1. J. Arab Neonatal Forum 2006; 3:5-13
5
Coarctation of the Aorta: A Comprehensive Review
Mohamed A Hamdan
Department of Paediatrics, Tawam Hospital, Al Ain, United Arab Emirates
__________________________________________________________________________________________
Abstract
Coarctation of the aorta (COA) causes obstruction in the descending aorta (DAO), but has a heterogeneous
spectrum of clinical presentation. There is increasing evidence of genetic influence to explain its occurrence in
family members and association with other left heart obstruction (LHO) lesions. Several treatment modalities
are available including trans-catheter interventional procedures, but long-term morbidity related to hypertension
remains substantial.
Key words: Coarctation of aorta, fetal echocardiography, genetics, paediatrics
COA was first described by Morgagni in 1760 as a
zone of constriction in the DAO.1
COA accounts for
5-7% of congenital heart disease (CHD), with an
incidence of 0.3-0.4/1000 live births.2,3,4
In necropsy
studies, COA is found in 6% of fetuses, 43% of
which have chromosomal anomalies, particularly
monosmoy X.5
Similar to other forms of LHO
lesions, there is a slight male preponderance. Natural
history studies showed that the median age of death
for unrepaired COA was 31 years.6
Death was
attributed to cardiac failure in 26% of patients, while
aortic rupture, infective endocarditis, and inracranial
haemorrhage occurred in 21%, 18%, and 12% of
patients, respectively.6
Aetiology
Coarctation is a narrowing in the DAO, at the
insertion site of the ductus arteriosus (DA), adjacent
to the origin of the left subclavian artery (LSCA),
'juxtaductal' coarctation (Fig. 1). However,
obstruction could also occur in the transverse aortic
arch, or abdominal aorta. It can be discrete or tubular,
and is associated with various cardiac and non-
cardiac abnormalities in up to 50 % of patients.7,8,9
(Table 1).
Although it results from abnormalities in the
development of the embryonic fourth and sixth aortic
arches, COA is quite diverse in severity and
presentation. The underlying mechanisms are not
fully understood, but two concepts have been
proposed: the ductal tissue theory, and reduced-flow
theory.
Ductal tissue theory
Tissue from the DA invades the DAO just distal to
the aortic isthmus. When the DA constricts,
coarctation occurs. This is supported by the fact that
neonatal coarctation manifests only after ductal
closure, ('infantile' type), and usually has more
severe symptoms. The obstruction appears as an
_____________________________
Contact Address: Dr. Mohamed A. Hamdan
Department of Pediatrics, Tawam Hospital, P.O. Box
15258, Al Ain, UAE
Tel.: (+971) 3707 2181
Fax: (+971) 3707 2731
Email: mhamdan@tawam-hosp.gov.ae
Figure 1: Echocardiogram of an 8 day-old neonate with
critical coarctation of the aorta (yellow asterisk) after
commencing prostaglandin E1 infusion. There is large
patent ductus arteriosus (white asterisk) alleviating the
obstruction adjacent to the origin of the left subclavian
artery (LSCA). The ‘posterior shelf’ contributing to the
obstruction is seen (small arrows). AAO: ascending aorta
Table 1: Prevalence of associated lesions
Lesion Prevalence
Bicuspid aortic valve 50%
Ventricular septal defect 30%
Transverse arch hypoplasia 30%
Aortic stenosis 15%
Mitral valve abnormalities 10%
Complex congenital heart
disease
6%
Berry aneurysm in the circle
of Willis
5%
Extracardiac anomalies 28%
Chromosomal anomalies 40%
Adapted from Kiraley et al.7
, Beekman et al.8
, and Paladini
et al.9
indentation (posterior shelf) in the postero-lateral side
of thoracic descending aorta (DAO) opposite to the
insertion site of the DA.8,10
(Fig. 1). This theory
however, fails to explain the occurrence of
coarctation in several other sites.
