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Current Treatment Options in Cardiovascular Medicine

DOI 10.1007/s11936-012-0209-2

Valvular Heart Disease (AS Desai and PT O'Gara, Section Editors)

Diagnosis and Management


of Ebstein Anomaly
of the Tricuspid Valve
Eric V. Krieger MD1
Anne Marie Valente MD2,*
Address
1
Seattle Adult Congenital Heart Service, University of Washington Medical Center,
Seattle Children’s Hospital, 1959 NE Pacific Street, Seattle, WA 98195, USA
Email: ekrieger@u.washington.edu
*,2Boston Adult Congenital Heart and Pulmonary Hypertension Program, Brigham
& Women’s Hospital, Children’s Hospital Boston, Cardiology, 300 Longwood
Avenue, Boston, MA 02115, USA
Email: amvalente@partners.org

* Springer Science+Business Media, LLC 2012

Keywords Ebstein anomaly I Ebstein’s I Tricuspid valve I Congenital heart disease

Opinion Statement
Ebstein anomaly (EA) is a rare congenital heart defect that may not be detected until
late in adolescence or adulthood. Since the original description in a 19-year-old laborer
with severe tricuspid valve (TV) regurgitation in 1866, our understanding of this rare
condition has increased to the recognition that it is an abnormality not only of the TV,
but also of the right ventricle (RV). EA is the result of failure of delamination of the TV
leaflets from the interventricular septum, resulting in adherence of the leaflets to the
underlying myocardium. This results in a wide variety of abnormalities, including apical
and posterior displacement of the dilated TV annulus; dilation of the “atrialized” por-
tion of the RV; and fenestrations, redundancy, and tethering of the anterior leaflet of
the TV. The malformed TV is usually regurgitant, but may rarely be stenotic. The clinical
manifestations of EA in the adult depend on several factors, including the extent of TV
leaflet distortion, degree of tricuspid regurgitation (TR), right atrial pressure, and pres-
ence of a right-to-left atrial level shunt. Over the past several decades, advances in di-
agnostic imaging and surgical techniques have contributed to our current management
of this challenging congenital heart defect.

Introduction
Ebstein anomaly (EA) is a rare congenital heart defect cases of congenital heart disease. The etiology of EA is
that encompasses a wide spectrum of anatomical and not known; however, in rare cases, genetic factors such
functional abnormalities of the tricuspid valve (TV) as mutations in the transcription factor NKX2.5,
and right ventricle (RV). The incidence of EA is ~1 per 10p13-p14 deletion and 1p34.3-p36.11 deletion have
200,000 live births, accounting for less than 1 % of all been described. The morphologic defect of EA involves
Valvular Heart Disease (AS Desai and PT O'Gara, Section Editors)

a failure of delamination of the TV leaflets, resulting in ten symptomatic arrhythmia or right-sided heart failure
adherence of the leaflets to the underlying myocardium. symptoms. The physical examination findings may in-
This results in a wide variety of abnormalities, including clude a RV lift, loud first heart sound with systolic clicks
apical and posterior displacement of the dilated TV an- and a holosystolic murmur of TR. The jugular venous
nulus, dilation of the “atrialized” portion of the RV, pressure may often be normal, despite severe TR, due
and fenestrations, redundancy, and tethering of the ante- to the large, compliant right atrium. If an associated atrial
rior leaflet. The malformed TV is usually regurgitant, but level septal defect [Atrial septal defect (ASD) or patent fo-
may rarely be stenotic. Table 1 lists the clinical and mor- ramen ovale (PFO)] is present, the patient may be cya-
phological features of the unoperated EA patient. The notic due to right-to-left shunting. The cardiothoracic
clinical manifestations of EA in the adult depend on sev- ratio on chest radiograph is enlarged, transthoracic echo-
eral factors, including the extent of TV leaflet distortion, cardiography (TTE) confirms the diagnosis, and addi-
degree of tricuspid regurgitation (TR), right atrial pres- tional imaging modalities are used when appropriate.
sure, and presence of a right-to-left atrial level shunt. Ad- Adults with EA may not recognize their exercise limita-
ditional cardiac associations, such as pulmonary valve tions, and objective exercise testing is useful, particularly
stenosis or left ventricular (LV) non-compaction, may for documenting any cyanosis with exercise, due to
further contribute to a patient’s clinical presentation. a right-to-left atrial shunt. Atrial arrhythmias are
Asymptomatic patients with EA without right-to-left common, with accessory pathways present in
shunting or significant cardiomegaly may be managed ~50 % of these patients. Indications for surgical
medically and may not require intervention. When the interventions include symptoms of right-heart fail-
diagnosis of EA is made in adults, the presentation is of- ure or decreased exercise tolerance, cyanosis, para-

