Pathophysiology

Coarctation of the aorta imposes significant afterload on the left ventricle (LV), which results in
increased wall stress and compensatory ventricular hypertrophy.

The afterload may be imposed acutely, as occurs following closure of the ductus arteriosus in
neonates with severe coarctation. These infants may rapidly develop CHF and shock. Rapid
constriction of the ductus arteriosus, producing sudden severe aortic obstruction, seems to be the
most likely explanation. As the ductus (aortic end) constricts, the left ventricular afterload
rapidly increases, with a resultant increase in left ventricular pressures (systolic and diastolic).
This causes elevation of the left atrial pressure, which may open the foramen ovale, causing left-
to-right shunt and dilatation of the right atrium and right ventricle. If the foramen ovale does not
open, pulmonary venous pressures and pulmonary artery pressures increase, and right ventricular
dilatation develops.

Cardiomegaly revealed by chest roentgenography and right ventricular hypertrophy seen on ECG
and echocardiography are related to the indirect effects of rapid development of severe aortic
obstruction.

LV afterload may also gradually increase, allowing children with less severe coarctation to
develop arterial collateral vessels that partially bypass the aortic obstruction. These children may
be asymptomatic until hypertension is detected or another complication develops.

The mechanism for development of hypertension is not clearly understood; mechanical

obstruction and renin-angiotensin–mediated humoral mechanisms have been postulated.

The 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
proximal to coarctation. However, this theory does not explain the following:

 The lack of relationship between the degree of elevation of blood pressure and the
magnitude of obstruction
 The increased peripheral vascular resistance distal to the site of obstruction
 The delayed or lack of reduction of blood pressure immediately following relief of
obstruction

The humoral theory postulates activation of the renin-angiotensin system secondary to reduction
of renal blood flow and appears to explain most of the clinical features.[6, 7, 8] However,
measurement of plasma renin activity in both animal models and human subjects did not show
consistently elevated plasma renin levels in the early studies. The reasons for the inability to
demonstrate elevation of renin levels may be related to inadequate measurement of salt intake,
posture, extracellular fluid volume, and sympathetic influences on renin release. More recent
studies demonstrated abnormalities in renin-angiotensin-aldosterone systems.[9] In addition,
activation of central sympathetic nervous system may also be responsible for hypertension of
aortic coarctation.[10]
Associated anomalies greatly influence pathophysiology.[11] VSD is also frequently present, and
coarctation exacerbates the associated left-to-right shunt. Other levels of left heart obstruction
(aortic stenosis, subaortic stenosis) may be present and may add to LV afterload.

Numerous neurohumoral changes occur with CHF.[12] Sympathetic nervous system activation
occurs, resulting in increases in heart rate and blood pressure (BP). The renin-angiotensin system
is activated in patients with CHF, particularly in coarctation of the aorta, in which lower-body
BP and renal perfusion may be reduced. Activation of the renin-angiotensin system results in
vasoconstriction, cell hypertrophy, and the release of aldosterone. The role of the renin-
angiotensin system in CHF and the use of drugs to modulate this system are an intense area of
research. Unlike most cases of CHF, coarctation of the aorta is more complex because
precoarctation and postcoarctation hemodynamics are quite different.

Drugs typically used to treat patients with CHF, such as ACE inhibitors and, more recently,
angiotensin II antagonists, may have adverse effects in patients with coarctation of the aorta.
Attempts to achieve a normal precoarctation BP with these drugs may result in inadequate lower-
body perfusion and may precipitate renal failure.

Vasopressin is also increased in heart failure, although its major stimulus for release is
angiotensin II. Vasopressin affects free water retention and may result in hyponatremia. The
vasoconstrictive properties of vasopressin may further elevate BP in coarctation.

Other substances, such as human brain natriuretic peptide (BNP), an endothelin, may be
activated by CHF, although their specific role in coarctation has not been studied.

An additional cause of coarctation of the aorta is trauma that results in aortic dissection.
Compromise of the true lumen of the aorta can result in the clinical picture of coarctation with
reduced lower-extremity pulses. Urgent intervention is required in this circumstance.