Reduced-flow theory
Under this concept, defects can develop secondary to
haemodynamic disturbances that reduce flow to the

2. Coarctation of the Aorta
6
Table 2: Presentation of coarctation
Fetus
-ventricular disproportion
-great vessel disproportion
-associated with other congenital heart
disease
-nuchal thickening/
-chromosomal abnormality
(Turner’s syndrome)
Neonate
-collapse, acidosis
-heart failure
-systolic/continuous murmur
conducted to back
-weak or absent femoral pulses
-upper limb hypertension
Infant
-heart failure
-systolic/continuous murmur conducted to
back
-weak or absent femoral pulses
-upper limb hypertension
-cardiomyopathy rarely
Child, adolescent, and adult
-systolic/continuous murmur conducted to
back (collateral murmurs over scapula
rarely)
-weak or absent femoral pulses (radio-
femoral delay in older patients)
-upper limb hypertension
-exercise intolerance, leg fatigue and
claudication, or cold feet
-cardiac arrest (left ventricular
hypertrophy and arrhythmia)
-hypertensive retinopathy
-aortic dissection/ rupture
-intracranial bleed
-infective endocarditis
(Reproduced with permission from: Coarctation of the
aorta from fetus to adult: curable condition or life long
disease process? Rosenthal E. Heart 2005;91:1495-1502,)
affected sites. In the normal fetus, the left ventricle
(LV) ejects 30% of the combined ventricular output,
but the aortic isthmus (proximal DAO between
LSCA and patent DA) receives only 10%, resulting
in a much smaller diameter than the DAO. If the LV
flow is further reduced, further narrowing of the
isthmus occurs, and coarctation develops (Fig. 2).
This explains the common association between the
different types of LHO lesions.8,10
The work by
Fishman and colleagues supports this hypothesis.11
Lamb models of hypoplastic left heart syndrome
(HLHS) and congenital aortic stenosis were created
by altering the pre- and afterload conditions of the
LV.11
Normal preload resulted in normal LV growth,
but banding the ascending aorta resulted in
hypoplastic stenotic aortic valve, and extremely-thick
diminutive LV.11
Recently, Loscalzo et al showed an
association between fetal lymphoedema and COA in
patients with Turner syndrome, where jugular
lymphatic obstruction in fetal life compresses and
reduces flow to the ascending aorta, resulting in
several LHO lesions, including COA, bicuspid aortic
valve, and HLHS.12
Role of Genetics
It is now well-known that cardiovascular
morphogenesis is controlled by numerous genes and
trasnscription factors.13,14
Animal models of CHD
were developed in transgenic and knockout mice. For
LHO lesions, although no specific candidate gene(s)
have yet been identified, there is evidence of ‘left
heart obstruction gene(s)’ to explain the association
between the different types of LHO lesions among
family members. First-degree relatives of patients
with LHO have increased incidence of other LHO
lesions, especially bicuspid aortic valve.15
The
recurrence risk in the offspring of patients with LHO
is 7-13% which is higher than other types of CHD.16
Levy et al found that 14% of the offspring of mothers
with phenylketonuria have CHD, compared to 1% for
the control group.17
LHO lesions accounted for the
majority of the defects including COA (20%), HLHS
(11%), and aortic and mitral valve problems (12%
and 6% respectively). This is thought to result from
specific mutations in the phenylalanine hydroxylase
gene.17
Patients with Turner syndrome provide another
evidence of genetic influence. CHD occurs in up to
71% of patients with Turner syndrome, mostly LHO
including COA (14%), bicuspid aortic valve (12%),
and HLHS (1.2%).18,19,20
Whether this is caused by
haploinsufficiency for X-chromosome gene(s)
involved in cardiovascular development, or
secondary to fetal lymphoedema (webbed neck)
interfering with cardiac and vessel formation is
unclear.12,19
Boucher et al however, provide evidence
that fetal lymphoedema in patients with Turner
syndrome, may indeed be related to a critical region
for lymphoedema gene located at Xp11.4.21
Finally, Andelfinger et al described a family with
dysmorphic features and renal anomalies, associated
with bicuspid aortic valve, and COA.22
Linkage
analysis showed a mutation in the gene KCNJ2
encoding for inward-rectifying potassium channel
Kir2.1.22
Pathophysiology
The haemodyanamic effects of COA vary, and
depend on the severity of the obstruction, associated
cardiac lesions, and compensatory mechanisms. In