Table 1. Clinical features and evaluation of unoperated patient


The disorder has the following features in common:
• Adherence of the tricuspid valve leaflets to the underlying myocardium (failure of delamination)
• Apical displacement of the septal and posterior leaflets of the tricuspid valve below the AV
junction in the right ventricle
• Atrialization and dilation of the inflow of the right ventricle to varying degrees
• Redundancy, tethering, and fenestrations of the anterior tricuspid valve leaflet
• Varying degrees of TR
• Enlargement of the right atrium
• Varying degrees of cyanosis.
Associated lesions include the following:
• More than 50 % of patients have a shunt at the atrial level with either a PFO or secundum
ASD, which results in varying degrees of cyanosis
• One or more accessory conduction pathways, increasing the risk of atrial tachycardias
(approximately 25 %)
• VSD
• Varying degrees of anatomic and physiological RVOT obstruction
• Occasionally, other anomalies such as mitral valve prolapse
• Abnormalities of LV morphology and function.

With permission from Warnes CA et al. [1]


Reprinted with permission from Journal of the American College of Cardiology
Volume 52, Issue 23, 2 December 2008, Pages e143–e263
AV, atrioventricular
TR, tricuspid regurgitation
PFO, patent foramen ovale
ASD, atrial septal defect
VSD, ventricular septal defect
RVOT, right ventricular outflow tract
LV, left ventricular
Diagnosis and Management of Ebstein Anomaly Krieger and Valente

doxical embolism, progressive cardiomegaly, RV di- any interatrial communications, and antiarrhythmia
lation or dysfunction [1]. Surgical options include procedures. This article highlights the diagnosis and
tricuspid valve repair or replacement, closure of management of adults with EA.

Diagnostic Testing
Echocardiography
& Transthoracic echocardiography (TTE) is the cornerstone of imaging the
patient with Ebstein anomaly of the tricuspid valve. The goals of the TTE
are to a) distinguish EA from other etiologies of TV disease, b) deter-
mine TV anatomy c) assess the degree of TV dysfunction, d) evaluate
right ventricular (RV) function, and e) identify associated anomalies
such as an atrial septal defect, right ventricular outflow tract obstruction,
or myocardial non-compaction. TTE is essential in assessing the valve
morphology and eligibility for surgical repair [2]. While other types of
TV disease (rheumatic, endocarditic, traumatic, carcinoid, dysplastic
valve) can mimic EA, EA is unique in that the insertion site of the septal
leaflet is apically displaced≥0.8 cm/m2 relative to the anterior mitral
leaflet insertion site (Fig. 1). In more severe cases, the posterior leaflet is
apically displaced as well [3, 4]. These findings are usually best visual-
ized from the apical window. In EA, the septal leaflet has excessive
attachments to the interventricular septum as a result of incomplete
delamination of the septal and posterior leaflets from the myocardi-

