the role of PTCA in the management of patients with angina whose symptoms are adequately controlled with medical therapy
remains controversial.[32] [33] In the Angioplasty Compared to Medicine (ACME) trial,[32] the efficacy of PTCA was compared to best medical
management in 212 patients with proven ischemia, one-vessel disease, and stenoses greater than 70%.[34] In this study, patients undergoing PTCA
demonstrated better relief from angina, reduced need for antianginal medications, a better quality of life, and improved exercise tolerance; there was no
difference in mortality. Some PTCA patients (19%), however, required additional catheter-based interventions, and 7% underwent CABG surgery. In
the medically managed group of patients, 11% progressed to PTCA, and none underwent CABG surgery. At 4 years, the patients who were initially
randomized to PTCA had a better quality of life and significantly fewer episodes of UA. Thus, in this trial the increased number of reinterventions in the
first year and higher costs for the PTCA-treated group appeared to be justified owing to the lower incidence of late procedures and lower costs in the
extended follow-up period. In the Coronary Angioplasty Versus Medical Therapy for Angina: The Second Randomised Intervention Treatment of
Angina (RITA-2) trial,[33] PTCA provided better early symptomatic relief from angina compared to medical treatment. This, however, was not sustained
over time and at 2.9 years the PTCA group had a 3% greater risk of death or myocardial infarction ( Table 59–6 ).
A major limitation of these studies was the absence of preprocedural cardiac stress and myocardial viability testing. Without this information, it is
difficult to know whether the PTCA and medical therapy groups were equally matched. It is also difficult to extrapolate the findings to current practice
since the studies were performed prior to the use of coronary stents. Nevertheless, it appears that patients with angina may be treated relatively safely
with either medical management or PTCA intervention. The risk of early complications associated with PTCA, however, needs to be taken into
consideration prior to proceeding with the interventional approach in this group of patients.
CABG Surgery Versus PTCA
One of the first large-scale, prospective, randomized studies comparing PTCA and CABG surgery was the Bypass Angioplasty Revascularization
Investigation (BARI) trial reported in 1996.[35] Patients with multivessel disease were randomly assigned to either CABG surgery (n = 914) or PTCA (n
= 915) and followed for a mean of 5.4 years. In the short term, the incidence of Q-wave myocardial infarction was higher in the CABG surgery group
(4.6% vs. 2.1%); the stroke rates were similar (0.8% vs. 0.2%). At the end of 5 years, the survival rate was 89.3% for the CABG surgery cohort and
86.3% for the PTCA cohort (P = 0.19). Among the PTCA patients, however, 54% required additional revascularization procedures. Thirty-one percent
underwent CABG surgery, 34% underwent additional PTCA, and 11% underwent both interventions. In contrast, only 8% of the CABG surgery
patients required repeat revascularization. Thus it appears that although PTCA did not compromise the 5-year survival rate in patients with multivessel
disease, subsequent revascularization including CABG surgery was required more often. Among the diabetic patients, the 5-year survival rate for the
CABG surgery patients was markedly greater (80.6% vs. 65.5%) (see Table 59–6 ). In the Emory Angioplasty versus Surgery Trial (EAST), the primary
endpoints were death, myocardial infarction, or the presence of a large myocardial ischemic defect at 3 years. Secondary endpoints included all-cause
mortality and the requirement for repeat revascularization procedures after extended follow-up. After the 3-year anniversary visit, patients were
contacted and medical records examined until death or the 8-year anniversary. The results showed that after 3 years the number of repeat interventions
was similar. At 8 years, the survival rate for both groups was also similar: angioplasty, 79.3%, and CABG surgery, 82.7%. There was a tendency for
patients with proximal LAD stenosis and those with diabetes to have better late survival with CABG surgery (see Table 59–6 ). Owing to the relatively
small number of patients enrolled, however, the study was underpowered to detect significant survival differences.
A strength of the BARI trial was that the myocardial infarction classification methodology was based on symptoms, ECG results obtained at
predetermined time intervals, and a core laboratory was used to measure myocardial enzyme levels. The entry criteria, however, resulted in the selection
of patients preferentially suited for PTCA. Thus the, findings may not be fully applicable to patients with complex multivessel disease. Also, in both the
BARI and EAST studies, few CABG surgery patients underwent extensive arterial grafting, and they were performed when stents and new
pharmacologic agents, such as clopidogrel and other glycoprotein (GP) IIb/IIIa inhibitors, were not available. Despite these limitations, it appears that
CABG surgery clearly confers a survival benefit to diabetic patients that is superior to angioplasty. With respect to quality of life and economic issues,
although the data collected in 934 of the 1829 patients enrolled in the BARI trial showed that PTCA patients returned to work sooner, CABG surgery
patients had better functional status scores (Duke Activity Status Index). Also, although the cost of angioplasty was 95% that of CABG surgery (P =
0.047), the actual difference between the two interventions was less than $3000, and this was observed only in patients with two-vessel disease. PTCA
appeared to be more expensive in patients with three-vessel disease.
In summary, patients with stable angina can safely undergo PTCA as a first intervention for CAD. CABG surgery confers a superior long-term survival
benefit in patients with specific anatomic lesions and is associated with an increased freedom from angina, a significant reduction in antianginal
medications, and fewer subsequent PCIs. CABG surgery is the treatment of choice in diabetic patients. Although controversial, the eco-nomic impact
appears to be comparable when the frequency of repeat PCI is taken into consideration.
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TABLE 59-6 -- Pertinent Studies Comparing PTCA and CABG Surgery