the fetus, minimal haemodyanamic disturbance
occurs because only 10% of the combined ventricular
output passes through the isthmus. However, after
birth, ductal closure leads to various disturbances
ranging from mild systemic hypertension, to
congestive heart failure, and shock. Aortic
obstruction impedes LV output, causing significant

3. J. Arab Neonatal Forum 2006; 3:5-13
7
pressure overload, and elevated LV end-diastolic
pressure. In neonates, the pressure load happens
acutely with the onset of ductal closure, resulting in
myocardial dilation, and symptoms of congestive
heart failure. With severe obstruction, myocardial
dysfunction, reduced stroke volume, and cardiogenic
shock develop. Compensatory mechanisms aiming at
augmenting cardiac output are activated including the
Frank-Starling mechanism, the renin-angiotesin, and
the sympathetic systems.8
However, these
mechanisms may not be effective in the immature
neonatal myocardium, because of decreased ß-
adrenergic receptor innervation, and decreased LV
compliance compared to the adult myocardium.8,10
With chronic or gradual obstruction, other
compensatory mechanisms are activated including
LV hypertrophy.
Several vascular abnormalities develop in patients
with COA in the vessels proximal and distal to the
obstruction.23
Neonates and children with COA have
reduced distensibility, and increased reactivity to
norepinephrine in the vessels proximal to the
coarctation site.24,25,26
Plasma renin activity increases
substantially, and baroreceptor reflexes are reset to a
higher blood pressure (BP).27,28
These abnormalities
can persist long after surgical repair, and contribute
to the development of systemic hypertension, and
premature coronary and cerebrovascular death.
Clinical Presentation
The spectrum of clinical manifestations of COA is
variable, and depends on the degree of obstruction,
and associated lesions. Table 2 summarizes the
clinical manifestations in different age groups.3
Neonates and infants
The presence of weak femoral pulses, and upper-to-
lower extremity difference of BP, correctly identifies
neonates with COA.29,30
Crossland et al showed that
an isolated upper-to-lower extremity BP difference of
>20 mmHg has a sensitivity rate of 92%.30
An
important differential diagnosis of shock in the
neonatal period is LHO, including COA.
Unfortunately, in infants below 6 months of age with
COA and cardiac failure, the diagnosis can be
mistaken for sepsis or pulmonary disease in almost
half.29
Children and adolescents
Although older children and adolescents present with
more classical signs of COA (Table 2), Ing et al
showed that only 4% of children over 1 year of age
were correctly diagnosed with COA prior to
cardiology referral, despite having abnormal femoral
pulses, or upper-extremity hypertension in the
majority of them.31
Physical Examination
In infants with cardiogenic shock, murmurs can be
absent, secondary to poor cardiac output, and
minimal flow across the coarctation site. Once
prostaglandin E1 (PGE1) infusion is commenced, a
soft systolic ejection murmur or continuous murmur
radiating to the back can be appreciated (Table 1).
Signs of congestive heart failure and poor perfusion
are present. In older children, a systolic ejection
murmur radiating to the back, or continuous murmur,
represent the most common cause of referral for
cardiac evaluation.31
Diagnosis
Antenatal diagnosis
Fetal echocardiography can correctly identify COA
in 30-71% of cases.9,32,33
Asymmetry of the size of
the great vessels or ventricles, and narrowing of the
aortic arch may provide a clue to diagnosis(Fig. 3).3,32
Antenatal diagnosis improves survival, and
perioperative clinical outcome. In a comparative
study, infants with postnatal diagnosis of COA had
significant increase in the perioperative morbidity
and mortality, secondary to ventricular dysfunction,
and end-organ failure.34
Chest roentgenogram
Neonates presenting in cardiac failure or shock, have
cardiomegaly and pulmonary oedema, which is not
specific for COA. Older children and adolescents can
develop mild cardiomegaly secondary to LV
hypertrophy. A "figure-of- 3" sign on the frontal film
may be seen because of localized indentation at the
coarctation site, associated with prominent arch
proximally and DAO distally. Rib notching is
commonly seen after 5 years of age. It results from
erosions in the inferior edge of the ribs secondary to
tortuous pulsating intercostal arteries.