Figure 1. Apical four-chamber echocardiogram of a patient


with Ebstein anomaly. The tricuspid valve is apically dis-
placed from the tricuspid valve annular plane (bracket). RA,
right atrium; RV, right ventricle; LA, left atrium; LV, left
ventricle.
Valvular Heart Disease (AS Desai and PT O'Gara, Section Editors)

um[5–7]. The septal and posterior leaflets can be thickened, dysplastic


and muscularized which complicate surgical repair.
& The anterior leaflet of the TV is not displaced but is usually elongated,
redundant, often fenestrated [8, 9], and may have restricted motion. It is
best imaged from the apical four-chamber view. Other findings, such as
severe tricuspid regurgitation (TR), severe right atrial or RV dilation and
dysfunction, are common in EA. Due to the apical displacement of the
septal and posterior leaflets, the functional TV annulus is apically dis-
placed, which directs the valve coaptation point anteriorly and, in severe
cases, into the right ventricular outflow tract [10]. This is best imaged
from a parasternal window, either in short-axis view or long-axis view
with anterior angulation of the transducer probe. The true annulus of
the TV is identified at the insertion site of the anterior leaflet. The se-
verity of apical displacement of the TV annulus and the amount of
atrialized RV has been correlated with worse outcomes [11]. Most
patients with EA have at least moderate TR. Tricuspid regurgitation se-
verity can be evaluated using color Doppler, and confirmed by flow
reversal in the hepatic veins. However, due to the eccentricity of the TR
jet and highly compliant right atrium, hepatic vein flow reversal is not
always seen, even in the setting of severe TR.
& Transesophageal echocardiography (TEE) may be helpful in deter-
mining TV morphology; however, because the valve is positioned
anteriorly, remote from the transducer, the visualization may be
limited. Intraoperatively, TEE is used routinely. Three-dimensional
echocardiography is useful in its ability to acquire an “en face” view
of the TV, and demonstrates not only the valve leaflet positions, but
also may demonstrate fenestrations in the leaflets and show tethering
of the leaflets to the RV free wall.
& Echocardiography should also attempt to evaluate RV function, al-
though this can be difficult due to its complex, non-geometric shape.
Several techniques to measure RV performance have been explored, but
none has gained universal acceptance. Right ventricular systolic func-
tion can be assessed by fractional area change using two-dimensional
echocardiography [12, 13], or by tricuspid annular plane systolic ex-
cursion measured by M-mode (TAPSE). Preserved TAPSE appears to be
associated with prolonged asymptomatic phase, even in the setting of
severe valve malformation [14]. RV systolic dysfunction has been as-
sociated with both early and late post-operative mortality [15].
& It is important to identify associated cardiac defects in patients with EA,
such as an intra-atrial communication (patent foramen ovale or secun-
dum type atrial septal defect), which is present in 49–75 % of patients [8,
16, 17]. Left heart abnormalities are increasingly recognized in association
with EA, and can include LV systolic dysfunction, diastolic dysfunction
and non-compaction [18–20]. Diastolic dysfunction may be related to
ventricular-ventricular interaction, whereby a volume-overloaded RV
compresses the LV, impairing filling. However, intrinsic abnormalities in
LV compliance are also present and do not entirely resolve after surgery
[21], and may be related to intrinsic myocardial stiffening or fibrosis.
Diagnosis and Management of Ebstein Anomaly Krieger and Valente

Cardiac Magnetic Resonance Imaging


& Cardiac magnetic resonance imaging (CMR) is well suited for the
evaluation of patients with EA as it has excellent spatial resolution, an
ability to clearly distinguish the blood-myocardial border, and proven
reproducibility in the measurement of RV size and function [22–24,
25••]. Measurements of atrial and ventricular volumes are typically
performed in axial or short-axis orientation with endocardial contours
drawn in end-systole and end-diastole. Steady state free precession
imaging should be performed in multiple planes, including two-
chamber and four-chamber orientations. Analysis of both the func-
tional RV and atrialized RV is performed. The functional RV is the
portion of the ventricle apical of the attachment points of the TV leaflets,
whereas the atrialized RV is the area between true tricuspid annular
plane and the functional tricuspid annulus [25••]. The ability to accu-
rately and reproducibly measure RV volume is important, as RV size has
been associated with outcomes in patients with EA [12].
& CMR also has an excellent ability to detect associated cardiovascular
abnormalities, including LV systolic dysfunction and non-compac-
tion [20, 26]. EA is often associated with thinning and dyskinesis of
the RV free wall. The degree of intracardiac shunting and TR can be
accurately and reproducibly measured using phase-contrast CMR
[27]. Late gadolinium enhancement can detect LV fibrosis, which
may lead to insights into the underlying myocardial abnormalities in
these patients, and this has prognostic significance, given the rela-
tively poor outcome of EA patients with LV dysfunction [28].