Study, No. of Patients (N),
and Follow-Up (F/U) Reference Purpose Findings
Arterial Revascularization J Am Coll Cardiol Evaluated effectiveness of Complete revascularization more frequently accomplished by
Therapies Study (ARTS) N = 39:559–564, 2002 revascularization on event-free CABG surgery. Patients randomized to stenting with incomplete
205 F/U = 400 days survival revascularization had greater need for subsequent bypass
surgery
N Engl J Med Compared CABG surgery and Coronary stenting was less expensive than bypass surgery
344:1117–1124, stenting for multivessel ($3000/patient) and conferred same degree of protection against
2001 disease death, stroke, and myocardial infarction. Stenting was associated
with greater need for repeat revascularization.
Bypass Angioplasty N Engl J Med Compared CABG surgery with Initial strategy of PTCA did not compromise 5-year survival of
Revascularization 335:217–225, 1996 angioplasty in patients with overall population but was associated with more frequent
Investigation (BARI) N = 1829 multivessel disease subsequent revascularizations. Five-year survival for treated
F/U = 5.4 years diabetics was better after CABG surgery (PTCA 65.5%, CABG
80.6%).
JAMA 277:715– Analyzed clinical and Angina-free episodes were greater in CABG patients. Use of
721, 1997 functional outcomes in anti-ischemic medications was higher in PTCA group. Need for
patients with multivessel revascularization was greater in PTCA patients (52%) vs.
disease CABG surgery (6%).
Circulation 96: Assessed influence of diabetes Patients with treated diabetes mellitus assigned to initial strategy
1761–1769, 1997 in CABG and PTCA patients of CABG had a marked improvement in survival (CABG
with multivessel disease 94.2%, PTCA 79.4%). Survival benefit of CABG surgery was
confined to patients receiving at least one ITA graft.
N Engl J Med Analyzed cost and quality of CABG surgery was associated with better quality of life. PTCA
336:92–99, 1997 life had a lower 5-year cost than CABG surgery only in patients
with 2VD.
Circulation Analyzed outcome for women The 5-year unadjusted mortality rate for women and men
98:1279–1285, undergoing CABG and PTCA was similar. Due to higher risk
1998 profiles, female gender was an independent predictor of
improved 5-year survival after adjusting for risk factors.
F/U = 7.8 years J Am Coll Cardiol Assessed long-term outcomes Survival benefit of CABG surgery over PTCA was more
35:1122–1129, in diabetic patients pronounced at 7 years (CABG 76.4%, PTCA 55.7%).
2000
Emory Angioplasty Versus J Am Coll Cardiol Evaluated long-term outcome Patients with proximal LAD stenosis and those with diabetes
Surgery Trial (EAST) N = 392 35:1116–1121, tended to have better late survival with CABG surgery (did not
F/U = 8 years 2000 reach statistical significance).
Randomized Intervention Lancet 341:573– Examined long-term effects of Prevalence of angina was three times higher in the PTCA group
Treatment of Angina (RITA)-1 580, 1993 PTCA vs. CABG in patients at 6 months and 1.5 times higher at 2 years. Antianginal drugs
N = 1011 F/U = 2.5 years with 1VD, 2VD, and 3VD were more frequent in the PTCA group. CABG surgery was
associated with a lower risk of angina and fewer additional
diagnostic and therapeutic interventions.
F/U = 2 years Lancet 344:927– Analyzed cost of intervention Cost of PTCA was 80% of CABG surgery at 2 years.
930, 1994
F/U 6.5 year Lancet 352:1419– Analyzed clinical outcomes No difference in mortality. In the PTCA group, the prevalence
1425, 1998 and cost of angina was higher and 26% underwent CABG. PTCA and
CABG surgery costs were similar after 5 years.
RITA-2 Coronary Angioplasty Lancet 350:461– Compared long-term effects of Early intervention with PTCA was associated with greater
Versus Medical Therapy for 468, 1997 PTCA and conservative care symptomatic improvement but also with small but real excess
Angina N = 1018 F/U = 2.7 hazard due to procedure-related complications.
years
CABG, coronary artery bypass grafting; PTCA, percutaneous transluminal coronary angioplasty; LAD, left anterior descending coronary artery; ITA,
internal thoracic artery; 1VD, one-vessel disease; 2VD, two-vessel disease; 3VD, three-vessel disease.
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Whether this will remain the case with drug-eluting stents is unknown.
Acute Coronary Artery Syndromes
Patients with ACSs represent a cohort of patients who have signs and symptoms that require expedited evaluation and treatment. It characterizes a
constellation of clinical conditions that reflect acute myocardial ischemia and includes the categories of UA, non–ST-elevation myocardial infarction
(NSTEMI), and ST-elevation myocardial infarction (STEMI). UA with an enzyme leak is termed an NSTEMI. More than 1.5 million patients with UA
or NSTEMI are admitted to the hospital annually, and 800,000 are admitted with STEMI. Of those suffering from an AMI, 213,000 will die; and half of
these patients do so within an hour after onset of symptoms. Arrhythmias, usually ventricular fibrillation, are the cause of early death. The categories of
AMI are NSTEMI, STEMI, non–Q-wave myocardial infarction (NQWMI) and Q-wave myocardial infarction (QWMI). Most patients with ST elevation
go on to develop an acute QWMI. Depending on presentation and diagnosis, the interventional management and outcomes may differ greatly among
patients with UA/NSTEMI and STEMI/AMI.
UA/NSTEMI
PCI Versus Medical Management
The Thrombolysis In Myocardial Ischemia (TIMI) IIIB trial[36] studied the effectiveness of thrombolytic therapy with tissue plasminogen activator (t-
PA) and early PTCA in the treatment of patients with UA. The results showed that thrombolysis conferred no treatment benefit; in fact, there was some
evidence that intravenous t-PA administration prior to PTCA increased the risk of periprocedural myocardial infarction. PTCA, however, did achieve a
high rate of angiographic success and at 1-year follow-up, the cumulative mortality was only 2.0%. The recurrent ischemia rate, however, was frequent;
rehospitalization was required in more than one third of the patients, and repeat revascularization was performed in 28%. Ten percent of the patients
underwent CABG surgery within 12 months. Thus, it appears that although PTCA may be an acceptable therapeutic option in patients with UA, it is
associated with the need for repeat revascularization procedures. Most patients in this study had one-vessel disease and left ventricular function was
only mildly compromised.
Adjunctive Therapy to PCI
To minimize the complications of acute coronary occlusion and restenosis after PTCA (35% to 45% at 6 months), contemporary catheter-based
revascularization now includes coronary stenting and the adjuvant use of platelet GP IIb/IIIa receptor inhibitors. The platelet GP IIb/IIIa receptor is a
site for fibrinogen binding and promotes platelet aggregation. Binding of more than 80% of these receptors results in potent antithrombosis. The ready
availability of these inhibitors (abciximab, tirofiban, eptifibatide) has led to several multicenter clinical trials to address their efficacy. In the Evaluation
of Platelet IIb/IIIa Inhibitor for Stenting (EPISTENT) trial, patients who underwent PTCA for UA/NSTEMI were randomized to stent use and
abciximab therapy versus placebo.[37] The stent patients also received ASA and the thienopyridine, ticlopidine. Adjunctive use of abciximab was
associated with a reduction in death, myocardial infarction, and urgent revascularization at 30 days. It was also associated, however, with a higher
incidence of severe bleeding. At 1 year, the mortality for the stented patients with abciximab was also less in the diabetic patients. In contrast, in a meta-
analysis of six randomized, placebo-controlled trials of patients with UA/NSTEMI receiving GP IIb/IIIa antagonists, there was only a slight reduction in
death or myocardial infarction in patients undergoing stenting. Major bleeding complications, however, remained a problem.
Thienopyridines, adenosine diphosphate (ADP) inhibitors, such as ticlopidine and clopidogrel, are also used to prevent the activation of platelets and
thrombosis and have been studied in patients treated conservatively and with PCI. The Clopidogrel in Unstable Angina to Prevent Recurrent Ischemic
Events (CURE) trial evaluated the effectiveness of clopidogrel in the noninterventional management of patients with UA.[38] Treatment was associated
with a lower rate of death, myocardial infarction, and stroke but a higher rate of major bleeding. In the PCI-CURE trial, patients were randomized to
clopidogrel and ASA, or placebo and ASA, preprocedurally and for 1 month after the procedure. PCI with adjuvant clopidogrel and ASA demonstrated
a reduction in cardiac death and myocardial infarction.[39] Although promising, it is premature to conclude that all patients with UA/NSTEMI with or
without PCI should be treated with IIb/IIIa receptor or ADP inhibitors. This is due, in part, to the differences in clinical trial design that pertain to entry
criteria and definable endpoints and the lack of sufficient long-term follow-up. It does appear, though, that coronary stenting and antiplatelet therapy
improve the efficacy and durability of PCI in managing patients with UA/NSTEMI. A major advantage to using thienopyridines as adjunct therapy is
the lower bleeding complication rates compared to the GP IIb/IIIa inhibitors.
CABG Surgery Versus Medical Management
One of the first studies to evaluate the role of CABG surgery in the treatment of patients with UA/NSTEMI was reported by Parisi and associates.[40]
Patients were stratified by clinical presentation and invasive evaluation of left ventricular function. Clinical presentations included progressive or new-
onset angina and prolonged episodes of angina unrelieved by medication. Abnormal left ventricular function was defined as an EF of less than 0.50.
Five-year follow-up revealed important survival differences in patients with three-vessel disease. The survival rate for the CABG surgery patients was
89%, whereas it was only 75% for medically treated patients. CABG surgery was also associated
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with fewer subsequent hospitalizations. At 8 years’ follow-up, Sharma and colleagues[41] reported that the cumulative survival rates for patients with
severe rest angina associated with ST-T changes on the ECG and abnormal left ventricular function were higher in surgical patients compared to
medically treated patients (87% vs. 54%). In another analysis of the data, medical therapy was determined to be the preferred therapy with UA patients
with only one- or two-vessel disease and normal EF, whereas surgery enhanced survival in patients with three-vessel disease or low EF.[42] These and
other studies demonstrate that CABG surgery is an effective treatment for the management of UA and is associated with sustained symptom relief and
excellent long-term survival (see Table 59–5 ).
CABG Surgery Versus PCI
With increasing evidence that PCI can be performed safely with good long-term survival rates in patients with chronic stable angina, PCI has now been
expanded to treat patients with UA. This has led to clinical trials designed to compare the survival rates between CABG surgery and PCI for patients
with UA/NSTEMI. The Angina with Extremely Serious Operative Mortality Evaluation (AWESOME) trial[43] randomized patients with medically
refractory myocardial ischemia and risk factors for adverse outcomes with CABG surgery into two groups: CABG surgery or PCI. The 30-day survival
rates for CABG surgery and PCI were 95% and 97%, respectively. At 3 years the survival rates were 79% and 80%, respectively. Thus, PCI appeared to
be an acceptable alternative to CABG surgery in patients with medically refractory myocardial ischemia. In a subanalysis of the Arterial
Revascularization Therapies Study (ARTS), patients with multivessel disease and UA were compared to patients with stable angina randomized to
either PCI with stent implantation or CABG surgery using arterial grafts.[44] Similar to the AWESOME trial, there was no difference in the rate of major
adverse events at 1 year. The need, however, for repeat revascularization was higher in the stented PCI patients. Whether long-term survival and
symptom relief in UA patients with multivessel disease treated with stented angioplasty will be comparable to CABG surgery is unknown.
STEMI/AMI
PCI Versus Medical Management for AMI
The role of primary angioplasty in the treatment of patients with STEMI/AMI is controversial. In a meta-analysis that included PTCA as an adjunct to
primary thrombolysis, there was no improvement in survival with delayed PTCA following thrombolytics.[45] In this analysis, there was a trend toward
an increase in the combined endpoints of death and myocardial infarction for patients who underwent deliberate PTCA within a few days of infarction.
When PTCA as an initial therapy was compared to the use of thrombolytics, however, there was a significant reduction in both in-hospital and 6-week
mortality and combined myocardial infarction/mortality in the PTCA patients. These findings suggest that PTCA has a survival advantage over
thrombolytics as an initial treatment for STEMI/AMI, and that use of delayed PTCA as an adjunct to therapy, including thrombolytics, does not affect
survival. In the Global Use of Strategies to Open Occluded Coronary Arteries in Acute Coronary Syndromes (GUSTO) IIb trial,[46] the composite
endpoint of death, nonfatal myocardial infarction, and nonfatal disabling stroke at 30 days was 9.6% for PTCA and 13.7% for thrombolytics. At 6
months, however, the outcomes were similar. This study suggests that although PTCA may confer a short-term benefit over medical management and
thrombolytics, the benefit does not persist over time. In contrast, a retrospective review of the National Registry of Myocardial Infarction (NRMI)-2,[47]
comparing PTCA and thrombolytic therapy for STEMI/AMI, showed no difference in in-house mortality (5.2% vs. 5.4%) or reinfarction rates (2.5% vs.
2.9%). However, in the cohort of patients who presented in cardiogenic shock, there was a survival advantage of 68% for PTCA versus 48% for
thrombolytics. In general, however, there appears to be no major advantage to PTCA for patients with STEMI/AMI.
Role of CABG Surgery
In patients with AMI, CABG surgery is usually performed in conjunction with an operation to treat a specific complication. Examples include refractory
postinfarction angina, papillary muscle rupture with mitral regurgitation, and infarction ventricular septal defect. The rationale for urgent or emergent
surgery is often based on high early mortality due to mechanical complications. Since there are an increasing number of patients who undergo
catheterization early after AMI, it is not surprising that there has been an increase in the number of patients who are identified as candidates for surgery.
The controversial aspect is the timing, since early operative mortality may be as low as 5% in patients with a subendocardial infarction or as high as
25% in patients with poor ventricular function.[48]
In general, patients who were operated on early after AMI are sicker, refractory to medical therapy, have a higher incidence of renal insufficiency,
require IABP insertion, are older, or have sustained a previous myocardial infarction. In one study, the mortality rate for patients undergoing urgent or
emergent CABG surgery less than 6 hours, 6 hours to 2 days, 2 to 14 days, 2 to 6 weeks, and more than 6 weeks following myocardial infarction was
9.1%, 8.3%, 5.2%, 6.5%, and 2.9%, respectively.[49] There was also a twofold higher mortality in patients undergoing CABG surgery in less than 48
hours versus more than 48 hours. The use of preoperative IABP was associated with an improvement in operative mortality. Thus, CABG surgery after
uncomplicated myocardial infarction can be accomplished with acceptable mortality rates provided appropriate supportive interventions, including
IABP, are used early to stabilize the patient prior to surgery. In STEMI/AMI patients who cannot be stabilized with aggressive medical therapy and
nonsurgical interventional support, CABG surgery should be entertained if the coronary
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anatomy is acceptable and a specific mechanical defect can be corrected.
CABG Surgery and Special Patient Populations
Diabetes Mellitus
Patients with diabetes mellitus are at increased risk for developing CAD. This is a significant problem since CAD accounts for 75% of deaths in diabetic
patients. Likewise, the mortality rate after CABG surgery is higher in diabetic patients than it is for the general population. In the prospective,
randomized BARI trial (see Table 59–6 ), CABG surgery mortality rates in diabetic patients receiving saphenous vein grafts only or PTCA were high,
18.2% and 20.6%, respectively. The mortality rate was considerably lower, however, in surgery patients who received ITA grafts (2.9%). In the EAST
trial (see Table 59–6 ), the findings were less conclusive, but there was a similar trend that favored surgery. Thus, CABG surgery that includes ITA
grafts appears to be the treatment of choice for diabetic patients. Currently the Bypass Angioplasty Revascularization Investigation 2 Diabetes (BARI
2D) trial is underway to determine whether treatment targeted to attenuate insulin resistance can arrest or retard progression of CAD and whether early
revascularization reduces the mortality and morbidity in patients with type II diabetes whose symptoms are mild and stable.[50]
Women
While women for every age group have a lower incidence of CAD than men, it is still the leading cause of death in women in the United States.
Historically, serious manifestations and associated complications of CAD in women were considered uncommon. This may explain, in part, why
women have received less intensive management and invasive treatment. For these reasons, and the fact that the early studies evaluating the efficacy of
CABG surgery focused primarily on men, the objective of more recent studies has been to determine whether female gender is an independent risk
factor for complications after interventional therapy. In the TIMI IIIB registry, the rates of myocardial infarction and death at 6 weeks after
thrombolytics and PCI for UA/NSTEMI were similar for women and men.[51] This was true even though the women were older and had significantly
more comorbidities (hypertension, diabetes). Multivessel disease was, however, less common in women: they had fewer critical coronary lesions with
60% or more stenosis, and their mean EF was higher. This may explain why fewer women (19%) underwent CABG surgery than men (27%). Among
the CABG surgery patients, the 6-week mortality rate, however, was higher for women (albeit the total number of deaths in the study was only 7). When
age was factored into a multivariate analysis of the data, gender was not linked to outcome.
In contrast, examination of the Society of Thoracic Surgeons (STS) database in two separate studies revealed that the operative mortality rate was
higher in women, namely 3.15% versus 2.61%.[52] [53] The database consisted of 97,153 women and 247,760 men. In this analysis, the mean left
ventricular function was better in women; they received fewer ITA grafts, were older, and had more comorbidities (diabetes, hypertension, peripheral
vascular disease).
Similar findings were observed in a retrospective age-stratified analysis of 51,187 patients (women = 15,178, 29.7%) in the National Cardiovascular
Network database.[54] In this study, women experienced a nearly twofold increase in hospital mortality after CABG surgery (5.3% vs. 2.9%). Gender-
based adjusted mortality, however, decreased inversely with age. Specifically, although women younger than 50 years of age had a twofold increase in
mortality, this differential decreased with age and disappeared in patients older than 79 years of age. Women also experienced more postoperative
complications such as renal failure (5.0% vs. 4.0%), neurologic complications (5.3% vs. 3.8%), and postoperative myocardial infarction (1.7% vs.
1.3%). Although these findings suggest that women do experience more complications and are at a higher risk of death after CABG surgery, it is
unclear why the mortality was inversely related to age and the complication occurred most frequently in women younger than 50 years of age. It is also
important to recognize that retrospective studies using a voluntary database have inherent limitations.
In a prospective study by Vaccarino and coworkers,[55] women were found to be older, underwent urgent operation more commonly (64.3% vs. 56.9%),
had a higher angina score, and presented more frequently with UA and congestive heart failure. Women also received fewer total bypass grafts and ITA
grafts. Although the hospital course for women and men were similar, women had a higher rate of hospital readmissions and more persistent problems
such as angina, dyspnea, incisional infection, decreased SF-36 physical function scores, and depressive symptoms. Thus, although still controversial, the
preponderance of evidence indicates that female gender is an independent risk factor for increased morbidity and mortality after CABG surgery.
Renal Disease
Renal insufficiency is also an independent risk factor for survival after CABG surgery. A serum creatinine level higher than 2.0 mg/dL is associated
with a twofold increase in mortality.[56] It has been estimated that approximately 14% of patients undergoing CABG surgery have some degree of renal
insufficiency when it is defined as a serum creatinine level higher than 1.5 mg/dL.[57] In one retrospective study[58] of 59,576 patients who underwent
either CABG surgery or PCI, a survival benefit with CABG surgery in patients with a serum creatinine level higher than 2.5 mg/dL was demonstrated.
The one-, two-, and three-year survival rates were 84.1%, 77.4%, and 65.9% for CABG surgery compared to 70.8%, 51.9%, and 46.1% for PCI. This
survival differential was not attributed to differences in left ventricular function, severity of CAD, and incidence of comorbidities. In another
retrospective study[59] of 15,784 hemodialysis-dependent patients undergoing CABG surgery, PTCA alone, and PTCA with stent,
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there was also a survival advantage for CABG surgery. Although early mortality was higher for CABG surgery (8.6%) versus PTCA (6.4%) or stenting
(4.1%), mortality equalized at 6 to 9 months. At 2 years, survival was demonstrably better with CABG surgery. Compared to PTCA, CABG surgery
provided a 20% reduction in death risk, whereas PTCA with stenting provided only a 6% survival advantage. This effect was more dramatic in diabetic
patients, in which CABG surgery was associated with a 27% lower risk of death. Thus, although CABG surgery in patients with renal insufficiency and
failure is associated with increased morbidity and mortality, CABG surgery is associated with better survival when compared to PCI.
Obesity
Obesity is a known risk factor for CAD, diabetes, hypertension, and stroke and is associated with a 50% to 100% higher risk of all-cause mortality when
compared to age-matched peers. Thus it is not surprising that obesity is generally assumed to be a risk factor for adverse events after CABG surgery.
However, contrary to various assumptions, there is a lack of agreement as to whether obesity per se is an independent predictor of mortality. In one
retrospective, multicenter study of 11,101 patients undergoing CABG surgery,[60] the mortality was similar in nonobese (body mass index [BMI] < 31),
moderately obese (BMI 31 to 36), and severely obese patients (BMI > 36). Although sternal wound infections were more frequent in the moderately and
severely obese patients, the incidences of bleeding complications and cerebral vascular accidents were the same. In contrast, an adjusted multivariate
analysis of data in the STS National Cardiac Database revealed that operative mortality was elevated in both the moderately and severely obese patient.
[61] In addition, the incidences of postoperative renal failure, prolonged ventilation, and sternal wound infection were also significantly higher. In this
analysis, obesity was defined as normal/mild (BMI < 35), moderate (BMI 35 to 39.9), and extreme (BMI ≥ 40). Thus, although obesity may affect
morbidity, its impact on mortality is unclear. This may be due to the marked variability of the definition of obesity, a reliance on anecdotal experience,
and the observational nature of the studies to date.
Reoperation for Coronary Artery Disease
Within 5 years, 15% of CABG surgery patients experience a recurrence of symptoms, typically angina. This increases to about 40% within 10 years.
Recurrent symptoms almost always indicate progression of disease in the native coronary circulation or graft disease. In most cases the indications to
proceed with coronary angiography, PCI with or without stenting, and/or repeat CABG surgery are the same as for the first operation. Patients who are
considered candidates for reoperative CABG surgery are usually older, have more diffuse CAD, and have diminished ventricular function. Factors that
increase the risk of reoperation include the absence of an ITA graft, younger age at the time of primary surgery, prior incomplete revascularization,
congestive heart failure, and New York Heart Association class III or IV angina.[62] Reoperative CABG surgery differs from that of the primary
procedure in that care is taken to avoid injury to the patent grafts. Manipulation of the old grafts is kept to a minimum to avoid distal coronary bed
microembolization. The mortality of reoperative CABG surgery may exceed that of primary CABG surgery; in some series it has been reported to be as
high as 10%. Although reoperative CABG surgery can be performed safely, overall patient survival and freedom from angina over time are diminished.
Maximal survival and freedom from reoperation are best achieved in patients by aggressive management of risk factors such as diabetes mellitus,
hypercholesterolemia, hypertension, and smoking.
Complications of CAD Amenable to Surgery
A region of the ventricular wall that is akinetic or dyskinetic and results in a reduction in left ventricular EF is termed a ventricular aneurysm. Surgical
treatment is designed to improve ventricular geometry and thus function, and often includes the use of prosthetic materials to restore normal ventricular
geometry and chamber volume and normalize ventricular wall tension. The incidence of ventricular aneurysm after AMI has been reported to be as high
as 35%. This has been declining due, in part, to the early and aggressive application of interventional therapies. Ninety percent of left ventricular
aneurysms are the result of a transmural myocardial infarction secondary to an acute occlusion of the LAD. Patients may develop an aneurysm as early
as 48 hours after infarction, but most patients do so within weeks. Approximately two thirds of patients who develop ventricular aneurysms remain
asymptomatic. The 10-year survival rate of these patients may exceed 90%. In contrast, the 10-year survival for symptomatic patients is less than 50%.
The most common causes of death are arrhythmias (>40%), congestive heart failure (>30%), and recurrent myocardial infarction (>10%). Mortality is
influenced by the patient’s age, onset of heart failure, extent of CAD, presence of mitral regurgitation, incidence and types of ventricular arrhythmias,
and reduced left ventricular function. The risk of thromboembolism is low, and long-term anticoagulation is not recommended, with the exception of
those patients who have evidence of a mural thrombus. The diagnosis is usually made by echocardiography. Thallium imaging or PET is useful in
detecting the extent of the aneurysm and viability of adjacent regions. In general, patients with symptoms of angina, congestive heart failure, and/or who
have refractory arrhythmias should be considered candidates for CABG surgery and resection of the aneurysm. Patients with a contained rupture and/or
evidence of a false aneurysm should undergo surgery soon after the diagnosis is made since these have a tendency to rupture spontaneously. Patients
experiencing thromboembolic events despite anticoagulation are also candidates for surgery. The most common cause of postoperative death is heart
failure. The 5-year survival rate after surgery has been reported to range between 60% and 80%. In general, surgical repair/resection in conjunction with
CABG
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surgery results in angina relief and resolution of heart failure symptoms for most patients.
Another complication of AMI is a postinfarction ventricular septal defect. This occurs in approximately 5% of patients and is associated with an acute
vessel occlusion. The defect is more common in men (3:2) and typically presents within 2 to 4 days of the infarction. The VSD is usually located in the
anterior or apical aspect of the ventricular septum. About 25% of patients present with a defect in the posterior aspect of the ventricular septum. This is
more commonly associated with an inferior wall myocardial infarction and is secondary to an occlusion of the RCA system or a distal branch of the
LCA. Approximately one third of the patients have evidence of a transient AV conduction block prior to the onset of septal rupture. A new, loud
systolic cardiac murmur after a myocardial infarction suggests the diagnosis and is an indication for echocardiography. The echocardiogram is effective
in determining the size and character of the VSD, as well as the degree of left-to-right shunting. Right heart catheterization typically shows a step-up in
oxygen saturation levels in the right ventricle and pulmonary artery. Once the diagnosis is established, patients should undergo immediate left heart
catheterization to characterize the degree of CAD, the magnitude of left ventricular dysfunction, and presence of mitral valve insufficiency.
Approximately 60% of patients with an infarction VSD have significant CAD in an unrelated vessel. The mortality rate in the untreated patient is high,
with 25% of the patients dying within 24 hours from refractory heart failure. Patient survival at 1 week, 1 month, and more than 1 year is 50%, 20%,
and less than 10%, respectively. Patients who are considered candidates for surgery should be managed early with closure of the defect and concomitant
CABG surgery. In the absence of refractory heart failure and hemodynamic instability, the survival rate may be as high as 75%.
Ischemic mitral regurgitation (IMR) may occur early or late and, depending on the severity of left ventricular dysfunction, may be life threatening.
Approximately 40% of patients who sustain an AMI develop IMR that is detectable by color-flow Doppler echocardiography. In 3% to 4% of the cases
the degree of mitral regurgitation is moderate or severe. Acute IMR may occur as a result of a papillary muscle necrosis and rupture due to occlusion of
overlying epicardial arteries that give rise to penetrating vessels that supply the papillary muscles. The posterior papillary muscle is involved three to six
times more often than the anterior muscle, and either the entire trunk of the muscle or one of the heads to which chordae attach may partially or totally
rupture. Another cause of IMR is ischemic papillary muscle dysfunction. The pathogenesis of acute and chronic IMR in the absence of papillary muscle
rupture is not completely understood but appears to be related to deformations of ventricular geometry. Patients usually present with chest pain and
shortness of breath and evidence of pulmonary edema, hypotension, and a heart murmur that radiates into the left axilla. The chest radiograph shows
signs of pulmonary congestion with interstitial pulmonary edema and cardiomegaly. A right heart catheterization demonstrates elevated pulmonary
artery pressures with prominent V waves, low mixed venous oxygen saturation, and a low cardiac output. Transthoracic echocardiography or TEE is
often diagnostic, but left heart catheterization is helpful in defining coronary artery anatomy. In most cases, prompt surgical intervention provides the
best chance for survival. Predictors of in-hospital death include congestive heart failure, renal insufficiency, and multivessel CAD. Emergent surgical
treatment usually involves mitral valve replacement and concomitant CABG surgery. The hospital mortality may be as high as 50%, although in
selected patients the mortality may be as low as 10% to 15%. The operation for chronic IMR is usually performed on an elective basis and more often
consists of complete myocardial revascularization and mitral valve repair rather than replacement.