Electrocardiography
Infants with COA may have normal
electrocardiogram. However, with heart failure and
shock, right or combined ventricular hypertrophy
develops, together with a 'strain' pattern of ST-
segment and T-wave depression indicating
subendocardial or myocardial ischaemia (Fig. 4). In
older children, LV hypertrophy can be seen with
prolonged obstruction.
Echocardiography
The cornerstone of diagnosis remains
echocardiography. Two-dimensional echocardio-
graphy can establish the diagnosis, and delineate the
site of obstruction and associated lesions (Fig. 1).
Flow and colour Doppler measure the peak pressure
gradient across the obstruction (Fig. 5). LV
dimensions and function are assessed by M-mode.
Magnetic Resonance Imaging (MRI)
Recent advances in MRI technology enabled its use
in patients with COA to provide high-quality two-
and three-dimensional images. MRI can define the
exact location and severity of COA, the anatomy of
the aortic arch, and presence of collateral flow (Fig.
6). Measurements using cardiac MRI correlate well
with cardiac catheterization gradient and identify
patients who require transcatheter or surgical
treatment.35

4. Coarctation of the Aorta
8
Figure 2: Diagram of normal fetal circulation. (A) Superior
vena cava (SVC) blood flow (blue arrow) is directed
through the tricuspid valve to the pulmonary artery via the
arterial duct to the lower body segment. Inferior vena cava
(IVC) blood flow (red arrow) containing oxygenated blood
from the placenta is directed across the foramen ovale to
the left ventricle, ascending aorta and upper body segment
with little flow across the isthmus, between the left
subclavian artery and the arterial duct. (B) Coarctation of
the aorta in utero does not affect the fetal blood flow
pattern. (C) After birth there is a fall in pulmonary
resistance and increased pulmonary blood flow (blue
arrows) and forward flow from the aortic arch to the
descending aorta (red arrow). (D) As the ductus constricts,
the narrowing of the coarctation is accentuated and the
increasing obstruction leads to a gradient (red dotted line).
(Reproduced with permission from: Coarctation of the
aorta from fetus to adult: curable condition or life long
disease process? Rosenthal E. Heart 2005;91:1495-1502).
Figure 3: Fetal echocardiogram showing hypoplastic
transverse aortic arch (white asterisk), and coarctation of
the aorta (yellow asterisk). AAO: ascending aorta, DAO:
descending aorta.
Cardiac Catheterization
Non-invasive diagnostic tools (such as
echocardiography, and cardiac MRI) have mostly
replaced cardiac catheterization as a mode of
diagnosis. The main role of cardiac catheterization
Figure 4: An electrocardiogram of a 10 day-old neonate
with critical coarctation of the aorta, who presented in
cardiogenic shock. There is tall R in V1 and deep S in lead
I, indicating right ventricular hypertrophy. The strain
pattern is evidenced by ST-segment depression and T-wave
abnormalities seen in several leads (asterisks). Peaked P-
waves in lead II indicates right atrial enlargement.
Figure 5: Color Doppler echocardiogram of discrete
coarctation of the aorta (COA), showing mosaic color
turbulence across the obstruction. AAO: ascending aorta,
DAO: descending aorta.
