Coarctation of the Aorta

P. Syamasundar Rao, MD

Address the media and superimposed neointimal tissue. The local-

Division of Pediatric Cardiology, The University of Texas/Houston ized constriction may form a shelf-like structure with an
Medical School, 6431 Fannin, MSB 3.130, Houston, TX 77030, USA. eccentric opening or it may be a membranous curtain-like
E-mail: P.Syamasundar.Rao@uth.tmc.edu
structure with a central or eccentric opening. The coarcta-
Current Cardiology Reports 2005, 7:425434
tion may be discrete, or a long segment of the aorta may be
Current Science Inc. ISSN 1523-3782
Copyright 2005 by Current Science Inc. narrowed; the former is more common. In the past, AC has
been described as preductal (or infantile) type and post-
ductal (or adult) type, depending upon whether the coarc-
Coarctation of the aorta is an important, treatable cause of
tation segment is proximal or distal to the ductus
secondary hypertension. Its prevalence varies from 5% to
arteriosus, respectively. However, a detailed review of the
8% of all congenital heart defects. This condition is most
anatomy suggests that all coarctations are juxtaductal.
often detected because of a murmur or hypertension found
Dilatation of the descending aorta immediately distal
on routine examination. Delayed or absent femoral pulses
to the coarctation segment, poststenotic dilatation, is usu-
and an arm/leg systolic blood pressure difference of 20 mm
ally present. Varying degrees of hypoplasia of the isthmus
Hg or more in favor of the arms may be considered as evi-
of the aorta (the portion of the aorta between the origin of
dence for aortic coarctation. The coarctation may be dem-
the left subclavian artery and ductus arteriosus) and trans-
onstrated on a suprasternal notch two-dimensional
verse aortic arch (the portion of the aorta between the ori-
echocardiographic view along with increased Doppler flow
gins of left common carotid and left subclavian artery) are
velocities across the coarctation site. Cardiac catheteriza-
present in the majority of patients with thoracic coarcta-
tion reveals significant systolic pressure gradient (> 20 mm
tion. Collateral vessels that connect arteries from the
Hg) across the coarctation and angiography demonstrates
upper part of the body to the vessels below the level of
the degree and type of aortic narrowing. Aortic obstruc-
coarctation may be seen; these may be present as early as a
tion may be relieved by surgery or by transcatheter tech-
few weeks of life.
niques; the latter include balloon angioplasty and stent
implantation. In the past, surgery has been used exclusively,
but because of morbidity and complications associated with
Prevalence
surgery, catheter techniques are increasingly used in the
The prevalence of AC was found to vary between 5% to 8%
management of aortic coarctation. Balloon angioplasty in
of all congenital heart defects [1,2]. Slight male preponder-
children and stents in adolescents and adults are becoming
ance has been observed in older patients, although it is
initial therapeutic options for management of coarctation.
minimal in infants.
Studies evaluating long-term follow-up results of the inter-
ventional techniques are needed.
Associated Defects
Bicuspid aortic valve may be seen in nearly two thirds of
Introduction infants with AC, whereas only 30% of older children may
Coarctation of the aorta is an important cause of secondary have such an anomaly. Mitral valve anomalies, although less
hypertension and consequently, inclusion of this entity frequent than those of the aortic valve, are also seen with
when discussing secondary hypertension is appropriate. In AC. Some patients with AC may have cerebral aneurysms,
this review, definition, pathology, prevalence, pathogenesis, predisposing them to development of cerebrovascular acci-
pathophysiology, clinical features, noninvasive and invasive dents with severe hypertension later in life. AC is the most
evaluation, treatment, and prognosis of aortic coarctation common cardiac defect seen in Turners syndrome.
(AC) in children, adolescents, and adults is discussed.

Definition and Pathology
Coarctation of the aorta is defined as a congenital cardiac
anomaly consisting of a constricted aortic segment com-
prising localized medial thickening with some infolding of
Pathogenesis
The exact mechanism by which AC is produced is not
clearly understood [3]. Two hypotheses are most com-
monly invoked: hemodynamic and ectopic ductal tissue. In
the first hypothesis, an abnormal preductal flow or abnor-
426 Hypertension
mal angle between the ductus and coarctation are invoked.