PREDICTION OF CABG SURGERY OUTCOMES USING RISK STRATIFICATION MODELS
Improvements in operative technique and the anesthetic management of cardiac surgery patients have resulted in the application of CABG surgery to
sicker and more complex patients. Since there are few absolute contraindications to CABG surgery, it is important to understand hospital mortality and
morbidity in the context of preoperative risk. This has resulted in the creation of risk stratification models that are designed to provide a better
understanding of variations in institutional outcomes and a more accurate prediction of short-term mortality and morbidity. Examples of risk-adjusted,
multi-institutional databases include the Department of Veterans Affairs (VA) Continuous Improvement in Cardiac Surgery Program, the STS National
Database, and the Northern New England (NNE) Database. Risk stratification outcomes are provided to both participating institutions and surgeons.
This information is then used as a screening tool to evaluate and improve quality of care.
Risk-adjusted databases are particularly helpful in identifying and prioritizing preoperative variables predictive of outcomes. A meta-analysis[63] of seven
large databases of patients identified 7 core (unequivocally related to operative mortality) and 13 level 1 (likely relation to short-term CABG surgery
mortality) risk factors. The core variables included acuity of operation, prior heart operation, age, EF, gender, severity of CAD, and presence of LMCA
disease. These variables were acknowledged as predictive of mortality after CABG surgery in all seven databases. The presence of elevated serum
creatinine levels, PTCA during index admission, recent myocardial infarction less than 1 week, history of angina, ventricular arrhythmia, congestive
heart failure, mitral regurgitation, diabetes, cerebrovascular disease, peripheral vascular disease, chronic obstructive pulmonary disease, height, and
weight were considered less predictive.
To determine differences between voluntary and mandatory databases, a 2001 10-year comparative analysis of CABG surgery risk factors and outcomes
was performed
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using the voluntary STS National Database (n = 1.1 million) and the mandatory VA Continuous Improvement in Cardiac Surgery Program (CICSP) (n
= 74,000).[16] Although there were differences in demographics, both datasets produced similar risk factors, with similar odds ratios for 30-day mortality
after CABG surgery. The three strongest predictors of risk for mortality in the STS and VA databases were serum creatinine level higher than 3.0
mg/dL, need for preoperative IABP, and prior heart surgery. In the 1990s, CABG surgery mortality in both databases declined from 3.8% to 2.7% in the
STS registry and from 4.3% to 2.7% in the CICSP registry. This occurred despite an increase in preoperative risk factors in both databases. This
explains, in part, the significant decline over time in the observed-to-expected mortality ratios in both databases. These risk-adjusted databases provide
an opportunity for hospitals and surgeons to identify system problems and address them with the intent to improve outcomes and enhance patient care.
There are, however, subtle but important regional, institutional and provider variances that cannot be entirely accounted for in a generalized model of
risk stratification.

ALTERNATIVE METHODS FOR MYOCARDIAL REVASCULARIZATION
Off-Pump Coronary Artery Bypass Grafting Surgery
The use of CPB is reportedly associated with a whole-body inflammatory response (WIR), a response mediated, in part, by activation of complement,
macrophages, and cytokines. This phenomenon has been related to the contact of blood components with the surface of the bypass circuit. It has been
hypothesized that the WIR contributes to postoperative bleeding, neurocognitive dysfunction, thromboembolism, fluid retention, and reversible organ
dysfunction. In an attempt to minimize these complications, OPCAB surgery is used with increasing frequency and is currently considered an
acceptable alternative method for myocardial revascularization. A number of studies and clinical trials have been performed to evaluate the efficacy and
safety of this operation and define the subsets of patient who are most likely to benefit from this approach ( Table 59–7 ). The findings to date have been
mixed.
In one retrospective multicenter analysis of 7867 registry patients published in 2001,[64] OPCAB patients had a lower incidence of IABP use (2.3% vs.
3.41%), lower incidence of postoperative atrial fibrillation (21.2% vs. 26.3%), and a shorter length of stay (5 vs. 6 days) compared to CABG surgery
with CPB. The incidences of stroke, mediastinitis, and bleeding requiring reoperation were similar, however, and there was no difference in mortality,
namely 2.5% for off-pump and 2.6% for on-pump. In another observational study involving 1570 patients, OPCAB surgery was associated with less
blood loss, higher postoperative hemoglobin levels, fewer blood transfusions, and the length of stay in the ICU and hospital was shorter.[65] In this study,
there was no difference in perioperative myocardial infarction, use of inotropic agents, incidence of postoperative atrial fibrillation, neurologic
complications, prolonged ventilation, renal failure, or death. Thus, it appears that the operation may be a safe alternative to conventional CABG surgery
with CPB.
To address CPB-mediated WIR more directly, Ascione and associates conducted a prospective, randomized OPCAB surgery trial to examine the
relationship between biomarkers of WIR and postoperative morbidity and mortality in OPCAB surgery and CABG surgery patients (see Table 59–7 ).
Serum neutrophil elastase, IL-8, C3a, and C5a levels were higher in the CABG surgery with CPB group immediately after surgery. These elevated
levels were also associated with a higher incidence of infection, longer intubation time, greater blood loss, greater transfusion requirements, and longer
ICU and hospital length of stay. There was no difference, however, in the incidence of postoperative myocardial infarction, acute renal failure, stroke, or
death. The failure to demonstrate a reduction in the WIR and the incidence of death and myocardial infarction could have been due, in part, to the
relatively small number of patients studied. Alternatively, there may be no direct relationship between CPB-induced WIR, as assessed by certain
biomarkers, and death and myocardial infarction.
With respect to neurocognitive dysfunction, a prospective, randomized trial was performed to determine the possible relationship between the number
of high intensive transient signals (HITSs) using transcranial Doppler ultrasound (as a surrogate marker of cerebrovascular microemboli) in patients
undergoing conventional CABG surgery versus OPCAB surgery patients.[66] The results suggested that the occurrence of microemboli and incidence of
cognitive impairment were increased in the patients subjected to CPB. The median number of HITSs in the OPCAB patients was 11 versus 394 in the
patients undergoing conventional CABG surgery. This correlated with two of three neurologic tests, which indicated the incidence of neuropsychiatric
and neurocognitive dysfunction was greater in the on-pump CABG surgery patients. These findings support the hypothesis that OPCAB surgery is
associated with a reduction in the occurrence of microemboli and adverse neurocognitive outcomes. Using a different approach, Patel and colleagues[67]
studied 2327 consecutive patients and divided them into three groups: on-pump, off-pump with aortic manipulation (aorta used as source of graft
inflow), and off-pump without aortic manipulation (pedicle-based inflow). In this study, CPB was a risk factor for focal neurologic deficit, but there
were no differences in focal deficits between the OPCAB surgery patients with or without aortic manipulation. Although this study also supports the
concept that CPB may be associated with more neurologic events, it does not appear to be related to aortic manipulation as defined by the investigators.
Other investigators, however, have not demonstrated superior neurocognitive protection with OPCAB surgery (see Table 59–7 ). Whether OPCAB
surgery results in greater cerebral protection will have to await the results of the VA
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TABLE 59-7 -- Representative Prospective, Randomized Studies Comparing Results of OPCAB Surgery to CABG Surgery with CPB
Study, No. of Patients (N), and Follow-
up (F/U) Reference Purpose Findings
LOW-RISK PATIENTS
Beating Versus Arrested Heart Eur J Cardiothorac Evaluated efficacy and safety OPCAB is safe and effective and associated with
Revascularization: Evaluation of Surg 15:685–690, reduction in troponin I release.
Myocardial Function in a Prospective, 1999
Randomized Trial N = 80 F/U = 1 week
On-Pump Versus Off-Pump Ann Thorac Surg Analyzed postoperative renal Glomerular filtration, as assessed by creatinine
Revascularization: Evaluation of Renal 68:493–498, 1999 function clearance and microalbumin/creatinine ratio, was
Function N = 50 F/U = 1 week significantly reduced below preoperative levels in
CABG patients compared with OPCAB subjects at
24 and 48 hours after surgery. There were no
instances of acute renal failure, death, or myocardial
infarction in either group.
Economic Outcome of Off-Pump Ann Thorac Surg Safety and cost analysis OPCAB was safe and effective. On average, the cost
Coronary Artery Bypass Surgery: A 68:2237–2242, was 30% lower with OPCAB. Total mean cost per
Prospective, Randomized Study N = 200 1999 patient for operating materials, bed occupancy, and
F/U = 1 week transfusion requirements was $3731 for on-pump and
$2615 for off-pump.
Inflammatory Response After Coronary Ann Thorac Surg Effect of surgery on the OPCAB was associated with reduced inflammatory
Revascularization With or Without 69:1198–1204, inflammatory response response and postoperative infection (24 and 60
Cardiopulmonary Bypass N = 60 F/U = 1 2000 hours postoperatively).
week
Serum S-100 Protein Release and J Thorac Evaluated S-100 protein Brain and/or blood-brain barrier may be more
Neuropsychologic Outcomes During Cardiovasc Surg release up to 24 hours after adversely affected during CABG surgery with CPB.
Coronary Revascularization on the Beating 119:148–154, 2000 operation and This was not reflected in detectable neuropsychologic
Heart: A Prospective, Randomized Study neuropsychologic outcomes deterioration at 12 weeks.
N = 60 F/U = 12 weeks
Cognitive Outcome After Off-Pump and JAMA 287:1405– Analyze effect of procedures OPCAB patients had improved cognitive outcomes at
On-Pump Coronary Artery Bypass 1412, 2002 on cognitive outcome 3 months after surgery, but effects were limited and
Surgery N = 281 F/U = 3 and 12 months became negligible at 12 months.
Complete Revascularization in Coronary Ann Thorac Surg Evaluate the feasibility of OPCAB was safe and effective, but the rate of
Artery Bypass Grafting With and Without 71:165–169, 2001 CABG surgery without CPB incomplete revascularization was higher.
Cardiopulmonary Bypass N = 80 F/U = 2 to achieve complete
weeks revascularization
Early Outcome After Off-Pump Versus Circulation Evaluate cardiac outcome and OPCAB is safe and results in a similar short-term
On-Pump Coronary Bypass Surgery N = 104:1761–1766, quality of life cardiac mortality and quality-of-life outcome similar
281 F/U = 1 month 2001 to CABG surgery with CPB. Note that creatine
kinase-MB release was 41% less in the OPCAB
group.
HIGH-RISK PATIENTS
Different CABG Methods in Patients With Ann Thorac Surg Determine effect of different OPCAB procedures were more advantageous than
Chronic Obstructive Pulmonary Disease N 71:152–157, 2001 CABG techniques on on-pump procedures for patients with chronic
= 37 F/U = 2 months pulmonary function obstructive pulmonary disease.
CABG, coronary artery bypass grafting; OPCAB, off-pump CABG; CPB, cardiopulmonary bypass.
1877
Prospective Randomized Cooperative Study when it concludes in 2007.
Another rationale for performing OPCAB surgery is the potential for reducing the incidence of postoperative renal failure. In one prospective,
randomized trial, patients were studied to determine whether OPCAB surgery patients were at lower risk of elevations in creatinine clearance and the
urinary microalbumin/creatinine ratio in the immediate postoperative period.[68] In the OPCAB surgery patients, the values were lower and N-acetyl-β-
glucosaminidase levels, a sensitive marker of renal injury, were less (see Table 59–7 ). This difference was observed despite the use of mannitol and the
maintenance of normal mean arterial blood pressure in the patients undergoing conventional CABG surgery. Neither group, however, demonstrated any
clinical manifestations of renal failure. Thus, it is unclear whether OPCAB surgery actually reduces the risk of significant clinical renal failure
compared to CABG surgery with CPB.
Robotics
Rapid advances in technology have led to the application of robotic CABG surgery. Robotically assisted microsurgical systems have the theoretical
advantage of enhancing surgical dexterity and minimizing the invasive nature of conventional coronary artery surgery.[69] One major system currently in
use is the da Vinci system by Intuitive Surgical (Mountain View, CA). It consists of three major components: the surgeon-device interface module, the
computer controller, and the specific patient interface instrumentation. Both allow real-time surgical manipulation of tissue, advanced dexterity, and
optical magnification of the operative field via minimal access ports. Although only a few preliminary studies have been initiated, the results to date
suggest that CABG surgery can be safely performed with satisfactory graft patency rates. Current limitations include its lack of applicability to all
patients, prolonged operating room time, limited applicability to access all vessels, cost, and limited training opportunities.
Transmyocardial Laser Revascularization
Patients with UA and diffuse multivessel disease are candidates for transmyocardial laser revascularization (TMLR). This controversial surgical therapy
is employed for the surgical treatment of end-stage ischemic heart disease not amenable to percutaneous or conventional surgical operations.[70] [71] [72]
TMLR uses a high-energy laser beam to create myocardial transmural channels that were originally thought to provide direct access to oxygenated
blood in the left ventricular cavity. This is no longer considered the mechanism by which TMLR results in a reduction in symptoms of ischemic heart
disease. Although some local neovascularization has been documented, the magnitude of changes do not account for any substantive increases in
myocardial perfusion. Despite reports of anginal relief, SPECT 201 Tl imaging, PET imaging, and other perfusion studies have failed to show any
significant improvement in regional blood flow.[73] [74] One mechanism that has been proposed relates to a local effect on cardiac neuronal signaling. It
has been hypothesized that local tissue injury by TMLR damages ventricular sensory neurons and autonomic efferent axons, and this leads to local
cardiac denervation and anginal relief. Regardless, TMLR therapy is associated with a reproducible improvement in symptoms; patients undergoing
TMLR have shown a persistent improvement in angina class using the Canadian Cardiovascular System.[72] This improvement is achieved in 60% to
80% of patients within 6 months after the operation. The procedure is usually performed on patients in conjunction with other revascularization
procedures.