Exercise Testing
& While the optimal timing of elective surgery in patients with EA remains
unknown, symptoms are a common indication for surgery [1]. How-
ever, patients with congenital heart disease are often unaware of func-
tional limitations, and deterioration in exercise capacity has important
prognostic implications [29]. Even relatively asymptomatic patients
with EA have significant decreases in exercise performance and their
peak oxygen consumption is significantly reduced [29, 30]. Right-to-left
shunting can be exacerbated during exercise, and exercise-induced cy-
anosis can be a powerful impact on exercise capacity [31]. This can
manifest as inefficient ventilation and an elevated VE/VCO2 [30]. Pro-
gressive decline in exercise performance should be considered a relative
indication for surgery, even in the absence of overt symptoms [31].

Electrocardiogram
& The surface electrocardiogram (ECG) can give important clues about the
patient’s anatomy, presence of an accessory pathway and predisposition
for atrioventricular re-entrant tachycardia [32]. The surface ECG of a
patient with EA is rarely normal (Fig. 2). A wide P wave with a pro-
longed PR interval indicates slowed conduction through a dilated atri-
um [33]. Right bundle branch block is common [34]. However, patients
Valvular Heart Disease (AS Desai and PT O'Gara, Section Editors)

Figure 2. Twelve-lead electrocardiogram of a patient with Ebstein anomaly of the tricuspid valve. It demonstrates normal sinus
rhythm with a premature ventricular contraction, right axis deviation, complete right bundle-branch block with bizarre terminal
portion of the QRS (*) consistent with severe RV conduction delay.

may also have RV conduction delay associated with hypertrophy,


pseudonomalization of the right bundle branch block through a slow
accessory pathway [35], or overt preexcitation. The morphology of the
right bundle branch block and terminal portion of the QRS is bizarre
due to late activation through a scarred atrialized right ventricle [32].
& Wolff–Parkinson White syndrome, defined as manifest preexcitation,
is seen in as many as 20 % of EA patients. However, concealed ac-
cessory pathways are present in up to 50 % of patients [35–38]. The
majority of accessory pathways are right-sided and located in the
right posterior and right posteroseptal region [39••, 40]. Many
patients have multiple accessory pathways [10, 32, 38, 39••]. ECG
algorithms for precise accessory pathway localization have relatively
poor accuracy in patients with EA [41]. Patients with EA are at par-
ticular risk for primary atrial tachycardia due to severe right atrial
enlargement, and can be predisposed to rapid conduction down the
accessory pathway [42].
Diagnosis and Management of Ebstein Anomaly Krieger and Valente

Preoperative Electrophysiological Study


& Due to multiple potential arrhythmic substrates—which include
primary atrial tachycardia, orthodromic reentrant tachycardia, atrio-
ventricular node reentrant tachycardia and ventricular tachycardia—
recent data suggest that all EA patients that are planning to undergo
cardiac surgery undergo preoperative electrophysiology (EP study),
regardless of symptoms [34, 40, 43]. Localization of accessory
pathways is often challenging due to a massively enlarged right heart,
displaced tricuspid annulus and distortion of anatomic landmarks,
all of which can make catheter stability difficult [40].
& Percutaneous ablation of accessory pathways should be performed with
caution in those EA patients with a right-to-left interatrial communi-
cation (ASD, PFO) due to the risk of paradoxical embolus. The EP study
serves to individualize the surgical management of arrhythmias, as EA
patients may require focused cryoablation of atrial circuits, annular
accessory pathways, or sites of ventricular tachycardia. These focused
arrhythmia interventions are often combined with a right atrial Maze
procedure or bilateral Maze procedure for those with atrial fibrillation.