Spontaneous postnatal closure of the ductus arteriosus
completes the development of aortic obstruction. A high
incidence of AC in congenital heart defect patients with
decreased antegrade aortic flow in utero and virtual absence
of AC in patients with right heart obstructions would lend
credence to the hemodynamic hypothesis. Abnormal exten-
sion of ductal tissue into the aorta (ectopic ductal tissue)
has been postulated to create coarctation shelf, and with
ductal closure, development of aortic coarctation. This the-
ory, however, does not explain the variable degrees of isth-
mic and aortic arch hypoplasia seen with AC.
Pathophysiology
In children, adolescents, and adults, the mode of presenta-
tion is hypertension or systolic murmur. The aortic
obstruction is deemed to have developed slowly with evi-
dence for development of left ventricular hypertrophy
(LVH) and collateral circulation. The mechanism for devel-
opment of hypertension is not clearly understood;
mechanical obstruction and rennin-angiotensinmedi-
ated humoral mechanisms have been postulated [3].
Mechanical obstruction theory explains the increased
blood pressure by postulating that a higher blood pressure
is required to maintain flow through the coarcted segment
and collateral vessels. The stroke volume, ejected into the
limited aortic receptacle, produces a higher pressure proxi-
mal to coarctation. However, this theory does not explain
1) the lack of relationship between the degree of elevation
of blood pressure and the magnitude of obstruction, 2)
increased peripheral vascular resistance distal to the site of
obstruction, and 3) delayed or lack of reduction of blood
pressure following relief of obstruction.
Humoral theory postulates activation of the rennin-
angiotensin system secondary to reduction of renal blood
flow and appears to explain most of the clinical features.
However, measurement of plasma rennin activity both in
animal models and human subjects did not show consis-
tently elevated plasma rennin levels. The reason for the
inability to demonstrate elevation of rennin levels may be
related to lack of inadequate accounting for salt intake, pos-
ture, extracellular fluid volume, and sympathetic influences
on rennin release. More recent studies did demonstrate
abnormalities in rennin-angiotensin-aldosterone systems.
In addition, activation of central sympathetic nervous sys-
tem may also be responsible for hypertension of AC.
Clinical Features
Most often, the coarctation is identified because of a murmur
or hypertension detected on routine examination. The AC is
not frequently recognized by the primary care physician [4,5],
and consequently it is recommended that palpation of femo-
ral pulses and measurement blood pressure be undertaken
during routine examination to avoid delay in the diagnosis.
Palpation of the brachial and femoral artery pulses simulta-
neously will reveal decreased and delayed or absent femoral
pulses. Blood pressure in both arms and one leg must be
determined; a pressure difference of more than 20 mm Hg in
favor of the arms may be considered evidence for AC.
The LV impulse may be increased. A thrill is usually felt
in the suprasternal notch. The first and second heart
sounds are usually normal in isolated aortic coarctation.
Because of the large percentage (up to 60%) of patients
with AC have associated bicuspid aortic valve, an ejection
systolic click may be heard at the apex and left mid and
right upper sternal borders; this click is constant and does
not change with respiration. An ejection systolic murmur
may be heard best at the left or right upper sternal borders,
but is usually heard best over the back in the left interscap-
ular region. Sometimes, a faint continuous murmur may
be heard in the left interscapular region, secondary to con-
tinuous flow in the coarcted segment or on the back (sec-
ondary to flow in the collateral vessels).
Noninvasive Assessment
Chest radiograph
Chest roentgenogram may show significant cardiomegaly
or the heart size may be normal. Rib-notching secondary
to collateral vessels may also be seen. Other roentgeno-
graphic features include a 3 sign on a highly penetrated
chest radiograph (frontal view) or inverted 3 sign of the
barium-filled esophagus.
Electrocardiogram
The electrocardiogram may be normal or it may show LVH.
Sometimes the LVH may be manifested by increased S
waves in leads V5 and V6, the so-called posterobasal LVH.