FUTURE DEVELOPMENTS
CABG surgery has evolved into a mature treatment modality for the management of patients with ischemic heart disease. It is now the safest and most
reliable method for completely revascularizing the ischemic heart and is associated with excellent medium- and long-term outcomes. Preclinical and
clinical studies are now underway to develop and evaluate new methodologies that will make the operation even safer and more effective. This includes
use of less-invasive techniques, testing of smaller extracorporeal circulation devices, developing methods to improve myocardial protection, and
techniques to enhance graft patency. With respect to less-invasive operative techniques, new enabling technologies are being developed to facilitate the
performance of more precise surgical maneuvers within more confined spaces. There are already a variety of bypass graft coupling devices under
investigation that are designed to facilitate proximal and distal coronary artery graft anastomoses. These include interrupted clips, magnetic docking
ports, and specialized metallic intracoronary stents. Hopefully, a usable device will be available within the next few years. Also, clinical trials are
underway to determine whether normalization of left ventricular geometry in patients with dilated ischemic heart disease will enhance the beneficial
effects of complete myocardial revascularization. The development of smaller, more efficient ventricular assist devices, without a propensity for
infection and thromboembolic complications, could lead to circulatory support systems that will obviate the need for orthotopic heart transplantation for
ischemic left ventricular dysfunction. In regard to myocardial protection, there is increasing evidence that even mild necrosis during the CABG surgery
operation (as measured by creatine kinase (CK) and CK-MB) occurs not only more frequently than previously appreciated but is also associated with a
decrease in medium- and long-term survival. This has led to renewed interest in developing more effective methods for protecting the heart. One such
strategy under intense investigation is to mimic the phenomenon of IPC pharmacologically.[75]
Finally, it is now known that vascular intimal hyperplasia (VIH) is an important component of vein graft occlusive disease. Gene-based therapies may
make it possible
1878
to transfect human saphenous veins prior to grafting and prevent VIH. It also may be possible to manipulate hs-CRP and slow the progression of the
atherosclerotic heart disease process in native coronary arteries. Likewise, since statins have been shown to increase survival in patients with CAD, it
may be possible to use these agents to promote endothelial protection and induce reversal of the inflammatory response cascade that leads to
atherosclerotic plaque formation. If all of these efforts are successful, it may be that the current survival benefit of CABG surgery to patients with
advanced CAD will be extended twofold within the near future.

Selected References
Allen Maycock CA, Muhlestein JB, Horne BD, et al: Intermountain Heart Study: Statin therapy is associated with reduced mortality across all age groups of individuals with
significant coronary disease, including very elderly patients. J Am Coll Cardiol 40:1777–1785, 2002.
Numerous clinical trials have demonstrated that statin therapy reduces the instance of myocardial infarction, stroke, and cardiovascular death among patients with
coronary artery disease (CAD). The results of this study revealed that statin therapy is associated with reduced mortality in all age groups of patients with advanced CAD,
especially very elderly patients. Since older patients are less likely to receive statin therapy, more aggressive statin use in this patient population may be warranted.
BARI Investigators: Influence of diabetes on 5-year mortality and morbidity in a randomized trial comparing CABG and PTCA in patients with multivessel disease: The Bypass
Angioplasty Revascularization Investigation (BARI). Circulation 96:1761–1769, 1997.
In 1987, the NHLBI initiated the BARI trial to compare the results of coronary artery bypass grafting (CABG) surgery and percutaneous transluminal coronary
angioplasty (PTCA) in patients with multivessel disease. One of the initial findings revealed that the mortality rate of patients with diabetes mellitus treated with oral
hypoglycemic agents or insulin was lower in patients who underwent CABG surgery than in PTCA patients. The present report examined cause-specific mortality and
CABG surgery efficacy by use of internal mammary artery (IMA) grafts versus saphenous vein grafts only. The investigators reported a much better 5-year survival with
CABG surgery compared to PTCA, which was due to reduced cardiac mortality (5.8% vs.20.6%, P = 0.0003). This was confined to those patients receiving at least one IMA
graft.
Chamberlain MH, Ascione R, Reeves BC, Angelini GD: Evaluation of the effectiveness of off-pump coronary artery bypass grafting in high-risk patients: An observational study.
Ann Thorac Surg 73:1866–1873, 2002.
In this nonrandomized study of 1570 consecutive patients, off-pump coronary artery bypass surgery (332 patients) was associated with less blood loss, fewer blood
transfusions, and a shorter length of stay in the intensive care unit and hospital when compared to conventional coronary artery bypass grafting surgery (1238 patients).
Despite these observations, there was no difference in neurologic complications or death between the two groups.
Fitzgibbon GM, Kafka HP, Leach AJ, et al: Coronary bypass graft fate and patient outcome: Angiographic follow-up of 5065 grafts related to survival and reoperation in 1388
patients during 25 years. J Am Coll Cardiol 28:616–626, 1996.
Coronary bypass graft disease and occlusion are common after CABG surgery and increase with time. The purpose of this study was to evaluate the long-term fate of
venous coronary bypass grafts angiographically and to correlate graft patency and disease with patient survival and reoperation. In this study the graft occlusion rate was
12% for 4592 saphenous vein grafts and 5% for 456 internal mammary artery (IMA) grafts early after operation. This increased to 51% for vein grafts after 12 years and
20% for IMA grafts. Overall survival from the time of the first bypass procedure was enhanced in patients who underwent reoperation.
Grover FL, Shroyer AL, Hammermeister K, et al: A decade’s experience with quality improvement in cardiac surgery using the Veterans Affairs and Society of Thoracic Surgeons
national databases. Ann Surg 234:464–472, 2001.
The objective of this report was to evaluate the similarities and differences between the Department of Veterans Affairs (mandatory) and the Society of Thoracic Surgeons
national (voluntary) risk-adjusted databases. Both databases showed a reduction in the risk-adjusted surgical death rate over the course of 10 years despite the fact that
patients presented with an increasing risk factor profile. Risk factors that predicted surgical death for coronary artery bypass grafting surgery were similar in the two
databases.
Higgins TL, Yared JP, Ryan T: Immediate postoperative care of cardiac surgical patients. J Cardiothorac Vasc Anesth 10:643–658, 1996.
This review article addresses important aspects of postoperative management of patients undergoing cardiac surgery. It emphasizes that postoperative care begins with the
preoperative visit and ends when the patient is ambulatory.
Parisi AF, Khuri S, Deupree RH, et al: Medical compared with surgical management of unstable angina: Five-year mortality and morbidity in the Veterans Administration Study.
Circulation 80:1176–1189, 1989.
Early reports regarding the role of coronary artery bypass grafting (CABG) surgery in the treatment of patients with unstable angina suggested that there was no
advantage of surgical therapy over medical therapy other than superior symptom relief. The purpose of this prospective, randomized Veterans Administration study was to
examine survival rates for the two groups of patients in the context of intermediate and long-term outcomes. Clinical presentations included (1) progressive or new-onset
angina relieved by medication and (2) prolonged bouts of angina poorly or incompletely relieved by medication. Of 468 patients, 237 were assigned to medical therapy and
231 to surgical therapy. Left ventricular function was abnormal in 134 patients and was defined as an ejection fraction less than 0.50. Five-year follow-up indicated a
superior survival rate with CABG surgery in patients with unstable angina and reduced ejection fraction or three-vessel coronary artery disease suitable for surgical
revascularization.
1879
Passamani E, Davis KB, Gillespie JF, Killip T: A randomized trial of coronary artery bypass surgery: Survival of patients with a low ejection fraction. N Engl J Med 312:1665–1671,
1985.
This report from the Coronary Artery Surgery Study sponsored by the National Heart, Lung, and Blood Institute was one of the first to demonstrate that coronary artery
bypass grafting (CABG) surgery for chronic stable angina was associated with a superior survival rate in a specific subset of patients when compared to medical therapy.
Specifically, the survival rate at the end of 7 years in patients with chronic stable coronary artery disease with triple-vessel disease and ejection fractions higher than 0.34
but lower than 0.50 was 84% with CABG surgery compared to 70% for medically treated patients. In patients with one-vessel and two-vessel disease, there was no
difference in survival rates between the two treatment groups.
Shah PK: Pathophysiology of coronary thrombosis: Role of plaque rupture and plaque erosion. Prog Cardiovasc Dis 44:357–368, 2002.
The development of a coronary thrombus is the immediate cause of most acute coronary syndromes of unstable angina, acute myocardial infarction, and sudden death. The
major mechanism appears to be plaque rupture. This article addresses triggers for plaque rupture, consequences of plaque rupture, and the concept of plaque stabilization.
Sharma GV, Deupree RH, Luchi RJ, Scott SM: Identification of unstable angina patients who have favorable outcome with medical or surgical therapy (eight-year follow-up of the
Veterans Administration Cooperative Study). Am J Cardiol 74: 454–458, 1994.
In this report of the Veterans Administration Cooperative Study, the 468 patients who had been randomized in the Veterans Administration Cooperative Study of unstable
patients noted in the Parisi report40 were risk stratified and analyzed using angiographic criteria of the number of coronary arteries diseased and left ventricular ejection
fraction (LVEF). At 8 years’ follow-up, the investigators reported that medical therapy appeared to be the preferred therapy for unstable angina patients with only one-
vessel or two-vessel disease and normal LVEF, and surgical therapy was associated with a superior survival advantage in patients with unstable angina, three-vessel disease,
or abnormal LVEF.
Yusuf S, Zucker D, Peduzzi P, et al: Effect of coronary artery bypass graft surgery on survival: Overview of 10-year results from randomised trials by the Coronary Artery Bypass
Graft Surgery Trialists Collaboration. Lancet 344:563–570, 1994.
In this meta-analysis of six randomized foundation trials, the authors compared the strategy of initial coronary artery bypass grafting (CABG) surgery with that of initial
medical therapy for the treatment of patients with stable coronary heart disease. The findings demonstrated that initial CABG surgery is associated with lower mortality
than medical management with delayed surgery, especially in high-risk and medium-risk patients. The improvement in survival was greatest for patients with left main
coronary artery disease, intermediate for those with three-vessel disease, and least for those with one-vessel or two-vessel disease. Greater survival prolongation was also
found among patients with abnormal exercise test and abnormal left ventricular function.

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67. Patel NC, Deodhar AP, Grayson AD, et al: Neurological outcomes in coronary surgery: Independent effect of avoiding cardiopulmonary bypass. Ann Thorac Surg 74:400–406,
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68. Ascione R, Lloyd CT, Underwood MJ, et al: On-pump versus off-pump coronary revascularization: Evaluation of renal function. Ann Thorac Surg 68:493–498, 1999.
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72. Horvath KA, Aranki SF, Cohn LH, et al: Sustained angina relief 5 years after transmyocardial laser revascularization with a CO2 laser. Circulation 104:I81–84, 2001.
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74. Landolfo CK, Landolfo KP, Hughes GC, et al: Intermediate-term clinical outcome following transmyocardial laser revascularization in patients with refractory angina pectoris.
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75. Mentzer RM Jr: Does size matter? What is your infarct rate after coronary artery bypass grafting? J Thorac Cardiovasc Surg 126:326–328, 2003.

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Chapter 60 - Acquired Heart Disease: Valvular
David A. Fullerton M.D.

Alden H. Harken M.D.
Valvular heart diseases may be considered surgical diseases. Stenotic or regurgitant cardiac valves create hemodynamic demands on one or both
ventricles of the heart. The compensatory mechanisms of the ventricles permit the heart to tolerate these lesions for varying periods of time, sometimes
years, before surgical intervention is required. Significant valvular lesions, however, ultimately produce systolic and/or diastolic ventricular
dysfunction, leading to heart failure. As a general rule, surgery for stenotic valve lesions may be deferred until the patient develops symptoms.
Regurgitant valve lesions, however, may produce significant ventricular dysfunction before symptoms develop; surgery in patients who do not have
symptoms may be indicated. Among the heart’s valves, the aortic and mitral valves are by far the most likely to acquire disease and thus are the focus of
this chapter.

HISTORICAL PERSPECTIVE
Heart failure from mitral stenosis was well recognized by the late 19th century, and efforts at surgical correction began well before the heart-lung
machine was available.[1] As early as 1897, Samways suggested (but never acted on) the possibility of dilating the stenotic mitral valve. Based on his
own postmortem studies of rheumatic heart disease in London, Brunton in 1902 proposed surgical intervention for mitral stenosis by passing a dilator
through the wall of the left ventricle retrograde into the mitral valve orifice; his proposal was shunned by London physicians, and Brunton never tried
this maneuver. The concept, however, was applied 20 years later in Boston when the first report of successful surgical correction of mitral stenosis
appeared in 1923; Cutler and Levine reported successful relief of mitral stenosis by incision of the valve with a knife introduced through an apical left
ventriculotomy. In 1925, Soutter performed the first successful closed mitral commissurotomy at the London Hospital by introducing his index finger
through the left atrial appendage. Despite Soutter’s success, he received no more patient referrals, and another 20 years elapsed before the procedure
became widespread. In June 1948, Bailey in Philadelphia and Harken in Boston each performed a successful closed mitral commissurotomy. Thereafter,
it became widely used for mitral stenosis.
By the mid 1970s, the closed technique was supplanted by open mitral commissurotomy. Although closed mitral commissurotomy did achieve good
palliation of mitral stenosis for its era, open mitral commissurotomy offers several advantages. First, the valvuloplasty may be performed under direct
vision. The primary reason for failure of closed mitral commissurotomy is residual stenosis, not restenosis. In up to 75% of patients, the subvalvular
apparatus of the mitral valve contributes significantly to the stenosis. The open technique permits precise and maximal division of fused commissures as
well as fused chordae. In addition, calcium may be sharply débrided from the valve and any residual mitral insufficiency may be corrected at the time of
operation. Finally, the closed technique has the disadvantage of potentially dislodging a left atrial thrombus, resulting in intraoperative embolization and
stroke.
Surgical attempts to correct aortic stenosis also began in the early 20th century.[1] In 1912, Tuffier, in Paris,
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attempted transaortic digital dilatation of a stenotic aortic valve. In 1947, Smithy (who died of aortic stenosis at 43 years of age) and Parker at the
University of South Carolina described an experimental model of aortic valvotomy. Three years later in Philadelphia, Bailey reported successful aortic
valvulotomy by insertion of a mechanical dilator across the stenotic valve of patients to open fused commissures. In 1952, Hufnagel and Harvey, at
Georgetown University, placed the first prosthetic ball valve into the descending aorta of a patient with aortic insufficiency. Surgery on the aortic valve
under direct vision required the development of cardiopulmonary bypass by Gibbon in 1954. In 1955, Swann performed the first successful aortic
valvotomy using hypothermia and inflow occlusion. Initially, open aortic valve operations were limited to aortic valve commissurotomy and
débridement of calcified aortic valve leaflets. Harken, in Boston in 1960, and Starr, in Portland in 1963, however, reported replacement of the aortic
valve with a ball-valve prosthesis. In 1962, Ross, in London, successfully performed orthotopic homograft valve replacement. In 1967, Ross performed
the first pulmonary autograft procedure (Ross procedure) for correction of aortic stenosis. In the mid 1960s, stent-mounted porcine aortic valves were
implanted, but these formaldehyde-fixed valves rapidly degenerated. In 1974, Carpentier, in Paris, reported superior longevity of the glutaraldehyde-
preserved porcine valve.