now is transcatheter treatment. Occasionally, further
information may be requested in neonates and infants
in the presence of associated lesions or arch
hypoplasia, prior to surgical treatment. Treatment of
coarctation is indicated when the obstruction gradient
during cardiac catheterization is > 20-30 mmHg.36,37
Management
When fetal diagnosis of COA is made or suspected,
delivery should be planned at a centre where cardiac
care services are available. After delivery, once the
diagnosis is confirmed, the neonate is either watched
carefully, or infusion of PGE1 is initiated until repair
is accomplished. In neonates with borderline
obstruction, regular assessment of femoral pulses and
four-limb BP should be done, until it is clear whether
coarctation is present or not.3
Once obstruction is
excluded based on clinical examination, and after the
ductus closes, these neonates can be discharged
home, to be reviewed at regular intervals up to six
months of age, as late presentation of coarctation is
possible.32
Infusion with PGE1 should be initiated
promptly in neonates who develop cardiac failure or
shock. These patents often require mechanical
ventilation, correction of acidosis, and judcious use

5. J. Arab Neonatal Forum 2006; 3:5-13
9
Table 3: Complications of treatment
Surgery
-residual obstruction
-bleeding, haemothorax,
chylothorax
-recurrent laryngeal nerve/ phrenic
nerve palsy
-Horner’s syndrome
-paradoxical hypertension
-paraplegia
-restricted arm growth
-vertebro-basilar steal
-cerebral ischaemia
-aneurysm
-scoliosis
-complications of cardiopulmonary
bypass
Transcatheter treatment
-residual obstruction
-paradoxical hypertension
-femoral artery damage
-bleeding
-aortic dissection/ rupture/
aneurysm
-balloon rupture/ embolus
-stent migration/ malposition
of inotropic agents. PGE1 dilates the ductus and
minimizes the obstruction in 80% of neonates up to
28 days of age, within a mean of 3 hours (Fig. 1).38,39
Lack of response to PGE1 could either be secondary
to complete anatomical closure, or irreversible
functional closure resulting from lack of receptor
sensitivity to PGE1.38
Effective dose of PGE1 varies
between 0.002-0.1 µg/kg/min, and it is unclear
whether ductal dilation is dose-dependant. Early
observations suggest that age >28 days, and weight
<4 kg, are associated with PGE1 failure.38
Lewis at al
reviewed the side effects in 492 infants treated with
PGE1 for various types of CHD.40
Major side effects
occurred in 12-16% of infants and were related to
low birth weight (<2 kg), prolonged use (>48 hours),
arterial infusion, and high dose (> 0.1 µg/kg/min).
The most common side effects observed were:
respiratory depression (11%), cutaneous vasodilation
(7%), rhythm disturbances (7%), seizures (7%), and
hyperthermia (4.5%).40
Once the patient’s haemodynamic condition
stabilizes, definitive treatment is undertaken.
Surgery
Surgical repair of COA is the oldest treatment
modality, described more than 50 years ago.41
Surgical options include resection of the narrow
segment with end-to-end anastomosis, patching with
the LSCA or synthetic patch, or placement of a 'jump'
graft. Among the different techniques, end-to-end
anastomosis is the most-widely used approach,
especially in neonates, and has the best long-term
results.42,43,44
LSCA-flap aortoplasty results in
collateral flow to the left upper extremity, resulting in
restricted arm growth, or blood 'steal' from the left
vertebro-basilar artery (Table 3). When synthetic
materials are used, there is risk of aneurysm
formation at the site of repair.43
Repair is usually
performed via left lateral thoracotomy without
cardiopulmonary bypass. However, median
sternotomy provides better exposure in the presence
of associated cardiac lesions (such as ventricular
septal defect) or with extensive arch reconstruction.
During repair, the proximal transverse arch is
clamped, resulting in controlled ischaemia of the
distal carotid and vertebral arteries. Blood supply to
the left brain is maintained through the contralateral
vessels proximal to the clamped site. Although this
is generally well tolerated, Azakie et al have recently
shown that oxygen supply to the left cerebral
hemisphere is impaired during arch clamping.45
The
long-term significance of this finding is unclear.
Perioperative mortality for repair of isolated COA is
low and ranges from 0-8.5%, but higher mortality
occurs in neonates and infants in the presence of
preoperative cardiac failure or shock.42,43,46-50
The most common complication after surgery is
recurrent coarctation and residual hypertension,
occurring in 3-4% and, 25-38% of patients,
respectively (Table 3).44,50-53
Transcatheter Treatment
Interventional treatment of COA has become an
accepted alternative to surgery. It has an excellent
safety profile, and at least comparable results to
surgery, especially in re-coarctation.