Echocardiography-Doppler studies
Echocardiographic imaging usually reveals the coarctation
in suprasternal notch, two-dimensional echocardiographic
views. Increased Doppler flow velocity in the descending
aorta by continuous wave Doppler and a demonstrable
jump in velocity at the coarcted segment by pulsed-Dop-
pler technique are usually present. Instantaneous peak
pressure gradients across the AC can be calculated by
employing a modified Bernoulli equation [6]:
2 2
P = 4 ( V 2 V1 )
where P is peak instantaneous gradient and V2 and V1
are peak flow velocities in the descending aorta distal to
coarctation (continuous-wave Doppler) and proximal to
the coarctation (pulsed Doppler), respectively.
MRI/MR angiography
These studies are useful in demonstrating the anatomy
clearly. If the clinical and noninvasive evaluation outlined
above indicates the need for intervention, I usually go ahead

with catheterization and angiography, and perform interven-
tional procedures. If the data are not clear, then MRI or MR
angiography are performed to define the problem further.
Cardiac catheterization and selective
cineangiography
Although not required for diagnosis, cardiac catheteriza-
tion is helpful in demonstrating the anatomic nature of the
aortic obstruction (discrete vs long segment), assessing the
extent of collateral circulation, determining the presence
and severity of associated lesions (especially in the neo-
nates and infants), and more recently as a prerequisite to
the consideration of transcatheter intervention.
Elevation of LV and ascending aortic peak systolic
pressures with a peak to peak systolic pressure gradient
across the coarcted segment are usually found. A peak to
peak gradient in excess of 20 mm Hg is generally consid-
ered indicative of significant obstruction. However, the
magnitude of the gradient is not necessarily indicative of
the degree of narrowing because the gradient is depen-
dent not only upon the extent of aortic narrowing but
also on the size and number of collateral vessels. In addi-
tion, cardiac output and the state of ductus arteriosus,
particularly in the neonate and young infant, also deter-
mine the pressure gradient.
Selective aortic root or aortic arch angiography is nec-
essary to clearly demonstrate the aortic narrowing. Aor-
tography is useful in demonstrating the type of AC
(diffuse, long segment, or aortic kinking [pseudocoarcta-
tion]), extent of collateral circulation, the size of ductus
arteriosus, if patent, and presence and degree of hypopla-
sia of transverse aortic arch and aortic isthmus. If thoracic
coarctation is not demonstrated despite clinical features
of coarctation or if neurofibromatosis is suspected,
abdominal aortography may be needed to demonstrate
(or exclude) abdominal coarctation.
Treatment
Significant hypertension or congestive heart failure are indi-
cations of intervention. Surgical relief of the aortic obstruc-
tion and catheter interventional techniques (balloon
angioplasty and stents) are available alternatives. Asymp-
tomatic patients should undergo the procedure electively. If
neither hypertension nor heart failure are present, elective
surgical or balloon therapy between the ages of 2 and 5
years is suggested. Waiting beyond 5 years of age is not
advisable because of evidence for residual hypertension if
the aortic obstruction is relieved after 5 years of age [7].
Medical management at initial presentation
In patients with congestive heart failure, initial treatment,
consisting of anticongestive measures including digitalis
preparations and diuretics should be promptly instituted
[3]. If hypertension (rather than heart failure) is the clini-
cal problem, it is better to relieve the aortic obstruction
Coarctation of the Aorta Rao 427
promptly rather than attempting to treat hypertension
with antihypertensive medications, although some clini-
cians use such an approach.
Surgical therapy
Since the introduction of surgical correction by Crafoord
and Nylin, and Gross and Hufnagel in the mid-1940s, sur-
gical therapy has become the treatment of choice for aortic
coarctation. A variety of techniques have been used in
repairing AC and these include resection and end to end
anastomosis, subclavian flap angioplasty, prosthetic patch
aortoplasty, and tubular bypass grafts. Several other modifi-
cations of the initially described techniques have been uti-
lized to improve the results of the operation. The type of
surgical procedure used depends upon the age of the
patient, aortic arch anatomy, and preference of the surgeon.
Although surgical correction has improved the prognosis of
patients with coarctation, there still remain problems asso-
ciated with surgical correction, which include operative
mortality and morbidity, recoarctation, aneurysms in all
types of coarctation repair [8], particularly well-docu-
mented following prosthetic patch angioplasty [9], devel-
opment of paraplegia, paradoxical hypertension, and
vascular complications related to subclavian flap repair.