DIAGNOSTIC CONSIDERATIONS
Valvular heart disease may be suggested by a patient’s history or by a heart murmur detected on physical examination. Regardless of the valve lesion in
question, echocardiography should be employed to assess the severity of the stenosis, regurgitation, or both. Information available from the
echocardiogram includes definition of valve anatomy, assessment of ventricular contractile function, determination of the magnitude of valve
regurgitation using color flow Doppler imaging, and determination of the severity of valve stenosis.
Transthoracic two-dimensional echocardiography is completely noninvasive and may provide the necessary information. If more information is needed,
transesophageal echocardiography may provide better definition of aortic and mitral valve anatomy; it is also a more sensitive imaging modality for
detection of mitral regurgitation.
Although most valve lesions may be accurately diagnosed by echocardiography, cardiac catheterization may be necessary to confirm the diagnosis or to
provide additional information pertaining to ventricular function. Before surgery, it may be necessary to exclude the presence of coronary artery disease.
Mitral or aortic valve areas may be determined at cardiac catheterization using the Gorlin formula,[2] which permits calculation of the valve area as
follows:
where C is an empirical constant: 44.5 for the aortic valve and 38 for the mitral valve.

MITRAL VALVE
Surgical Anatomy of the Mitral Valve
The normal function of the mitral valve is dependent on coordinated interaction of the mitral valve apparatus, which includes the mitral valve annulus,
the valve leaflets, the valve chordae tendineae, and the left ventricular papillary muscles. The normal mitral valve has two leaflets: the anterior (or aortic
leaflet) and the posterior (or mural leaflet). Two papillary muscles arise from the left ventricular wall; the posterior (or posteromedial) and the anterior
(or anterolateral). Each of the leaflets of the mitral valve is connected to each of the papillary muscles by tendons, the chordae tendineae.
The leaflets are suspended from the mitral annulus, a collagenous structure that encircles the orifice between the left atrium and ventricle. Although the
two leaflets have about the same surface area, they have very different shapes ( Fig. 60–1 ). The anterior leaflet is rectangular. Its base is attached to the
mitral annulus anteriorly, and the width of the base is about one third the circumference of the mitral annulus. This attachment of the anterior leaflet to
the mitral annulus extends to the aortic annulus through fibrous tissue, providing “fibrous continuity” between the aortic and mitral valves; the anterior
leaflet of the mitral valve is immediately visible as the surgeon looks down through the aortic valve. The posterior leaflet is rectangular, and its
attachment to the mitral annulus extends for about two thirds of the circumference of the mitral annulus. The two leaflets are separated by two distinct
commissures.
Figure 60-1 Anatomy of the mitral valve as it relates to other cardiac structures. Important surgical landmarks include the relationship of the mitral valve to the aortic valve, the
circumflex coronary artery, and the atrioventricular (AV) node. (From Buchanan SA, Tribble CG: Reoperative mitral replacement. In Kaiser LR, Kron IL, Spray TL [eds]: Mastery of
Cardiothoracic Surgery. Philadelphia, Lippincott-Raven, 1998, p 351.)
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There are three important surgical landmarks (see Fig. 60–1 ). First, the circumflex coronary artery runs along the epicardial surface of the heart
overlying the posterior mitral annulus. Just millimeters of left atrial muscle separate the artery from the annulus, making it susceptible to injury during
mitral valve surgery. Second, the aortic valve is in close approximation to the anterior leaflet of the mitral valve (aortomitral continuity). The
noncoronary leaflet of the aortic valve is therefore susceptible to injury during mitral surgery. Third, the atrioventricular node is located deep to the
posteromedial commissure of the mitral valve.
Mitral Stenosis
Etiology
Rheumatic fever is the principal cause of mitral stenosis, and about two thirds of patients with rheumatic mitral stenosis are female. Rheumatic fever
usually occurs in childhood or adolescence (mean age, 8 to 12 years) and creates an inflammatory infiltration of the myocardium and valves. Perhaps
because the disease afflicts young people and many years pass before symptoms are manifest, a prior history of rheumatic fever is often difficult to
confirm. As the mitral valve heals after acute rheumatic fever, the mitral apparatus may slowly become deformed, and the disease typically remains
asymptomatic for at least 10 years; symptoms most commonly appear during the patient’s third or fourth decade of life. Healing of the inflammation
from rheumatic fever ultimately causes the cusps and commissures of the mitral valve to thicken and fuse, with concomitant fusion and shortening of
the chordae tendineae. The structure of the valve apparatus then calcifies and narrows, becoming funnel shaped. Such thickening and fusion of the valve
not only creates stenosis but also often prevents complete closure of the valve. In fact, of all patients with rheumatic mitral valve disease, about half
have combined mitral stenosis and mitral regurgitation.[3]
Other causes of mitral stenosis that are far less common than rheumatic fever include malignant carcinoid, systemic lupus erythematosus, and
rheumatoid arthritis. Rarely, congenital malformation of the valve may cause mitral stenosis, and congenital mitral stenosis is almost never an isolated
congenital cardiac lesion.
Pathophysiology
The cross-sectional area of the normal mitral valve is 4 to 6 cm2 . A mitral valve area of 2 cm2 is considered “moderate” mitral stenosis, and an area of 1
cm2 is considered “severe” mitral stenosis. [4] Under normal conditions, there is no pressure gradient across the mitral valve, and the left atrial pressure is
normally less than 15 mm Hg. As the mitral valve becomes more narrowed, an increasing pressure gradient is required to move the blood across the
mitral valve from the left atrium into the left ventricle during diastole; a transvalvular gradient of 10 mm Hg indicates severe mitral stenosis. The
significance of the transvalvular gradient is that left atrial pressure progressively increases, as the mitral valve becomes more stenotic. In turn, the
increased left atrial pressure is transmitted retrograde into the pulmonary veins, pulmonary capillaries, and ultimately pulmonary arteries. A left atrial
pressure of about 25 mm Hg increases pulmonary capillary pressure enough to produce pulmonary edema.
The severity of obstruction across the valve is determined by the transvalvular gradient and the flow rate across the valve. The flow rate is a function of
both the cardiac output and the heart rate; because flow across the mitral valve occurs during diastole and diastole is shortened as heart rate increases, a
faster heart rate at any given cardiac output increases the transvalvular gradient and raises left atrial pressure. The contribution of the atrial contraction
(“kick”) to cardiac output is particularly important in mitral stenosis; it accomplishes as much as 30% of the transvalvular gradient. For these reasons,
the onset of symptoms is generally associated with exertional activities or with the onset of atrial fibrillation.
To maintain adequate left ventricular filling across a 1-cm2 valve, for example, a pressure gradient of 20 mm Hg is required. A normal left ventricular
end-diastolic pressure of 5 mm Hg results in a left atrial pressure of 25 mm Hg. Left atrial pressure rises farther if flow rate across the valve increases
(increased cardiac output), transit time across the valve is shortened (decreased diastolic time), or atrial kick is lost (atrial fibrillation).
Pulmonary hypertension is an important component of the pathophysiology of mitral stenosis and, when severe, may dominate the clinical picture. At
least three pathophysiologic mechanisms contribute to the pulmonary hypertension seen in long-standing mitral valvular disease: (1) increased left atrial
pressure transmitted retrograde into the arterial circulation, (2) vascular remodeling of the pulmonary vasculature in response to chronic obstruction to
pulmonary venous drainage (“fixed component”), and (3) pulmonary arterial vasoconstriction (“reactive component”).
Diagnosis
SYMPTOMS
Dyspnea is the principal symptom of mitral stenosis. Dyspnea is typically brought on with exertion or associated with the abrupt onset of atrial
fibrillation. The increased cardiac output or heart rate with exertion or the loss of atrial kick and tachycardia with atrial fibrillation result in an increased
transvalvular gradient. This, in turn, increases left atrial pressure, and the pulmonary veins and capillaries become engorged, producing the sensation of
dyspnea and promoting pulmonary edema. If the left atrium enlargement is sufficient to compress surrounding structures, the patient may complain of
dysphagia or hoarseness. Marked elevation in left atrial pressure may produce hemoptysis.
PHYSICALEXAMINATION
The left ventricle is typically normal in size, and the apex is therefore not displaced. The murmur of mitral stenosis
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is best heard at the apex. It is a low-pitched, rumbling diastolic murmur that is decreased with inspiration and increased during expiration; it may be
markedly decreased by Valsalva maneuver. An opening snap precedes the murmur, is heard at the apex, and represents the completed excursion of the
mitral valve leaflets. If the mitral leaflets are stiff or calcified, an opening snap may not be heard. In patients with pulmonary hypertension, signs of
elevated right ventricular and central venous pressure may predominate the clinical picture. Physical findings, such as distended neck veins,
hepatomegaly, ascites, and peripheral edema, combined with a loud pulmonary valve component of the second heart sound (P2 ) heard on cardiac
auscultation, all suggest significant pulmonary hypertension.
CHEST RADIOGRAPH
Several findings may be noted on the chest radiograph. The cardiac silhouette may be normal in size, but the left atrium is enlarged. The enlarged left
atrium may be seen as a double density behind the right atrium on the posteroanterior projection, or it may be seen to displace the left mainstem
bronchus superiorly. On the lateral projection, the enlarged left atrium may displace the esophagus posteriorly. Calcification of the mitral leaflets or the
mitral annulus may be seen. Pulmonary venous hypertension should be suspected when the pulmonary arteries are enlarged and there is cephalization of
pulmonary blood flow.
ECHOCARDIOGRAM
The echocardiogram is the principal tool used to confirm the diagnosis.[5] Using the echocardiogram, the mitral valve area may be determined by two
mechanisms. First, the mitral valve area may be determined directly from the echocardiogram by planimetry. Second, measurement of the velocity of
blood flow across the valve by Doppler echocardiography permits calculation of the transvalvular gradient. Because the transvalvular gradient persists
longer with greater stenosis of the valve, the time required for the transvalvular gradient to decline may be measured and is referred to as the pressure
half-time. The mitral valve area may then be calculated using the following formula:
CARDIAC CATHETERIZATION
Mitral stenosis may also be diagnosed by cardiac catheterization. In fact, before undergoing surgical correction of mitral stenosis, cardiac catheterization
should be performed in patients with a history of angina and in those who are older than 40 years of age to exclude coronary artery disease. At the time
of cardiac catheterization, left atrial pressure may be determined directly (by transatrial puncture) or inferred from pulmonary capillary wedge pressure.
Simultaneous measurement of the left ventricular diastolic pressure permits calculation of the transvalvular gradient; a transvalvular gradient of greater
than 10 mm Hg is consistent with significant mitral stenosis. Using the Gorlin formula, the mitral valve area (MVA) may be calculated as follows:
where ∆P is the mean diastolic transvalvular gradient (mm Hg), F is the mean diastolic mitral flow in milliliters per second (derived from the measured
cardiac output and a determination of diastolic duration), and 38 is a constant.
Natural History
The natural history of mitral stenosis has been altered by successful surgical intervention. Data collected from the era before widespread surgery for
mitral stenosis, however, indicate that after diagnosis, the mean survival among patients with asymptomatic mitral stenosis was 15 to 20 years; on the
other hand, patients with symptoms had a mean survival of only 2 to 7 years.[6] Left atrial distention predisposes to atrial fibrillation and its associated
intra-atrial thrombus formation. As many as 20% of patients with mitral stenosis and atrial fibrillation may sustain systemic embolization, especially
strokes.
Treatment
The symptom-free patient in sinus rhythm requires only prophylaxis against bacterial endocarditis.[5] When symptoms appear, medical treatment of
mitral stenosis includes diuretics to lower left atrial pressure and efforts to maintain sinus rhythm with β-blocking agents or calcium-channel blocking
agents. Digoxin is helpful in controlling ventricular rate in patients who do go into atrial fibrillation. Patients in atrial fibrillation should be
anticoagulated with chronic warfarin sodium (Coumadin) therapy because the risk for systemic embolization is high.
Mechanical relief of mitral stenosis should be considered when patients develop symptoms, when evidence of pulmonary hypertension appears, or when
the mitral valve area is reduced to about 1 cm2 . Other conditions that should prompt surgical consideration include systemic embolization, worsening
pulmonary hypertension, and endocarditis. The options for mechanical relief of mitral stenosis include balloon mitral valvuloplasty, open surgical mitral
valvuloplasty (commissurotomy), and mitral valve replacement.
BALLOON MITRAL VALVULOPLASTY
First performed in 1984, balloon mitral valvuloplasty has become the treatment of choice for selected patients with mitral stenosis.[7] Echocardiography
may be used to determine patients considered to be good candidates, including those with pliable valve leaflets but without valvular calcification or
deformation of the chordae tendineae. Contraindications to this procedure include the presence of moderate mitral regurgitation, thickening and
calcification of the mitral leaflets, and scarring and calcification of the subvalvular apparatus.[8] Performed in the cardiac catheterization suite under
fluoroscopic guidance, the
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technique entails advancement of one or two balloon catheters across the interatrial septum and inflation of the balloon within the stenotic mitral valve.
Balloon mitral valvuloplasty has provided good short-term and intermediate-term results in appropriately selected patients. Balloon inflation should
increase the mitral valve area to about 2 cm2 . This increase in mitral valve area is usually associated with a significant decline in left atrial pressure and
transvalvular gradient and with at least a 20% increase in cardiac output. The mortality rate associated with balloon mitral valvuloplasty is 0.5% to 2%.
Other risks associated with this procedure include systemic embolism, cardiac perforation, and creation of mitral regurgitation; the risk of each of these
complications is 1% to 2%. Increased pulmonary vascular resistance has been shown to normalize after successful balloon valvuloplasty. About 10% of
patients are left with a residual interatrial septal defect. Three years after balloon valvuloplasty, at least 66% of patients are free of subsequent
intervention. In appropriately selected patients, the results of balloon valvuloplasty compare favorably with surgical valvuloplasty.[9]
OPEN MITRAL COMMISSUROTOMY
Open surgical valvuloplasty (commissurotomy) permits careful examination of the mitral valve and the chordae tendineae under direct vision as well as
removal of left atrial thrombus. Because thrombus typically originates in the left atrial appendage, its orifice may be surgically oversewn from within
the left atrium, reducing the risk for subsequent embolization. The surgeon may then sharply divide fused commissures and leaflets, mobilize scarred
chordae, and débride calcification. Furthermore, reconstruction of the valve may eliminate preexistent mitral regurgitation. The presence of significant
mitral regurgitation, however, should prompt consideration of mitral valve replacement.
The mortality rate associated with open mitral valvuloplasty is less than 2%. [10] When performed in appropriately selected patients, the freedom from
subsequent mitral valve intervention is about 75% at 5 years.[9] Nonetheless, because of less procedure-related morbidity, balloon valvuloplasty is the
procedure of choice.
MITRAL VALVE REPLACEMENT
The mitral valve should be replaced when valvuloplasty is precluded by dense calcification of the leaflets or subvalvular apparatus or because of
concomitant mitral regurgitation. Regardless of whether a tissue or mechanical prosthesis is implanted, efforts should be made to preserve the continuity
between the left ventricular apex and the mitral annulus provided by the chordae tendineae. This may be readily accomplished by preservation of the
posterior leaflet of the native mitral valve.
The contribution of the mitral apparatus to left ventricular function has become appreciated in recent years.[11] [12] A mechanical advantage is afforded the
left ventricle by the connection of its apex (by way of the papillary muscles) to the mitral annulus through the chordae tendineae; elimination of this
connection by removal of the entire mitral apparatus leads to loss of left ventricular function. Convincing data from laboratory animals and humans
demonstrate that preservation of at least some of the chordae tendineae at the time of mitral valve replacement results in much better long-term left
ventricular function than mitral valve replacement with chordal separation. Therefore, if mitral valve replacement is required, efforts should be made to
preserve the posterior and, in some cases, the anterior leaflets of the native mitral valve.
The operative mortality rate associated with mitral valve replacement for mitral stenosis is 2% to 10%.[10] [13] Operative mortality is increased with
advanced age and the presence of coronary disease. Pulmonary hypertension typically resolves after valve replacement, but several weeks or months
may be required. The 5-year survival rate after replacement is 70% to 90%.[9] [10] [14]
Mitral Regurgitation
Etiology
Competency of the mitral valve requires an intact mitral valve apparatus. Abnormalities of any component of the mitral valve apparatus may produce
mitral regurgitation: the mitral leaflets, the chordae tendineae, the mitral valve annulus, or the papillary muscles. Worldwide, rheumatic fever remains
the most common cause of mitral regurgitation; it results in deformity and retraction of the leaflets and shortening of the chordae. The leaflets may be
perforated by trauma or infective endocarditis. Calcification of the mitral annulus may result in annular rigidity and may prevent valve closure, and
mitral annular dilatation resultant to left ventricular dilatation may likewise preclude leaflet apposition during systole. Chordal rupture may result from
trauma, endocarditis, rheumatic fever, or diseases of collagen formation; chordae to the posterior leaflet rupture more frequently than those to the
anterior leaflet. Mitral valve prolapse is found in about 2% of the U.S. population, and up to 5% of patients with mitral valve prolapse develop mitral
regurgitation secondary to chordal elongation or rupture. Coronary arterial disease may produce infarction of the papillary muscle, resulting in mitral
regurgitation. Infarction in the distribution of the anterior descending coronary artery may necrose the anterolateral papillary muscle, whereas the
posteromedial muscle may infarct if blood flow through the posterior descending coronary artery is interrupted. Mitral regurgitation resultant to
myocardial infarction typically presents as a new murmur several days after infarction.
Pathophysiology
The regurgitant mitral valve offers an alternative route by which blood may exit the left ventricle. During both isovolumetric contraction and systole,
blood is preferentially ejected into the low-pressure left atrium. The volume of the regurgitant flow (regurgitant fraction) is dependent on the size of the
regurgitant orifice and the pressure gradient between the left ventricle and left atrium.
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Increased left ventricular afterload or decreased forward left ventricular stroke volume increases left ventricular pressure and thereby increases the
pressure gradient between left ventricle and atrium. The mitral valve annulus is enlarged by dilatation of the left ventricle. Therefore, the size of the
regurgitant orifice is increased by diminished left ventricular contractility as well as increased left ventricular preload and increased afterload. Because
the valve leaks during systole, the volume of regurgitant flow also increases as heart rate (number of systoles per minute) increases.
The compensatory mechanism by which the left ventricle adapts to maintain an adequate systemic blood flow (forward cardiac output) is volume
overload; it must pump the combined volume of systemic and regurgitant flows ( Fig. 60–2 ). Volume overload leads to cardiac dilatation as well as left
ventricular hypertrophy. Because the left ventricle ejects into the reduced resistance of the left atrium, parameters of systolic function (ejection fraction)
are increased in mitral regurgitation. As with aortic insufficiency, however, the left ventricle ultimately fails with chronic volume overload. In fact,
normal parameters of systolic function indicate significant contractile dysfunction of the left ventricle. An ejection fraction of less than 40% in the
setting of mitral regurgitation indicates significant left ventricular contractile dysfunction.
As in mitral stenosis, left atrial hypertension results from mitral regurgitation. This pressure is transmitted retrograde into the pulmonary circulation and,
if high enough, produces pulmonary hypertension. The magnitude of the left atrial pressure is a function of the compliance of the left atrium. Normal or
low compliance of the left atrium, such as may occur in acute mitral regurgitation, results in a relatively rapid rise in left atrial pressure. On the other
hand, chronic, left atrial volume overload that develops slowly may create significant enlargement of a compliant left atrium with relatively low left
atrial pressure.
Figure 60-2 Pathophysiology and compensation for acute and chronic mitral regurgitation. A, With acute mitral regurgitation, end-diastolic volume (EDV) increases from 150 to 170
mL. Because the left ventricle ejects blood into both the aorta and the left atrium (LA), end-systolic volume (ESV) decreases from 50 to 30 mL. The ejection fraction therefore
increases acutely; but because a significant percentage is ejected into the LA, the volume of blood flow into the aorta (forward stroke volume [FSV]) decreases from 100 to 70 mL.
The regurgitant volume into the LA increases LA pressure. B, Myocardial compensation for chronic mitral regurgitation includes eccentric left ventricular hypertrophy. Left
ventricular EDV increases from 170 to 240 mL. The larger ventricle results in an increased total stroke volume as well as FSV. Enlargement of the LA increases in capacitance, which
accommodates the regurgitant volume at a lower pressure. The left ventricular ejection fraction is supernormal. C, Ultimately, the heart decompensates and the contractile force (CF)
of the left ventricle declines; the ESV increases from 50 to 110 mL as FSV declines. The left ventricle dilates, which further compromises the ability of the mitral valve apparatus to
close; the regurgitant volume increases. The ejection fraction remains above normal until contractile function declines further. SL, sarcomere length; ESS, end-systolic stress; RF,
regurgitant fraction; EF, ejection fraction. (From Carabello BA: Mitral regurgitation: Basic pathophysiologic principles. Mod Concepts Cardiovasc Dis 57:53, 1988.)
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Diagnosis
SYMPTOMS
The symptoms of mitral regurgitation are those of heart failure: shortness of breath, dyspnea on exertion, orthopnea, pulmonary edema, and diminished
exercise tolerance. Symptoms are determined by the degree of mitral regurgitation, the rate of its progression, the degree of pulmonary hypertension,
and the magnitude of left ventricular contractile dysfunction. For example, patients with mild mitral regurgitation may remain symptom free for most of
their lives. At the other extreme, patients with acute, severe mitral regurgitation, such as may occur with endocarditis or a ruptured chordae tendineae,
may have pulmonary edema and require urgent surgery. The onset of atrial fibrillation does impair the patient’s functional status, but not to the same
degree as with mitral stenosis. With chronic, moderate to severe mitral regurgitation, patients may be symptom free for long periods of time. Lack of
symptoms, however, may be very deceiving because the contractile function of the left ventricle may be slowly deteriorating from volume overload.
When symptoms occur, left ventricular contractile dysfunction may be irreversible.
PHYSICAL EXAMINATION
On cardiac auscultation, a holosystolic murmur is heard best at the apex and radiates to the axilla and left scapular region. The pulmonary examination
may be significant for rales and bronchospasm caused by increased pulmonary interstitial fluid. In fact, mitral valve pathology should be considered in
the differential diagnosis of patients with adult-onset asthma.
ELECTROCARDIOGRAM
The electrocardiogram is notable for left atrial enlargement and, frequently, atrial fibrillation.
CHEST RADIOGRAPH
The chest radiograph is significant for cardiomegaly and left atrial enlargement. Pulmonary venous hypertension may manifest as cephalization of
pulmonary blood flow and pulmonary edema.
ECHOCARDIOGRAM
The diagnosis is confirmed by echocardiography. Transesophageal echocardiography is particularly effective in providing an anatomic explanation for
the regurgitation, such as perforated leaflets, poor leaflet coaptation, or ruptured chordae. Doppler echocardiography reveals a high-velocity jet of
regurgitant blood flow into the left atrium during systole.
Unfortunately, the determination of the severity of mitral regurgitation is only semiquantitative. The severity of the regurgitation is gauged as a function
of the distance from the mitral annulus that the jet can be visualized (e.g., into the pulmonary veins) and by the width of the regurgitant jet. The
regurgitation is scored subjectively on a scale from 1 (mild) to 4 (severe). The chronicity of the regurgitation may be inferred from the size of the left
atrium; an enlarged left atrium suggests chronic mitral regurgitation. Contrast ventriculography, performed at cardiac catheterization, likewise
demonstrates mitral regurgitation during systole.
Natural History
The natural history of the disease is variable, determined by the cause of mitral regurgitation, the regurgitant volume, and the magnitude of left
ventricular systolic dysfunction. Patients with mild mitral regurgitation typically remain symptom free for years and rarely go on to develop severe
mitral regurgitation. As is the situation with most valve diseases, the natural history of mitral regurgitation is obscure because surgical intervention has
effectively altered this history. In the presurgical era, however, about 80% of patients with severe mitral regurgitation survived 5 years and 60%
survived 10 years.[15] [16] Patients with combined mitral stenosis and regurgitation had a worse prognosis, with a 5-year survival rate of only 67%.
Treatment
The cornerstone of medical management is diuresis and afterload reduction with angiotensin-converting enzyme inhibitors. The importance of afterload
reduction cannot be overemphasized. Because blood leaving the left ventricle travels the path of least resistance, lowering systemic vascular resistance
increases systemic cardiac output. In the setting of heart failure from acute mitral regurgitation, intravenous vasodilators (nitroprusside) may be needed.
When a patient’s condition is stabilized, conversion to oral angiotensin-converting inhibitors may be achieved. Diuretics function not only to relieve
pulmonary edema but also to reduce left ventricular diameter. The size of the mitral annulus is thereby diminished and the regurgitant fraction reduced.
The indications for surgical intervention include symptoms despite medical management; severe mitral regurgitation in the presence of an identified
structural abnormality, such as a ruptured chorda tendinea; development of pulmonary hypertension; or evidence of deteriorating left ventricular
contractile function as determined by echocardiography or contrast ventriculography.
It is difficult to judge left ventricular function in patients without symptoms, making close follow-up with serial echocardiograms essential. In fact,
asymptomatic left ventricular dysfunction may develop insidiously. Two parameters of left ventricular function are useful in making the decision
regarding timing of surgery: ejection fraction (EF) and end-systolic diameter (ESD). Because mitral regurgitation lowers the total impedance against left
ventricular ejection, the EF should be supernormal in the presence of normal myocardial contractile function. An EF of less than 60% suggests
myocardial dysfunction, and operative mortality increases.[17] The other useful parameter of left ventricular function is the left ventricular ESD.[17] ESD is
less preload dependent than is EF,
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Figure 60-3 Left ventricular (LV) ejection fraction after mitral valve repair (white squares) versus replacement (black squares). Ejection fraction is greater at rest and with exercise
after repair. (From Tishler MD, Cooper KA, Rowen M, LeWinter MM: Mitral valve replacement versus mitral repair: A Doppler and quantitative stress echocardiograph study.
Circulation 89:132, 1994. Copyright 1994, the American Heart Association.)
and the information it implies is complementary. When left ventricular ESD exceeds 45 mm, the prognosis after surgery is worse.[18] [19] Even in the
absence of symptoms, therefore, patients should be referred for surgery when the left ventricular EF is less than 60% or when the left ventricular ESD is
more than 45 mm.[20]
In those cases, there are two surgical options: mitral valve repair or replacement. When possible, the valve should be repaired. The final decision about
which of these options to employ is made intraoperatively after valve inspection. Mitral valve repair has several advantages over replacement. First, left
ventricular function is better preserved after repair ( Fig. 60–3 ).[21] [22] Valve repair preserves the continuity between the mitral annulus and ventricular
papillary muscle provided by the chordae tendineae; this provides the left ventricle a mechanical advantage and optimizes its function. When the
chordae tendineae are sacrificed during a mitral valve replacement, the postoperative ejection fraction typically decreases ( Fig. 60–4 ). Therefore, even
when mitral valve replacement is necessary for mitral regurgitation, the chordae tendineae should be preserved if possible.[23]
Second, mitral valve replacement subjects the patient to the risks associated with the valve prosthesis, such as thromboembolism and the risk for
prosthetic valve endocarditis. Bioprosthetic valves may ultimately experience structural deterioration, and mechanical prosthetic valves obligate the
patient to lifelong anticoagulation with warfarin sodium. After mitral valve repair, patients in sinus rhythm do not require long-term warfarin sodium
therapy.
Third, the operative mortality rate associated with mitral valve repair (0% to 2%) is significantly less than that for replacement (4% to 8%).[10] Long-
term survival appears better with repair as well. These outcomes likely derive from the superior left ventricular function after mitral repair than after
replacement.
Figure 60-4 Postoperative ejection fraction after mitral valve replacement with (circles) and without (squares) preservation of the chordae tendineae. Ejection fraction decreases
significantly without chords severed but is preserved with choral preservation. (From Roseate JD, Carabello BA, Ushere BW, et al: Mitral valve replacement with and without
chordal preservation in patients with chronic mitral regurgitation. Circulation 86:1718, 1992. Copyright 1992, the American Heart Association.)
Significant left ventricular dysfunction has long been recognized as a significant risk factor for operative death after surgical correction for mitral
regurgitation. Recently, however, several investigators have achieved excellent results with mitral valve repair even in patients with severe heart failure
and left ventricular ejection fractions below 20%. [24] Functional status of the patients has been significantly improved, and the need for hospital
admission for treatment of heart failure has been markedly decreased. Preservation of the mitral apparatus at the time of repair is essential to achieve
those results.