Cardiac Catheterization
& With advances in CMR and TTE, routine hemodynamic catheterization
is no longer warranted in most patients with EA. In the rare patient with
suspected pulmonary arterial hypertension, hemodynamic catheteri-
zation can be used for preoperative risk stratification. The presence and
magnitude of intracardiac shunting can also be determined by cathe-
terization. Preoperative coronary angiography is warranted in men
35 years and older, premenopausal women over 35 with coronary heart
disease risk factors, and post-menopausal women [1].

Treatment Options
Medical Management
& Patients with mild forms of EA should undergo regular evaluation by a
cardiologist with expertise in adult congenital heart disease (ACHD). The
evaluation must include thorough assessment of the patient’s heart
rhythm, as atrial arrhythmias are particularly common. Exercise testing
provides assessment of functional capacity and any arterial desaturation
with exertion. If signs or symptoms of right-sided heart failure are present,
standard heart failure medications are employed; however, there is little
evidence for the efficacy of these agents in patients with EA. Medical
management of arrhythmias should be individualized. Recommenda-
tions for physical activity are summarized in the Task Force 1 on Con-
genital Heart Disease [44]. Adults with mild EA, nearly normal heart size,
and no arrhythmias are not restricted from sports participation. However,
those with severe, unrepaired EA are precluded from sports.
& The late survival of unoperated adults with EA is less than expected. In a
natural history study of 72 unoperated EA patients, survival was 89 %,
Valvular Heart Disease (AS Desai and PT O'Gara, Section Editors)

76 %, 53 % and 41 % at 1, 10, 15 and 20 year follow-up, respectively.


Multivariate predictors of cardiac-related death included male gender,
cardiothoracic ratio greater than or equal to 0.65, and the severity of TV
leaflet displacement on TTE [45].
& Many women with EA have successfully carried full-term pregnan-
cies. In a literature review of 127 pregnancies in women with EA,
3.9 % were complicated by arrhythmias and heart failure was
reported in 3.1 % [46]. Women with EA should undergo pre-preg-
nancy counseling with a multidisciplinary team with expertise in
adult congenital heart disease (ACHD) [1].
& Antibiotic prophylaxis is usually not necessary in the acyanotic,
unoperated patient with EA. However, antibiotic prophylaxis before
dental procedures is reasonable in cyanotic EA patients, and recom-
mended in patients with a prosthetic heart valve.
Catheter Interventions
& Adults with EA may have varying degrees of right-to-left shunting
across an atrial level septal defect. Although there is a paucity of data,
percutaneous closure of these shunts in selected cases may reduce
cyanosis and improve functional capacity [47].
& EA patients with symptomatic arrhythmias should undergo electro-
physiological study and radiofrequency ablation by an electrophysiol-
ogist experienced in management of ACHD. The success rates for
catheter ablation in EA patients is lower than in those with structurally
normal hearts, and the risk of recurrent arrhythmia is increased [32, 38,
48]. Rarely, radiofrequency ablation in these patients has resulted in
right coronary artery stenosis [49]. The success rates for catheter ablation
in EA patients is lower than in those with structurally normal hearts, and
the risk of recurrent arrhythmia is increased [32, 38, 48].

Surgical Interventions
& Table 2 lists the ACHD Class I guidelines for tricuspid valve repair or re-
placement in EA patients, as well as indications for arrhythmia surgery [1].
& The operative management of patients with EA includes a) tricuspid
valve repair or replacement, b) right reduction atrioplasty, c) closure of
any atrial septal communications, d) antiarrhythmia procedures (Maze
procedure, surgical division of accessory conduction pathways, or cry-
oablation of atrioventricular node reentrant tachycardia), e) correction of
any associated cardiac anomalies (pulmonary stenosis) and f) consid-
eration of plication of the atrialized portion of the RV. Advantages to
plication of the RV include a reduction in the size of the nonfunctional
portion of the RV, theoretically improving the transit of blood through
the RV; b) reduction of LV compression and improving LV filling; c) re-
duction of the tension on the TV suture lines; and d) provision of more
space for the lungs (particularly in smaller patients). However, RV pli-
cation has the potential risk of injury to the right coronary artery that may
lead to arrhythmias and ventricular dysfunction. There is also concern
that the area of plication creates a folded area of tissue that may be par-
Diagnosis and Management of Ebstein Anomaly Krieger and Valente