Despite these problems surgical repair is considered by
many as a therapeutic option of choice in the treatment of
aortic coarctation. Some groups, including ours, consider
balloon angioplasty as initial therapy of choice and reserve
surgical intervention for those coarctations that 1) involve
the long segment of the aorta, 2) are completely or almost
completely occluded such that no catheter or guide wire can
be passed across the coarcted segment, and 3) are associated
with a large patent ductus arteriosus and ventricular septal
defect, which requires prompt surgical intervention for the
primary cardiac problem. There is no consensus with regard
to whether surgical or balloon therapy is the preferred treat-
ment for AC. The issues related to balloon angioplasty and
stenting coarctation segment are addressed below.
Balloon angioplasty
Since the initial descriptions of balloon angioplasty of
neonatal postmortem native aortic coarctation, postsurgi-
cal AC, and native coarctation, a large number of investiga-
tors reported their experiences with balloon angioplasty of
native aortic coarctation. The technique of balloon angio-
plasty is described elsewhere [911].
Immediate results
Despite an initial report of poor results [12], subsequent
experience with balloon angioplasty appears encouraging
and has been detailed elsewhere [9,13,14]. Reduction of
pressure gradient across the coarctation and increase in the
size of the coarctation segment are observed in all age
groups. An example of improvement in angiographic
appearance is shown in Figure 1. The femoral pulses,
which had been either absent or markedly reduced and
428 Hypertension
delayed (when compared with brachial pulse) become pal-
pable with increased pulse volume after balloon angio-
plasty. The patients who were in heart failure improved as
did their hypertension. None of our patients required
immediate surgical intervention.
Intermediate-term follow-up
Several investigators have reported 1- to 2-year follow-up
results, reviewed previously [9,13,14,15]; these studies
suggest continued improvement. From our own study
[15], 60 patients were followed; the residual peak gradi-
ents 14 11 (mean SD) months following angioplasty
remained low at 16 15 mm Hg. These gradients continue
to be lower (P < 0.001) than those prior to angioplasty (46
17 mm Hg) and are slightly higher (P < 0.05) than the
gradients (11 9 mm Hg) immediately following angio-
plasty (Fig. 2). Angiographically measured coarctation seg-
ment remained wide. There is only a modest increase (11
9 vs 16 15 mm Hg; P < 0.05) in peak gradients for the
group as a whole, but when individual patient values are
examined 15 (25%) of the 60 patients had evidence for
recoarctation, defined as a peak to peak systolic pressure
gradient in excess of 20 mm Hg. The incidence of recoarcta-
tion is higher in neonates (five [83%] of six; P < 0.01) and
infants (seven [39%] of 18; P = 0.011) than children (three
[8%] of 36); 10 of these children underwent repeat balloon
angioplasty and two patients underwent surgical resection,
all with good result. Aneurysms developed in three (5%) of
58 who underwent follow-up catheterization and angio-
plasty; one of these patients required surgical excision of
the aneurysm and the other two are followed clinically.
Long-term follow-up
There are scant data on long-term follow-up after balloon
angioplasty of native coarctation [15,1618], reviewed
elsewhere [19]. Despite the problems of recoarctation
and aneurysms, some requiring repeat intervention at
intermediate-term follow-up, the long-term follow-up
results (59 years) appear encouraging, in that there was
minimal incidence of late recoarctation and no late aneu-
rysm formation. Near-normal blood pressure and low
Figure 1. Selected cine frame from a posteroan-
terior view of left ventricular (LV) cineangiogram
(A) showing discrete aortic coarctation (white
arrow) in a 6-year-old child. Following balloon
angioplasty (B), aortogram revealed no evidence
for significant residual coarctation (black arrow).
Aoaorta; DAodescending aorta.
(From Rao [9]; with permission.)
gradient between arms and legs (Fig. 2) were found in the
majority of patients.