AORTIC VALVE
Surgical Anatomy of the Aortic Valve
The normal aortic valve is composed of three thin, pliable leaflets, or cusps, attached to the heart at the junction of the aorta and the left ventricle. The
leaflets are attached within the three sinuses of Valsalva of the proximal aorta and join together in three commissures, which create the shape of a
coronet. Because the coronary arteries arise from two of the three sinuses of Valsalva, the aortic leaflets are named after their respective sinuses as the
left coronary leaflet, the right coronary leaflet, and the noncoronary leaflet. There are two important surgical landmarks. First, the commissure between
the left and noncoronary leaflets is positioned over the anterior leaflet of the mitral valve. Second, the commissure between the noncoronary and the
right coronary leaflets is positioned over the left bundle of His. Injury to this conduction bundle during aortic valve surgery may create heart block ( Fig.
60–5 ).
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Aortic Stenosis
Etiology
Acquired aortic stenosis usually results from calcification of the aortic valve associated with advanced age. Although the process is most often
idiopathic,[25] rheumatic fever may affect the aortic valve in a process similar to that of the mitral valve. In rheumatoid aortic stenosis, inflammation
produces adhesions and fusion of the commissures and leaflets with thickening and calcification. Retraction of the leaflets often makes these valves both
regurgitant and stenotic. The inflammatory process of rheumatic fever rarely involves the aortic valve alone, usually involving the mitral valve as well.
In idiopathic degenerative or senile aortic stenosis, grossly normal leaflets become calcified as a result of normal leaflet stress at the flexion points,
causing leaflet immobility. This calcification may extend down onto the anterior mitral valve leaflet or
Figure 60-5 Surgical anatomy of the aortic valve. The commissure between the noncoronary and the left coronary leaflets lies anterior to the left bundle of His. Injury to this
conduction tissue during aortic valve surgery may result in heart block. AV, atrioventricular.
upward along the aorta, occasionally causing coronary ostial stenosis.
Congenital valvular abnormalities may be clinically significant immediately after birth, as with unicuspid and dome-shaped valves. Patients born with a
congenitally bicuspid aortic valve are uncommonly symptomatic in childhood but are prone to develop aortic stenosis early in adulthood. The bicuspid
valve produces turbulent flow across the leaflets, leading to fibrosis, calcification, and stiffening. Patients with a bicuspid aortic valve are prone to
develop aortic stenosis at an earlier age (fifth and sixth decades of life) than those with a tricuspid valve (seventh, eighth, and ninth decades) ( Fig. 60–
6 ).
Pathophysiology
In acquired aortic stenosis, there is a chronic, progressive narrowing of the aortic valve. As the valve narrows, the appropriate compensatory response of
the left ventricle is hypertrophy. As the ventricle hypertrophies, it becomes stiffer as its compliance decreases; a higher left ventricular-end-diastolic
pressure is needed to maintain the same volume of cardiac output. To achieve a sufficiently high left ventricular end-diastolic pressure (diastolic
loading), the heart becomes increasingly dependent on the atrial kick; loss of the atrial kick, as occurs with atrial fibrillation, may result in a significant
decline in cardiac output and acute hemodynamic decompensation.
Although left ventricular hypertrophy is an appropriate biologic response to an increasing afterload, it has detrimental effects. The combined effects of
any of the following will culminate in increased myocardial oxygen demand: greater left ventricular muscle mass; decreased left ventricular compliance,
resulting in greater ventricular wall tension; higher systolic ventricular pressure; and longer systolic ejection time. At the same time, coronary artery
blood flow is compromised by increased wall tension compressing the vessels and by higher left ventricular diastolic pressure, which lowers the
coronary artery perfusion pressure. These factors contribute to inadequate coronary arterial perfusion of the subendocardium, leading to chronic
ischemia. In turn, chronic ischemia leads to cell death and fibrosis.
Figure 60-6 Causes of aortic stenosis as a function of age. (From Passik CS, Ackermann DM, Pluth JR, Edwards WD: Temporal changes in the causes of aortic stenosis: A surgical
pathologic study of 646 cases. Mayo Clin Proc 62;119, 1987.)
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Left ventricular hypertrophy may allow the heart to achieve a normal cardiac output under resting conditions.[26] To do so, however, a pressure gradient
across the valve is required, and, as the aortic valve area becomes smaller, the gradient across the valve from left ventricle to aorta increases. This
relationship of flow across the valve, valve area, and transvalvular pressure gradient is expressed in the Gorlin formula,[2] as follows:
where ∆P is the mean pressure gradient across the valve. Aortic valve flow (F) equals cardiac output in milliliters per minute divided by systolic
ejection period in seconds per minute. AVA is the aortic valve area in square centimeters, and C is an empirical orifice constant, 44.5.
For quick calculations, this simplifies to the following:
The relationship of flow across the aortic valve and the transvalvular pressure gradient is shown in Figure 60–7 . As the valve area decreases to 1 cm2 ,
there is little change in the transvalvular gradient needed to generate the same flow, and patients frequently experience no symptoms. With a valve area
of 0.8 cm2 , patients invariably develop symptoms.[5]
Diagnosis
SYMPTOMS
The classic symptoms of aortic stenosis are angina, syncope, and heart failure. Patients may not develop symptoms until the aortic valve area is about 1
cm2 ; this usually requires years. When this degree of stenosis has
Figure 60-7 Chart illustrates the relationship between the mean systolic pressure gradient across the aortic valve and the rate of flow across the aortic valve per second of systole, as
predicted by the Gorlin formula. As the valve area is reduced to about 0.7 cm2 , little increase in flow is achieved despite marked increases in mean gradient, thus defining “critical”
aortic stenosis. (From Hurst JW, Logue RB, Schlant RC, Wenger NK (eds): Hurst’s The Heart: Arteries and Veins, 3rd ed. New York, McGraw-Hill, 1974, p 811.)
been reached, however, it may quickly narrow farther, with rapid onset of symptoms and occasionally sudden death.
Auscultation of the chest in patients with aortic stenosis reveals a systolic murmur best heard at the base of the heart that radiates into the carotid
arteries; it may be difficult to distinguish the murmur of aortic stenosis from a bruit in the carotid artery. This murmur is associated with a slow,
prolonged rise in the arterial pulse, called pulsus parvus et tardus. The murmur of severe aortic stenosis is soft and high pitched and is often described
as a “sea gull” murmur.
ELECTROCARDIOGRAM
The electrocardiogram is notable for left ventricular hypertrophy in 85% of patients and evidence of left atrial enlargement in 80% of patients. T-wave
inversion and ST-segment depression are common.
CHEST RADIOGRAPH
The cardiac silhouette on the chest radiograph is usually normal but may reveal poststenotic dilatation of ascending aorta or calcification of the aortic
valve. Patients with symptoms of heart failure may have visible evidence of pulmonary edema.
ECHOCARDIOGRAM
The severity of aortic stenosis may be accurately estimated by echocardiography. The peak transvalvular gradient may be calculated from velocity of
blood traversing the valve by the following formula:
where V is the maximal measured blood velocity (in meters per second) across the valve. Echocardiographic determination of the velocity across the
valve may also be used to calculate the aortic valve area using the continuity equation ( Fig. 60–8 ). [27]
The most accurate measure of aortic stenosis is determined by cardiac catheterization. A catheter may be
Figure 60-8 Determination of aortic valve area using the continuity equation. For blood flow (A1 × V1 ) to remain constant when it reaches a stenosis (A2 ), velocity must increase to
V2 . Determination of the increased velocity V2 by Doppler ultrasound permits calculation of both the aortic valve gradient and solution of the equation for A2 . A, area; V, velocity.
(From Carabello BA: Aortic stenosis. In Crawford MH [ed]: Current Diagnosis and Treatment in Cardiology. Norwalk, CT, Appleton & Lange, 1995, p 87.)
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pulled back from the left ventricle to the aorta to determine the transvalvular pressure gradient. Simultaneous aortic and ventricular pressure
measurements are more precise, however, and they are, in fact, mandatory when the patient is in atrial fibrillation. Patients older than 40 years of age
should have coronary angiography before aortic valve surgery to exclude coronary artery disease.
Natural History
The natural history of aortic stenosis was reported by Ross and Braunwald.[28] Patient survival is not diminished until patients develop symptoms, which
is associated with reduction in the aortic valve area from the normal 3 to 4 cm2 to less than 1 cm2 . After symptoms develop, patient survival is limited.
The three principal symptoms of aortic stenosis are angina, syncope, and congestive heart failure ( Fig. 60–9 ).[28] Angina is usually the earliest
symptom, and the mean survival of a patient with aortic stenosis and angina is 4.7 years. When a patient experiences syncope, survival is typically less
than 3 years. Patients with dyspnea and congestive heart failure, in keeping with their associated left ventricular dysfunction, have a mean survival of 1
to 2 years. Congestive heart failure is the presenting symptom in nearly one third of patients.
Treatment
Aortic stenosis is a mechanical obstruction to flow from the left ventricle. The only effective therapy is aortic valve replacement. The existence of
symptoms is an indication for valve replacement. Angina and syncope warrant elective surgical therapy, whereas congestive heart failure mandates
urgent intervention. The issue of aortic valve replacement in patients with aortic stenosis who do not have symptoms is less clear. A small number of
symptom-free patients do precipitously develop symptoms and then experience sudden death. Investigators agree, however, that in patients with aortic
stenosis without symptoms, survival is excellent.[29] [30] [31] The risk for sudden death in symptom-free patients with a transvalvular gradient greater than
or equal to 50 mm Hg or a valve area of less than 0.5 cm2 is about 4% per year.[32] In one study of 113 symptom-free patients with critical aortic stenosis,
38 developed symptoms within 2 years. There were no sudden cardiac deaths in 118 patient-years of follow up.[31] To identify better those symptom-free
patients likely to develop symptoms, a group of 123 adults (mean age, 63 years) with asymptomatic aortic stenosis with an initial mean transvalvular
gradient of 30 mm Hg were prospectively followed. During 2.5 years of follow-up, there were no sudden deaths. Among patients with an initial
transvalvular velocity of more than 4 m/sec, however, only 21% were alive and free of valve replacement at 2 years of follow-up.[33] Therefore, aortic
valve surgery should be recommended to patients with symptomatic and asymptomatic disease who have evidence of left ventricular decompensation or
a transvalvular gradient of more than 4 m/sec.
In patients with good ventricular function, aortic valve replacement is associated with an operative mortality rate of 2% to 8%.[10] Independent
perioperative risk factors include age, left ventricular function, New York Heart Association class, and pulmonary function. After aortic valve
replacement, the projected 10-year age-matched survival rate is 80% to 85%.[34] Symptoms are relieved in nearly all patients; however, improvement in
ejection fraction and resolution of ventricular hypertrophy may require months to occur. Surgical mortality increases exponentially with decreasing left
ventricular ejection fraction. Aortic valve replacement in patients with congestive heart failure carries a mortality rate of up to 24%.[10] In patients with
aortic stenosis and coronary artery disease, valve replacement and myocardial revascularization should be performed concurrently. Perioperative
mortality is higher in patients who do not undergo simultaneous coronary artery bypass grafting.
For patients with severe aortic stenosis who are not candidates for aortic valve replacement, percutaneous aortic balloon valvuloplasty may provide
some palliation of aortic stenosis. In this procedure, either one or two balloon catheters may be passed through the aortic orifice and then inflated in an
effort to “crack” the calcium that is retarding leaflet motion. The immediate results show an increase in the aortic valve area of only 50%, with a 3% to
10% mortality rate. The long-term results are even more
Figure 60-9 The natural history of medically treated aortic stenosis. (From Ross J, Braunwald E: Aortic stenosis. Circulation 37:V61, 1968. Copyright 1968, the American Heart
Association.)
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disappointing: 30% to 35% of patients have recurrent symptoms within 6 months, and the mortality rate is 60% within 18 months after the procedure.[35]
There is a recurrence of symptoms, death, aortic valve restenosis, or a combination of these in more than half of patients within 6 months. The only
potential role of aortic balloon valvuloplasty may be in aged, frail, and possibly senile patients whose long-term survival is poor.
Aortic Insufficiency
Etiology
Aortic insufficiency may result from disease of the valve leaflets or of the aortic root. Rheumatic fever may affect the leaflets by shortening the distance
from the leaflet-free edge to the aortic annulus rather than by leading to commissural fusion. This prevents coaptation of the leaflets during diastole and
results in a central leak. Congenital bicuspid aortic valves typically lead to aortic stenosis but may become regurgitant if a leaflet prolapses. Endocarditis
may destroy leaflets.
Dilatation of the aortic root produces aortic regurgitation despite normal leaflet morphology by precluding leaflet coaptation. The most common of
these conditions is annuloaortic ectasia, an idiopathic dilatation of the aortic root and annulus; as the sinuses of Valsalva and the proximal aorta dilate,
diastolic coaptation of the leaflets is precluded, resulting in valvular insufficiency. Similarly, myxoid degeneration of the aortic root may lead to
dilatation of the root, as seen in Marfan’s syndrome, Ehlers-Danlos syndrome, and cystic medial necrosis. Those conditions may lead to leaflet
redundancy, progressive prolapse, and regurgitation. Trauma or dissection of the aortic wall may produce aortic regurgitation if it leads to loss of
commissural suspension and leaflet prolapse.
Pathophysiology
The aortic valve leaks during diastole, which lowers diastolic pressure and widens the pulse pressure. Because coronary blood flow occurs primarily in
diastole, the lower diastolic blood pressure lowers coronary perfusion pressure. Unlike aortic stenosis, in which the pathologic process is left ventricular
pressure overload, the pathophysiology of aortic insufficiency derives from left ventricular volume overload. The increased left ventricular end-diastolic
volume (preload) results from filling through the mitral valve as well as the incompetent aortic valve. Patients with chronic aortic insufficiency may
have the greatest left ventricular end-diastolic volume of any form of heart disease. Because left ventricular compliance is often increased, however, left
ventricular end-diastolic pressure may or may not be elevated. With left ventricular dilatation, normal forward stroke volume and ejection fraction may
be maintained by increased left ventricular end-diastolic and end-systolic volumes. According to the law of Laplace, this left ventricular dilatation
increases the left ventricular wall tension required to develop systolic pressure. Such increased wall stress not only increases myocardial oxygen
demand but also initiates left ventricular hypertrophy and increases left ventricular wall mass. Ultimately, myocardial fibrosis occurs.
With well-compensated aortic insufficiency, exercise may be tolerated because peripheral vascular resistance declines, lowering left ventricular
afterload and increasing effective forward flow. At the same time, heart rate increases, which shortens diastolic time, thereby decreasing the regurgitant
flow. Because the ventricle ultimately decompensates, however, the left ventricular end-diastolic volume increases even without an increase in aortic
regurgitant volume. The end-systolic volume increases as the forward stroke volume declines because ventricular emptying is impaired; the ventricle
fails ( Fig. 60–10 ).
In severe aortic regurgitation, increased myocardial oxygen demand exceeds myocardial oxygen supply, causing ischemia despite normal coronary
arteries. Increased left ventricular mass and wall tension occur concurrently with low diastolic pressures (low coronary perfusion pressure).
Consequently, and particularly with exercise when the diastolic period shortens, coronary blood flow may not meet demand.
Diagnosis
SYMPTOMS
The compensatory mechanisms of aortic regurgitation may permit patients to remain symptom free for long periods. When these compensatory
mechanisms begin to fail, however, left ventricular dysfunction becomes manifest, and patients experience symptoms of heart failure. Symptoms,
generally the result of an elevation in left atrial pressure, include dyspnea on exertion, orthopnea, and paroxysmal nocturnal dyspnea. Nocturnal angina
occurs occasionally as a result of a slow heart rate and an exceedingly low diastolic pressure with resultant poor coronary flow.
The physical examination of patients with aortic regurgitation is distinctive because of the wide pulse pressure. The peripheral pulses rise and fall
abruptly (Corrigan’s or “water-hammer” pulse), the head may bob with each systole (de Musset’s sign), and the capillaries visibly pulsate (Quincke’s
sign). Auscultation reveals a highfrequency decrescendo diastolic regurgitant murmur. A middle to late diastolic rumble may be heard (Austin-Flint
murmur) and represents rapid antegrade flow across the mitral valve that closes prematurely as a result of rapid ventricular filling secondary to the
aortic regurgitation.
CHEST RADIOGRAPH
The chest radiograph typically reveals an enlarged cardiac silhouette with an enlarged left atrial shadow and chronic aortic regurgitation. With acute
aortic regurgitation, however, the cardiac size may not be enlarged.
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Figure 60-10 A series of stress-volume loops is shown. As afterload is progressively increased, wall stress is increased such that ejection, which initially occurred at b, now occurs at
d, and then f. Stroke volume diminishes from b to c to d to e and then to f to g. At maximal wall stress h, there is no stroke volume but rather simply isovolumic contraction. On the
right, the inverse relationship between stroke volume and end-systolic wall stress is portrayed. The two negative slopes represent families of stroke volumes generated with a left
ventricular end-diastolic pressure of 5 mm Hg. (From Weber KT, Janicki JS, Shroff SG, Laskey W: The mechanics of ventricular function. Hosp Pract 18:113, 1983.)
ELECTROCARDIOGRAM
The electrocardiogram is usually nonspecific but may reveal left ventricular hypertrophy and left atrial enlargement.
ECHOCARDIOGRAPHY
Doppler echocardiography is the most accurate noninvasive technique to confirm the diagnosis of aortic regurgitation and to determine the severity of
aortic insufficiency. As with mitral regurgitation, the severity is graded semiquantitatively as mild, moderate, or severe.
The severity of the aortic regurgitation may be visualized angiographically at cardiac catheterization. As with echocardiography, the severity is graded
subjectively from mild to severe.
Natural History
Because of the compensatory mechanisms discussed previously, patients with chronic aortic regurgitation may be symptom free for long periods of
time. In fact, patients with mild to moderate aortic regurgitation have an excellent long-term prognosis; the 10-year survival rate after diagnosis is 85%
to 95%. Studies in which patients with severe aortic regurgitation have been included revealed a 70% 10-year survival rate and a 50% 20-year survival
rate. Once symptoms of congestive heart failure occur, survival is markedly decreased; almost 50% of patients with left ventricular failure die within 2
years.
Treatment
Medical therapy for aortic regurgitation is based on a combination of afterload reduction and diuretics. Afterload reduction with nifedipine has been
demonstrated to delay the need for aortic valve replacement.[20] [36] Chronic use of angiotensin-converting enzyme inhibitors is more common for
afterload reduction.
Patients with symptomatic aortic insufficiency require surgical therapy because their prognosis when treated medically is only a few years. Optimal
timing of surgical intervention in patients with or without symptoms, however, may be a very difficult clinical decision.[32] [37] Such patients may be
successfully managed with diuretics and afterload reduction for long periods of time. Significant irreversible left ventricular systolic dysfunction may
develop insidiously and before clinical evidence of congestive heart failure.
Therefore, symptom-free patients should be carefully followed noninvasively with serial echocardiography or radionuclide ventriculography for
evidence of systolic dysfunction or decreasing ejection fraction. Aortic valve replacement should be performed before the left ventricle has irreversibly
dilated. An end-systolic dimension greater than 55 mm Hg estimated by echocardiography has been associated with irreversible left ventricular
dysfunction even after aortic valve replacement,[32] [37] and aortic valve replacement should be performed before the ventricular dimension exceeds this.
At cardiac catheterization, the end-systolic volume may help in determining management for these symptom-free patients. When end-systolic volume is
less that 30 mL/m2 , prognosis after surgical therapy is excellent. Progressive systolic dysfunction
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with end-systolic volumes greater than 90 mL/m2 have poor intermediate short-term and long-term results. When left ventricular dysfunction is noted in
patients with diminished ejection fraction and good exercise tolerance, elective operation is recommended. Persistent medical management of these
patients severely jeopardizes surgical outcome and ultimate prognosis.[20]
The mortality rate associated with aortic valve replacement for aortic insufficiency is 4% to 6%.[10] Long-term survival is dependent on preoperative left
ventricular function. Both early and late results are improved when surgical intervention precedes left ventricular decompensation.