Table 2. Recommendations for surgical interventions


Class I
1. Surgeons with training and expertise in CHD should perform tricuspid valve
repair or replacement, with concomitant closure of an ASD, when present, for
patients with Ebstein’s anomaly with the following indications:
a. Symptoms or deteriorating exercise capacity. (Level of Evidence: B)
b. Cyanosis (oxygen saturation less than 90 %). (Level of Evidence: B)
c. Paradoxical embolism. (Level of Evidence: B)
d. Progressive cardiomegaly on chest x-ray. (Level of Evidence: B)
e. Progressive RV dilation or reduction of RV systolic function. (Level of Evi-
dence: B)
2. Surgeons with training and expertise in CHD should perform concomitant
arrhythmia surgery in patients with Ebstein’s anomaly and the following
indications:
a. Appearance/progression of atrial and/or ventricular arrhythmias not ame-
nable to percutaneous treatment. (Level of Evidence: B)
b. Ventricular preexcitation not successfully treated in the electrophysiology
laboratory. (Level of Evidence: B)
3. Surgical re-repair or replacement of the tricuspid valve is recommended in
adults with Ebstein’s anomaly with the following indications:
a. Symptoms, deteriorating exercise capacity, or New York Heart Association
functional class III or IV. (Level of Evidence: B)
b. Severe TR after repair with progressive RV dilation, reduction of RV systolic
function, or appearance/progression of atrial and/or ventricular arrhyth-
mias. (Level of Evidence: B)
With permission from Warnes CA et al. [1]
Reprinted with permission from Journal of the American College of Cardiology
Volume 52, Issue 23, 2 December 2008, Pages e143–e263
CHD, congenital heart disease
ASD, atrial septal defect
RV, right ventricular
TR, tricuspid regurgitation

ticularly arrhythmogenic. Therefore, careful epicardial inspection is


warranted to minimize coronary injury during the plication procedure.
Tricuspid valve repair is usually preferred over TV replacement in patients
with EA. Bidirectional cavopulmonary shunt (Glenn procedure) may be
considered in selected EA patients with severe RV dysfunction and pre-
served LV systolic function and low left atrial pressure. Older EA patients
often have septal dyskinesis, likely due to the massively dilated RV, and
the LV ejection fraction may improve after surgery. EA patients with se-
vere LV systolic dysfunction rarely may be considered for heart trans-
plantation [50]. In considering surgery for adults with EA, it is important
to recognize that these patients may have undergone prior cardiac sur-
gery; comprising over 25 % of patients in the Mayo clinic experience. The
Mayo clinic recently reported low early mortality in a group of EA
patients950 years old; however, the long-term survival was less than
expected; suggesting that interventions in adults with EA may need to be
considered earlier [51••].
Valvular Heart Disease (AS Desai and PT O'Gara, Section Editors)