Results in adult patients
Although balloon angioplasty of AC has most frequently
been used in infants and children, it has been used in adult
patients as well. Lababidi et al. [20] were the first to apply
this technique in an adult patient (27-year-old man), which
resulted in reduction of peak systolic pressure gradient
across the coarctation from 70 to 15 mm Hg, angiographic
improvement, and reduced hypertension (190/124 mm Hg
vs 130/80 mm Hg). They subsequently reported their
experience with balloon dilatation of native coarctation in
eight consecutive adults, aged 19 to 30 years (25 5 years)
[21]. The systolic pressure gradient across the coarctation
was reduced from 48 19 mm Hg to 7 5 mm Hg. The size
of the coarcted segment increased from 6.8 to 2.2 mm to
15.2 5.0 mm. No complications were encountered. Clini-
cal and echocardiographic-Doppler follow-up 1 year after
the procedure revealed good result with no more than 15
mm Hg peak systolic blood pressure difference, measured by
cuff, between arms and legs. They concluded that results in
young adults are similar to those observed in children; bal-
loon angioplasty should be considered as an option to
surgical intervention; and follow-up studies (longer than 1
year) are required. Other reports followed [2233], which
are tabulated in Table 1. Based on the review of these publi-
cations, the conclusion drawn by Attia and Lababidi [21] are
appropriate but, aortic perforation and dissection during the
procedure and aneurismal formation at follow-up are also
seen in adults. Therefore, it is prudent to 1) avoid manipula-
tion to tips of the catheters and guide wires in the region of
freshly dilated coarctation, 2) chose an appropriate-sized
balloon (no larger than the diameter of the descending aorta
at the level of the diaphragm), and 3) monitor for
development of aneurysms, which, if found, should be
closely followed by repeated angiography or MRI.
Recoarctation
Residual and recurrent obstructions cannot be easily dis-
tinguished from each other and the term recoarctation may
Table 1. Results of balloon angioplasty of aortic coarctation in adult patients
Gradient across coarctation
Age, y (mean SD) mm Hg*
Follow-up: n/mean
Study N Mean SD Range Pre Post FU duration, mo Comments
Attia and Lababidi [21] 8 25 5 1930 48 19 75 < 15 8/12 No complications
Suarez de Lezo et al. [22] 9 22 5 1831 11 9 13 10 / Pre-BA gradient for the group combined
with 19 children was 49 16 mm Hg
Erbel et al. [23] 7 29 14 1449 59 22 13 7 69 7/6 Intimal dissection in one patient at 6-mo
follow-up
Tyagi et al. [24] 35 23 7 1437 81 23 15 13 16 13 26/13 Three aneurysms
Fawzy et al. [25] 23 23 9 1555 66 19 88 89 22/15 One aortic perforation, two developed
restenosis requiring repeat balloon, two
developed a small aneurysm
Kale et al. [26] 8 28 12 1856 71 12 14 9 20 9 4/6 No aneurysms, one restenosis
Phadke et al. [27] 13 30 14 1661 69 30 8 10 9/20 Two aneurysms requiring surgery
Schrader et al. [28] 29 25 1454 62 18 21 13 14 13 29/48 One death and one surgery for aneurysm
deGiovanni et al. [29] 23 33 1558 46 14 10 10 10 7 22/33 Recoarctation in three and aneurysm in one
Koerselman et al. [30] 19 29 1467 49 21 58 19/20 One patient had suboptimal result
Paddon et al. [31] 16 28 1560 51 18 18 16/88 One patient required surgery
Walhout et al. [32] 17 36 1667 50 22 59 /59 Unsuccessful in all three postoperative
recoarctations
Fawzy et al. [33] 49 20 7 66 23 11 7 66 49/122 Repeat balloon in four & aneurysms in four

*Peak to peak systolic pressure gradient measured at cardiac catheterization except for .

Systolic pressure difference between arms and legs.

Data not given.

Includes patients previously reported in 1992.
BAballoon angioplasty; FUfollow-up; Preprior to angioplasty; Postimmediately after angioplasty.
Coarctation of the Aorta Rao
429
430 Hypertension
Figure 2. Bar graph demonstrating immediate and follow-up results
after balloon angioplasty of aortic coarctation. Peak to peak systolic
pressure gradients across the coarctation in mm Hg (mean + SEM) are
shown. Note significant (P < 0.001) drop in the gradient following
angioplasty (Pre, prior to vs post, immediately following). The gradient
increases (P < 0.05) slightly at a mean follow-up to 15 mo (range, 4
56 mo). However, these values are lower (P < 0001) than prior to
angioplasty. At late follow-up (LFU), 6 months to 9 years (median 5 y)
following balloon angioplasty, blood pressure-measured arm-leg peak
pressure difference is lower than catheterization measured peak gradi-
ents prior to (P < 0.001) balloon angioplasty and those obtained at
intermediate-term follow-up (P < 0.01). FUfollow-up; Prepre-
angioplasty; Postpost-angioplasty. (From Rao [3]; with permission.)
be used to describe both these entities and is defined as
peak to peak systolic pressure gradient in excess to 20 mm
Hg with or without angiographically demonstrable nar-
rowing [34]. Recoarctation may take place both following
surgical correction and balloon angioplasty.