OPERATIVE TECHNIQUE
Aortic Valve Replacement
The standard incision for an aortic valve replacement is a median sternotomy. Once the incision has been made, the patient is connected to the
cardiopulmonary bypass circuit by cannulation of the distal ascending aorta and the right atrium. Myocardial protection is achieved by topical
myocardial cooling and retrograde cardioplegia. Most surgeons employ moderate systemic hypothermia (28°C to 32°C) during the operation.
After the heart fibrillates and the aortic cross-clamp has been applied, a transverse aortotomy is performed about 4 cm distal to the origin of the right
coronary artery. The aortotomy is extended to the left and right, thus exposing the aortic valve ( Fig. 60–11 ). The native aortic valve leaflets are
excised, with great care to remove any particles of calcium. Once the leaflets have been removed, an appropriately sized prosthetic valve is sewn in
place (see Fig. 60–11 ). The aortotomy is closed, cardiac function is resumed, and the patient is weaned from cardiopulmonary bypass.
Mitral Valve Replacement and Repair
The standard incision for mitral valve replacement is a median sternotomy, although a right thoracotomy may sometimes be appropriate for
reoperations. The patient is connected to the arterial limb of the cardiopulmonary bypass circuit by cannulation of the distal ascending aorta. Venous
drainage for the cardiopulmonary bypass is established by cannulation of the superior and inferior vena cavae (bicaval cannulation). Myocardial
protection is achieved by topical myocardial cooling and retrograde cardioplegia. Most surgeons employ moderate systemic hypothermia (28°C to 32°
C) during the operation.
Surgical exposure of the mitral may be particularly difficult and may be achieved by using several different incisions on the heart. The most common
incision used to expose the valve is a transverse left atriotomy made in the right lateral wall of the left atrium, just anterior to the left pulmonary veins.
An alternative surgical approach to the mitral valve is through an incision in the right
Figure 60-11 Aortic valve replacement. The diseased leaflets are excised (A), and the prosthetic valve is sewn in place with interrupted pledgeted mattress stitches (B and C). (From
Albertucci M, Karp RB: Prosthetic valve replacement. In Al Zaibag M, Duran CMG [eds]: Valvular Heart Disease. New York, Marcel Dekker, 1994, p 615.)
atrium, then through the interatrial septum, which provides excellent exposure to the left atrium and the mitral valve.
Once the mitral valve has been exposed, it must be carefully examined to determine whether it may be repaired or must be replaced. If the valve must be
replaced, efforts should be made to preserve the native mitral valve apparatus if possible to preserve the mechanical continuity between the mitral valve
annulus and the left ventricular apex. This may usually be accomplished by imbricating the leaflets of the mitral valve with sutures and placing an
appropriately sized prosthetic valve within the annulus of the native valve ( Fig. 60–12 ).
If the valve can be repaired, a variety of surgical techniques may be applied to restore valve competency. In most cases, an incompetent portion of one
or both of the mitral valve leaflets must be resected and the leaflet then reapproximated ( Fig. 60–13 ). At the time of mitral valve repair, the specific
pathology responsible for the regurgitation is addressed. For example, a common cause of mitral regurgitation is a ruptured chorda tendinea. At the time
of surgery, the prolapsed or flail leaflet subtended by the ruptured chorda tendinea is resected, the leaflet is primarily reapproximated, and the
circumference of the mitral annulus is reduced by use of an annuloplasty ring. The adequacy of the repair is judged under direct vision by filling the left
ventricle with saline under modest pressure. After the patient has been weaned from cardiopulmonary bypass, a final determination about the
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Figure 60-12 A to C, Mitral valve replacement with preservation of the posterior leaflet. This preserves the annular-apical connection by means of the chordae tendineae. (From
Albertucci M, Karp RB: Prosthetic valve replacement. In Al Zaibag M, Duran CMG [eds]: Valvular Heart Disease. New York, Marcel Dekker, 1994, p 613.)
competency of the repair is made with use of intraoperative transesophageal echocardiography. The durability of a given mitral valve repair is largely
dependent on the pathology responsible for the regurgitation. In most series, however, the failure rate of mitral valvuloplasty for mitral regurgitation is
less than 1% per year. The mitral annulus is invariably dilated in surgical cases of mitral regurgitation, contributing to poor coaptation of the anterior
and posterior mitral valve leaflets during systole. To return the enlarged mitral annular diameter to normal and to reinforce the leaflet repair, an
annuloplasty ring is sewn to the perimeter of the mitral annulus.