Tricuspid Valve Repair


& TV repair is preferable to TV replacement. Factors that are favorable
for reparability include a large, mobile anterior leaflet with a free
leading edge, delamination of the inferior leaflet, and the presence of
adequate septal leaflet tissue. Whereas, significant leaflet tethering
(adherence to the edge or body of the leaflet to the RV free wall) and
severe apical displacement of the coaptation point with TV leaflet
tissue in the right ventricular outflow tract make repair more difficult.
TV repair for EA was first described in 1958 by Hunter and Lillehi
[52]. In 1972, Danielson developed a repair that included a longi-
tudinal plication of the free wall of the atrialized RV, posterior tri-
cuspid annuloplasty and excision of the redundant RA wall. This
repair resulted in a monocuspid valve with the functional TV annulus
brought up to the true TV annulus, and competency of the valve is
achieved by coaptation of the anterior TV leaflet with the ventricular
septum [53]. Since that time, multiple modifications of the TV repair
technique have been described [54–56]. Two major modifications
have been to repair the valve in the position that it maintains in the
RV, by bringing the RV free wall papillary muscles down to the
ventricular septum, and to perform transverse RV plication. The
Mayo clinic reported the surgical outcomes of 539 patients with EA,
182 of which had undergone TV repair. Early mortality was 5 % for
the entire cohort, and freedom from late reoperation was 84 %, 73 %
and 56 % at 10, 15, and 20 years, respectively. Independent predic-
tors of death or reoperation included male gender, pulmonary valve
stenosis, significant postoperative LV dysfunction, prior cardiac pro-
cedures, or a Maze procedure [15, 57•]. A recent TV repair described
by da Silva known as the “cone reconstruction” has the advantage of
a near anatomic repair by coaptation of leaflet tissue with leaflet
tissue, similar to a normal TV mechanism. The reconstructed TV is
reattached at the true TV annulus at the atrioventricular junction. RV
plication reduces the size of the enlarged RV and reduces the tension
on suture lines. Relative contraindications to the cone reconstruction
include age950 years, moderate pulmonary hypertension, severe LV
dysfunction, or complete failure of delamination of septal and in-
ferior leaflets with poor delamination of the anterior leaflet [58]. Due
to severe true tricuspid annulus enlargement, an annuloplasty ring is
often placed [43]. In the early reported surgical experience of 40
patients who had undergone the cone reconstruction, there was one
operative mortality. At a mean follow-up of 4 years, one additional
patient had died and two required repeat TV surgery [58].

Tricuspid Valve Replacement


& When TV repair is not feasible, bioprosthetic valve replacement is a
reasonable alternative for adults with EA. Porcine bioprosthetic valves
are preferred over bovine bioprosthetic valves, as the latter tend to cal-
Diagnosis and Management of Ebstein Anomaly Krieger and Valente

cify at a faster rate in the TV position. Porcine valves are also preferred
over mechanical valves due to good durability in the TV position and
lack of need for warfarin anticoagulation [59]. In adults who require
warfarin anticoagulation for another reason (i.e., atrial fibrillation), a
mechanical valve may be considered, but should be avoided in cases of
significant RV dysfunction, due to a greater propensity of TV throm-
bosis. The surgical technique involved in TV replacement involves de-
viating the suture line toward the atrial side of the atrioventricular node
and membranous septum inferiorly to avoid injury to the conduction
system, as well as cephalad to the atrioventricular groove anteriorly to
avoid right coronary artery injury.

One and One-Half Ventricle Repair


& A bidirectional cavopulmonary shunt may be performed in select cases
of severe RV dysfunction or if the TV repair has resulted in significant
stenosis (mean gradient96 mmHg), which is often present in very cy-
anotic patients. By directing the superior vena caval blood to the branch
pulmonary arteries, this procedure reduces the hemodynamic stress by
reducing the volume of the RV by 35–45 %, depending on the patient’s
size. However, it is important to confirm that the pulmonary vascular
resistance and left atrial pressures are low in order for this strategy to be
effective [60, 61]. Disadvantages of the bidirectional cavopulmonary
shunt include pulsations of the head and neck veins, facial swelling, and
development of pulmonary arteriovenous malformations.
Summary
Ebstein anomaly is a rare, complex congenital heart defect with a broad
anatomical and clinical spectrum. A thorough understanding of the under-
lying pathology and associated defects is critical to caring for the adult with
EA. Newer imaging modalities are allowing increased understanding of the
mechanisms of TV and RV function, and surgical modifications continue to
be proposed in hopes of improving the long-term outcomes of patients with
EA.

Acknowledgments
The authors thank Amy Harmon, BA; Rebecca Schwaegler, BS, RDCS; Dr. Frank Cecchin and Dr. Pedro Del
Nido for their thoughtful contributions to this manuscript.

Disclosure

No potential conflicts of interest relevant to this article were reported.


Valvular Heart Disease (AS Desai and PT O'Gara, Section Editors)

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