Development of recoarctation following surgery is
independent of the type of surgical repair [35]; it has been
observed following resection with end to end anastomosis,
subclavian flap angioplasty, prosthetic patch repair, subcla-
vian artery turn-down procedure, and interposition tube
grafts. The reported incidence of recoarctation has varied,
depending on the study. In the article by Pinzon et al. [8],
reporting on a large number of patients, recoarctation
occurred in 23% of patients studied. The younger the child
at surgery, the higher is the probability for recoarctation.
There is general agreement among cardiologists that bal-
loon angioplasty is the treatment of choice for postsurgical
aortic coarctation. The immediate and follow-up results of
balloon angioplasty for postsurgical recoarctation are
essentially similar to those of native coarctation and have
been reviewed in detail elsewhere [19,35,36].
Restenosis following balloon angioplasty also appears
to be aged-dependent; the younger the child, the greater
is the probability for recoarcta tion [15,34]. We
[15,19,37] and others [16] recommend repeat balloon
dilatations in such cases, whereas still others [38] prefer
surgical intervention.
Intravascular stents
Vascular stenotic lesions can be opened up by balloon
angioplasty, but because of elastic recoil of the vessel wall,
the vessel lumen may return to the predilatation size follow-
ing withdrawal of the balloon catheter. Such recoil and vas-
cular dissection, if any, following balloon dilatation can be
circumvented by implantation of endovascular stents.
Initially, stents were used in the treatment of peripheral arte-
rial disease and coronary artery stenotic lesions in adults
[39]. The technique was then extended to the treatment of
other stenotic vessels including aortic coarctation [40,41].
Despite reasonably good short- and long-term results of
balloon angioplasty, some problems remain, including rest-
enosis, probability of aortic rupture, formation of aneurysms,
and inability to effectively treat long-segment tubular
narrowing. Because of these and other reasons, endovascular
stenting of AC has gained acceptance over the past decade
[4248,49,5057,58]. The perceived advantages of
stents over balloon angioplasty are 1) the ability to expand
tubular long-segment coarctation and hypoplastic isthmus
and distal transverse aortic arch, 2) ability to increase
coarcted segment diameter independent of the intimal tear,
3) ability to decrease the probability of restenosis, 4) preven-
tion of dissection of a torn intimal flap by facilitating apposi-
tion of the intima against the media, and 5) prevention of
aneurysms because of support of a weakened aortic wall with
the stent and neointima.
To my knowledge, OLaughlin et al. [42] were the first
to report use of a stent for treatment of aortic coarctation,
although the results in a 12-year-old child were marginal.
S u b s e q u e n t l y, a n u m b e r o f o t h e r w o r k e r s [ 4 2
48,49,5057,58] reported use of stent in AC with
encouraging results.
The indication for employing stents are 1) long-seg-
ment coarctation, 2) associated hypoplasia of the isthmus
or aortic arch, 3) tortuous coarctation with malalignment
of proximal with distal aortic segment, and 4) recurrent
AC or an aneurysm following prior surgical or balloon
therapy. Because of issues related to growth and the need
for large sheaths for implantation, most cardiologists limit
stent usage to adolescents and adults.
Immediate results
Reduction of peak systolic pressure gradients and increase
in the diameter of the coarcted segment (Table 2) have
been demonstrated following stent implantation. Stenting
was found effective in postsurgical and postballoon reco-
arctations as well as in native coarctations. Improvement in
the size of hypoplastic isthmus or transverse aortic arch
and exclusion of the aneurysm, if such is present, also
occurred after stent placement.