SURGICAL OUTCOMES
According to the Society of Thoracic Surgeons (STS) National Cardiac Surgery Database, approximately 70,000 valve operations are performed in the
United States annually.[38] The operative mortality rate for valve replacement
Figure 60-13 A to E, Example of mitral valve repair. In this example, the specific pathology is a flail posterior leaflet. It is repaired by resection of the flail segment, reapproximations
of the leaflet, and reduction of the mitral annulus circumference using an annuloplasty ring. (From Perier P, Clausnizer B, Mistarz K: Carpentier “sliding leaflet” technique for
repair of mitral valve: Early results. Ann Thorac Surg 57:383, 1994.)
TABLE 60-1 -- Operative Mortality Rates

AVR MVR AVR/CAB MVR/CAB
Society of Thoracic 4.0 6.0 6.8 13.3
Surgeons
New York Cardiac Surgery 3.3 6.2 7.1 12.8
Reporting System
Department of Veterans 3.9 5.9 7.3 11.8
Affairs
AVR, Aortic valve replacement; MVR, mitral valve replacement; CAB, coronary artery bypass grafting.
From Grover FL, Edwards FH: Similarity between STS and New York State databases for valvular heart disease. Ann Thorac Surg 70:1143, 2000.
surgery is influenced by several variables, including which valve is replaced, whether coronary bypass surgery is performed at the same operation, and
other patient-specific variables.
As shown in Table 60–1 , the operative mortality rate in the STS Database for isolated aortic valve replacement is approximately 4%. On the other hand,
the operative mortality rate for combined mitral valve replacement and coronary bypass grafting is much higher at 13%.[39] [40] Other databases, including
the New York State Department of Health Cardiac Surgery Reporting System and the Department of Veteran Affairs Cardiac Surgery Database, have
found very similar mortality rates for cardiac valve operations.
The inherent risks all surgical procedures is influenced by patient-specific risk factors, and large databases such as those just mentioned provide the
statistical power to identify patient-specific factors contributing to the risks of valve surgery. Table 60–2 lists some the major patientspecific risk factors
for the most common valve operations from the STS Database.[38]
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TABLE 60-2 -- Independent Risk Factors for Operative Mortality (Odds Ratios) for Valve Replacements
Risk Factor AVR AVR+CAB MVR MVR+CAB
Salvage status 7.12 7.00 6.39 3.40
Dialysis-dependent renal 4.32 4.60 4.74 1.83
failure
Emergency status 3.46 1.89 3.57 2.38
Non-dialysis-dependent 2.20 2.11 2.31
renal failure
First reoperation 1.70 2.40 1.45 1.31
AVR, Aortic valve replacement; MVR, mitral valve replacement; CAB, coronary artery bypass grafting.

CHOICE OF PROSTHETIC VALVES
For replacement of either the aortic or the mitral valve, there are two principal choices of cardiac valve prostheses: mechanical and bioprosthetic.
Bioprosthetic valves are either porcine valves or bovine pericardial valves. The hemodynamic performances of the valves are similar. The operative
risks associated with cardiac valve replacement are unassociated with the choice of prosthesis.
The choice of prosthetic valve must be patient specific. Mechanical valves have excellent durability and will perform indefinitely without structural
deterioration, but because they are thrombogenic, mechanical valves obligate the patient to lifelong anticoagulation (warfarin sodium). Hence, the
patient with a mechanical valve incurs the risks of chronic anticoagulation. Bioprosthetic valves do not require anticoagulation but will undergo
structural deterioration. The durability of a bioprosthetic valve is inversely related to the patient’s age at the time the valve is implanted. Should a
bioprosthetic valve structurally deteriorate, the patient will require reoperation and valve re-replacement. It is important to recognize that approximately
80% of all aortic and mitral valve replacements in the United States are performed in patients above the age of 60 years. The patient’s age should be
considered because it may be dangerous to commit a geriatric patient to chronic anticoagulation.
The 10-year survival for patients after aortic valve replacement ranges from 40% to 70%, with an average in the literature of 50%.[42] The type of
prosthesis does not impact survival, but other patient-specific factors such as age at operation and presence or absence of coronary artery disease do
impact survival after valve replacement. Regardless of the type of prosthetic valve implanted, approximately one third of patients die of valve-related
causes. An important consideration for the choice of valve for any patient is therefore how the individual patient may be affected by valve-related
morbidity or mortality.
As shown in Figure 60–14 , the principal causes of valve-related death after valve implantation include thromboembolism, reoperation, bleeding, and
prosthetic valve endocarditis. The leading cause of valve-related death is thromboembolism. Largely because mechanical valves are
Figure 60-14 Causes of valve-related deaths after valve replacement surgery. Of all deaths after valve surgery 29% are valve related and 71% are not valve related. Valve-related
deaths are attributable to thromboembolism, reoperation, bleeding, and prosthetic valve endocarditis (PVE).
thrombogenic, the risk of thromboembolism is greater with mechanical valves. At 10 years after aortic valve replacement, the risk of thromboembolism
is 20% for mechanical valves[43] and 9% for bioprosthetic valves.[44]
The risk of prosthetic valve endocarditis is not different between mechanical or tissue valves. It is approximately 4% spread over the patient’s lifetime.
However, if prosthetic valve endocarditis does occur, it is associated with a 50% mortality rate.[45]
The choice of prosthetic valve must consider the risks of anticoagulation (mechanical valve) and the likelihood and risks of reoperation for structural
valve deterioration (bioprosthetic valve). The risk of bleeding complications from chronic anticoagulation is between 1% and 2% per year. In fact, 4%
of valve-related deaths result from bleeding (see Fig. 60–14 ). Bioprosthetic valves are indicated in patients with contraindications to anticoagulation
because of occupation or because of coexistent medical conditions. Likewise, patients who are medically noncompliant or whose level of
anticoagulation may not be closely monitored should not receive mechanical valves. Ten percent of valve-related deaths result from reoperation, and
this fact steers some patients and physicians away from bioprosthetic valves. However, data demonstrate that if actual rather that actuarial statistical
methodology is used to evaluate the likelihood of reoperation for structural valve deterioration of a bioprosthetic valve, the incidence of reoperation is
less than 15% for patients older than 60 years ( Fig. 60–15 ). [46] A joint task force from the American Heart Association and the American College of
Cardiology has provided some recommendations to help balance these risks. The task force recommended that tissue valves be placed in the aortic
position in patients older than 65 years and in the mitral position in patients older than 70 years.[20]
An advance in the treatment of aortic valve disease in young patients is the pulmonary autograft procedure (Ross procedure).[46] Initially performed by
Ross in 1967,
1899
Figure 60-15 After aortic valve replacement with a bovine pericardial bioprosthesis, the risk of undergoing reoperation for structural valve deterioration (SVD) is less than 15% at 15
years. (From Banbury MK, Cosgrove DM III, White JA, et al: Age and valve size effect on the long-term durability of the Carpentier-Edwards aortic pericardial bioprosthesis. Ann
Thorac Surg 72:753, 2001.)
Figure 60-16 A to C, Pulmonary autograft (Ross) procedure. The diseased aortic valve and proximal aortic root are excised. The pulmonary valve and the main pulmonary artery
(autograft) are excised, and the autograft is used to replace the aortic root. The coronary artery buttons are reimplanted into the pulmonary root. A pulmonary homograft is then used
to reconstruct the right ventricular outflow tract. (From Kouchoukos NT, Davila-Roman VG, Spray TL, et al: Replacement of the aortic root with a pulmonary autograft in children
and young adults with aortic-valve disease. N Engl J Med 330:1, 1994.)
the procedure has gained wider acceptance during the past two decades. The procedure entails use of the patient’s own pulmonary root as an autograft to
replace the diseased aortic valve and root. A cryopreserved pulmonary homograft is then used to replace the patient’s pulmonary root ( Fig. 60–16 ).
Although it is a technically demanding procedure, the operative mortality rate associated with the Ross procedure is 5% or less and is not different from
that associated with isolated aortic valve replacement when performed by experienced surgeons.[48] Intermediate-term data suggest excellent function of
the pulmonary autograft; need for autograft reoperation is rare within the first postoperative decade.[49] The durability of the pulmonary homograft is
excellent; 80% of patients are free of homograft dysfunction at 16 years.[49] Chronic anticoagulation is not required, and the risk of valve-related
complications is extremely low.[50]

Selected References
Banbury MK, Cosgrove DM III, White JA, et al: Age and valve size effect on the long-term durability of the Carpentier-Edwards aortic pericardial bioprosthesis. Ann Thorac Surg
72:753, 2001.
This study highlights the importance of using actual rather than actuarial statistical methodology in the assessment valve-related events after prosthetic valve implantation.
The paper highlights the fact that even though bioprosthetic valves may structurally deteriorate, the likelihood of reoperation is low because a majority of patients may die
before that.
Bonow RO, Carabello B, De Leon AC, et al: ACC/AHA guidelines for the management of patients with valvular heart disease. J Am Coll Cardiol 32:1486, 1998.
This is a very comprehensive reference that addresses virtually all aspects of valvular heart disease, including indications for surgery.
Jamieson WRE, Edwards FH, Bero J, et al: Cardiac valve replacement surgery: The Society of Thoracic Surgeons national database experience. Ann Thorac Surg 67:43, 1999.
Using the power of the STS database, this report provides the foundation for risk-stratification for valve replacement surgery.
Ross J Jr, Braunwald E: Aortic stenosis. Circulation 38:V61, 1968.
This classic study provides the natural history of aortic stenosis.
Zellner JL, Kratz JM, Crumbly AJ III, et al: Long-term experience with the St. Jude Medical valve prosthesis. Ann Thorac Surg 68:1210, 1999.
The majority of valves implanted are mechanical valves. This report provides a comprehensive picture of valve-related morbidity and mortality after mechanical valve
implantation.

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Townsend: Sabiston Textbook of Surgery, 17th ed., Copyright © 2004 Elsevier