Figure 6: Magnetic resonance imaging of an adult with
discrete COA just distal to the takeoff of left subclavian
artery (black arrow). There are numerous large collaterals
present (white arrows).
AA: ascending aorta, COA: coarctation of the aorta, DA:
descending aorta, ITA: internal thoracic arteries, LA: left
atrium, LV: left ventricle. (Reproduced with permission
from: Aortic Coarctation and Bicuspid Aortic. Bruce CJ,
Breen JF. N Engl J Med 2000;342:249).

6. Coarctation of the Aorta
10
Figure 7: Antero-posterior aortogram in a 7 month-old
infant with (A) discrete coarctation of the aorta (arrow). (B)
balloon angioplasty. (Courtesy of Dr. Michael Slack,
Children’s National Medical Center, Washington, DC,
USA). Balloon angioplasty (BA)
Lock et al performed the first balloon angioplasty in
excised segments of human COA.54
Since then, BA
became a standard method of treatment in both native
and recurrent COA.37,55,56
BA produces a tear in the
thickened intima and media of the narrow aortic
segment, dilating the obstruction (Fig. 7). However,
this could extend into the healthy adjacent aorta
causing rupture, or aneurysms.54,55,57
BA is generally
avoided in the first 6-12 months of life in patients
with native COA, because of high risk of re-
coarctation (57%), aneurysm formation (17%), and
femoral artery damage (39%).58,59
In post-operative
coarctation however, BA can be successful in up to
91% of infants, and is advocated as alternative to
surgery.37
Although it may have higher risk of
aneurysm and femoral artery injury than surgery,
many centres use BA as a primary treatment for COA
beyond infancy, with excellent long-term results.57,60
Stent placement
Balloon-expandable stents have been used
successfully since the early 1990’s, to relieve the
obstruction in animal models, and humans with
COA.36,61,62,63
Stents support the integrity of the
vessel wall during balloon dilation and create a more-
controlled tear. This minimizes tear extension and
subsequent dissection or aneurysm formation.
Aneurysms occur in 4-7% after either BA or stent
placement for COA.55,56,62,63
Despite the initial
reports of stent placement in neonatal and infantile
COA, the long-term results were disappointing.62,63
Stents implanted at a young age are limited by their
small sizes to accommodate somatic growth. They
are therefore reserved for adolescents and adults as a
primary treatment.36
Long-term follow-up of stent
implantation in COA is currently lacking. Table 3
lists complications of surgical and transcatheter
treatment.
Prognosis
Despite excellent results overall for surgical and
transcatheter treatment in patients with COA, long-
term morbidity and mortality remain substantial.
Cohen et al reviewed the Mayo Clinic experience of
571 patients operated between 1946 to 1981.50
At a
median follow-up of 20 years, 11% of patients
required subsequent cardiac surgery (3% for re-
coarctation), 25% developed hypertension, and 15%
had late cardiac-related deaths. Survival analysis
showed that 91% of patients were alive at 10 years,
decreasing to 72% at 30 years after repair. Death
occurred at a mean age of 38 years, and was closely
related to older age of repair (> 9 years of age), and
post-operative hypertension. Forty-four percent of all
late deaths were secondary to coronary artery disease
or cerebrovascular accidents, indicating accelerated
vascular disease.50
Similar results were reported by
Toro-Salazar et al on 274 patients followed for more
than 50 years.49
Risk of death after coarctation repair
is estimated to be 3.8/1000 patient-year.64
An
important risk factor for death is persistent
hypertension, which is associated with older age of
repair, and residual obstruction.50,65
Hypertension
occurs in 7-28% of patients treated in infancy,
compared to 38% treated at 4 years of
age.36,50,51,52,53,57
The incidence of infective
endocarditis after coarctation repair is 1.2/1000
patient-year, so prophylaxis should continue to be
given.66
Conclusion
COA is a lifelong disease with physiological changes
that start in the fetal life, and continue into adulthood.
Early management may reduce long-term
complications, but close follow-up after treatment is
warranted.
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