In the first series of 10 patients published by Suarez de
Lezo et al. [44] in 1995, the peak systolic pressure gradient
across the coarctation decreased from 43 12 to 2 3 mm
Hg (P < 0.001). The ratio of isthmus/descending aorta
increased from 0.65 0.14 to 1 0.08 following the proce-
Table 2. Results of stent treatment of aortic coarctation from selected publications
Type of coarctation Mean gradient SD, mm Hg Coarctation segment
Patients
undergoing stent Mean age SD, y Post-
Study implantation, n (range) Native Post balloon Pre Post Pre Post
Suarez de Lezo et al. [44] 10 5 4 (0.143) 6 2 2 43 12 23 * 12 4
Bulbul et al. [46] 6 20 5 (1334) 2 4 0 37 17 13 23 92 16 3
Ebeid et al. [48] 9 30 18 (1463) 2 7 0 37 20 44 93 15 3
Suarez de Lezo et al. [49] 48 14 12 (0.145) 31 6 11 42 12 34 42 12 2
Magee et al. [51] 17 21 14 (445) 6 11 0 26 11 56 72 11 3
Marshall et al. [52] 33 19 14 (560) 6 27 0 25 5 84 13 4
Thanopoulos et al. [50] 17 11 4 (0.415) 8 5 4 50 25 22 52 14 4
Harrison et al. [53] 27 30 13 (1463) 19 7 1 46 20 35 74 17 2
Ledesma et al. [55] 56 22 9 (849) 51 1 4 50 20 58
Hamdan et al. [56] 34 16 8 (436) 13 19 2 32 12 4 11
Tyagi et al. [57] 21 29 11 (1861) 0 0 21 68 22 84 41 14 2
Pedra et al. [58] 21 24 11 () 21 0 0 47 20 11 43 13 2

*No data are available, but the ratio of isthmus/descending aorta changed from 0.65 0.14 to 1 0.08 (P < 0.01).

Includes 10 patients previously reported by the authors in 1995.

No data are available.

No data are available but the ratio of coarctation site to descending aorta increased from 0.46 0.16 to 0.92 0.16 (P < 0.001)
Preprior to stent deployment; Postimmediately after stent deployment.
Coarctation of the Aorta Rao
431
432 Hypertension
dure. Similar results have been reported subsequently by
other investigators which are listed in Table 2.
Complications
Ve s s e l d i s r u p t i o n [ 4 4 ] , d i s p l a c e m e n t o f s t e n t
[44,49,51,56], and aneurysms [49,50] may occur,
but infrequent. Balloon rupture [43,56] resulting in
inadequate stent expansion and stent migration has
been reported but may be prevented by avoiding curva-
ture of the balloon/stent assembly, use of newer stents
with less injurious ends [5961] and by the use of bal-
loon in balloon catheters. Because of large size sheath
required, loss of pulse [44,49,52] and bleeding from
puncture site [46,51] may occur. Use of vascular closure
devices may help circumvent this problem. Rare compli-
cations included myocardial infarctation [52], and
retroperitoneal hemorrhage [56].
Follow-up results
In most studies, there was only a short-term and incom-
plete follow-up in a limited number of patients. However,
a few studies [49,52,55,56] examined results of more
than 20 patients at a mean follow-up of 2 years or longer.
The pressure gradients across the coarctation site (blood
pressure, Doppler, or catheterization) remained low and
systemic hypertension decreased both in degree and fre-
quency with consequent decrease in the need for antihy-
pertensive medications. No evidence for recoarctation,
aneurismal formation, or stent fracture/displacement was
observed, although no systematic or complete follow-up
was achieved in the majority of studies. Residual or recur-
rent obstruction was present in a few patients and in these
successful redilatation with larger balloons was accom-
plished. Detailed angiographic studies by Suarez de Lezo et
al. [49] revealed no detectable neointimal proliferation
in 75% patients; in the remaining patients focal neointi-
mal ridge formation was observed at ends of the stent caus-
ing minimal restenosis. Segmental analysis of the aorta
revealed increase in nonstented segments of the aorta, con-
sistent with normal growth. Two (7%) young patients
developed small new aneurysms, which were obliterated
by coil placement following angiographic detection. The
overall follow-up results were encouraging, however.
Comments
Stent therapy appears to be an attractive method for treat-
ment of recurrent coarctation or aneurysm formation
following prior surgical or balloon intervention and for
long-segment hypoplasia. Most cardiologists use stents in
adolescents and adults, although a few have advocated
their use in younger children. The selection of the type of
stent and the type of balloon catheter used for stent
deployment appears to be evolving [40,62]. Balloon-
expandable instead of self-expandable stents are pre-
ferred. Use of balloon in balloon catheters for stent deliv-
ery appears to be gaining momentum in an attempt to
prevent balloon ruptures and perforation of other cardio-
vascular structures. Positioning the guidewire into the
right or left subclavian artery may avoid excessive curva-
ture, thus may prevent balloon rupture. Flexible [5961]
instead of rigid Palmaz (Cordis, Miami Lakes, FL) stents
are being utilized, and rightly so. Selected stent diameter
at implantation should be at least twice the diameter of
narrowest aortic segment to prevent stent displacement
during implantation.
Relief of obstruction both acutely and at follow-up
with a low incidence of major complications is well dem-
onstrated in several studies. Meticulous attention to the
technique and adoption of new technology may further
reduce the incidence of complications. Recoarctation rate
at follow-up appears to be low. Re-expansion of the stent
[63] to treat residual or recoarctation and growth related
narrowing appears feasible, safe and effective, although
such is based on limited experience. Comparison of
stents with balloon angioplasty was undertaken in a few
studies [58,64,65]; these studies suggest that stents may
be more effective than balloon angioplasty. Based on the
available data, stenting AC appears to be preferred alter-
native to surgical or balloon therapy in the adolescent
and young adult.
Covered stents
There is extremely limited experience in the use of covered
stents in the management of aortic coarctation [6670].
Different types of stents were used and include Jostent
grafts (Jomed International, Helsingborg, Sweden), C-P
stents (NuMed, Hopkinton, NY), and AneuRx (Medtronic,
Minneapolis, MN) to treat aortic coarctation. The indica-
tions for intervention are similar to those used for balloon
angioplasty and deployment of standard stent. The indica-
tions for use of covered stents are postangioplasty aneu-
rysm, tortuous aortic arch, and isthmus, associated patent
ductus arteriosus, prior surgical conduit, Takayasus arteri-
tis and extremely narrow (subatretic) coarcted segment.
When the assessed risk for development of aneurysm or
dissection is high, covered stent should be utilized. The
results of the limited use of covered stent appear to be
good [6670]. Some of the stents can only be expanded to
18 mm in diameter. Also, the stent shortens when
expanded to larger diameters. Use of covered stents has
also another disadvantage in that the vessels arising from
the aorta are blocked. Based on the currently available
data, the covered stents may be useful in highly selected
patients with aortic coarctation.
Conclusions and Prognosis
The immediate prognosis is good for isolated aortic coarc-
tation. The majority of mortality is related to severity of
associated defects. Once aortic obstruction is relieved by
surgical or balloon therapy, the significance of associated
defects should be evaluated and treatment instituted based

on their physiologic and clinical impact on the patient.
Sometimes, the initially mild aortic stenosis may increase
in severity during follow-up requiring balloon or surgical
aortic valvotomy. Subvalvar membranous aortic stenosis
may develop years after coarctation therapy; this can occur
both in isolated AC and AC with ventricular septal defect.
Relief of the subaortic obstruction may be necessary, when
it becomes severe or progressive. As mentioned in the pre-
vious section, recoarctation may develop requiring repeat
intervention. Reappearance of hypertension during adoles-
cence or adulthood has been noted [7,71]. Appropriate
therapy with antihypertensive medication or relief of aortic
obstruction if present, is a warranted in such cases. Because
of these and other problems that might be present, peri-
odic follow-up of patients after relief of aortic obstruction
is highly recommended.
There are scant data on long-term follow-up. The avail-
able studies [7,72] indicated that there is a significant mor-
tality at long-term follow-up of AC. The survival curve,
although not approaching normal population, is signifi-
cantly improved [72] compared with Campbells natural
history data [73]. The causes of death appear to be related to
recoarctation repair, aneurysms at the site of coarctation
repair or at a remote site, congestive heart failure, bacterial
endocarditis, and hypertension. Attempts to define factors
affecting long-term survival have been made, and age at
operation, and degree and duration of hypertension prior to
surgery/intervention appear to affect the long-term survival.
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Of importance
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