Epidemiology
Frequency

United States

Coarctation of the aorta is a common defect and occurs in 6-8% of patients with congenital heart
disease.[13, 14] However, coarctation may be found more frequently in infants who present with
symptoms prior to age one year.[11]

International

The prevalence of coarctation of the aorta appears to be lower (< 2%) in Asian countries than in
European and North American countries.[15]

coarctation of the aorta is a localized narrowing of the aortic lumen that results in upper-
extremity hypertension, left ventricular hypertrophy, and malperfusion of the abdominal organs
and lower extremities. Symptoms vary with the anomaly's severity and range from headache,
chest pain, cold extremities, fatigue, and leg claudication to fulminant heart failure and shock. A
soft bruit may be heard over the coarctation site. Diagnosis is by echocardiography or by CT or
MR angiography. Treatment is balloon angioplasty with stent placement, or surgical correction.

Coarctation of the aorta accounts for 6 to 8% of congenital heart anomalies. It occurs in 10 to

20% of patients with Turner's syndrome. The male:female ratio is 2:1.

Pathophysiology

Coarctation of the aorta usually occurs at the proximal thoracic aorta just beyond the left
subclavian artery. It rarely involves the abdominal aorta. Coarctation may occur alone or with
various other congenital anomalies (eg, bicuspid aortic valve, ventricular septal defect, aortic
stenosis, patent ductus arteriosus, mitral valve disorders, intracerebral aneurysms).

Physiologic consequences include left ventricular pressure overload, left ventricular hypertrophy,
hypertension in the upper part of the body including the brain, and malperfusion of the
abdominal organs and lower extremities. Malperfusion of the intestines increases the risk of
sepsis due to enteric organisms.

Untreated coarctation may result in left ventricular failure, rupture of the aorta, intracranial
hemorrhage, hypertensive encephalopathy, and hypertensive cardiovascular disease during
adulthood.

Symptoms and Signs

If coarctation is significant, circulatory shock with renal insufficiency (oliguria or anuria) and
metabolic acidosis may develop in the first 7 to 10 days of life and may mimic findings of other
systemic disorders such as sepsis.

Less severe coarctation may be asymptomatic during infancy. Subtle symptoms (eg, headache;
chest pain, fatigue, and leg claudication during physical activities) may be present as children
age. Hypertension is often present, but heart failure (HF) rarely develops after the neonatal
period. Rarely, intracerebral aneurysms rupture, resulting in subarachnoid or intracerebral
hemorrhage.

Typical physical examination findings include hypertension in the upper extremities, diminished
or delayed femoral pulses, and low or unobtainable arterial BP in the lower extremities. A grade
2 to 3/6 ejection systolic murmur is often present at the upper left sternal border, left axilla, and
sometimes most prominently in the left interscapular area. An apical ejection click is present if a
bicuspid aortic valve is also present. Dilated intercostal collateral arteries may cause a
continuous murmur in the intercostal spaces. Affected females may have Turner's syndrome, a
congenital disorder causing lymphedema of the feet, webbed neck, squarely shaped chest,
cubitus valgus, and widely spaced nipples.

Diagnosis
  Chest x-ray and ECG
 Echocardiography or CT or MR angiography

Diagnosis is suggested by clinical examination (including BP measurement in all 4 extremities),

supported by chest x-ray and ECG, and established by 2-dimensional echocardiography with
color flow and Doppler studies or with CT or MR angiography.

Chest x-ray shows coarctation as a “3” sign in the upper left mediastinal shadow. Heart size is
normal unless HF supervenes. Dilated intercostal collateral arteries may erode the 3rd to 8th ribs,
causing rib notching, but this is seldom seen before age 5 yr.

ECG usually shows left ventricular hypertrophy but may be normal. In neonates and small
infants, ECG usually shows right ventricular hypertrophy rather than left ventricular
hypertrophy.

Treatment

 For symptomatic neonates, prostaglandin E1 infusion

 For hypertension, β-blockers
 Surgical correction or balloon angioplasty (sometimes with stent placement)

Symptomatic neonates require cardiopulmonary stabilization with infusion of prostaglandin E1

(0.05 to 0.10 μg/kg/min—may titrate up to 0.4 μg/kg/min then back down to lowest effective
dose) to reopen the constricted ductus arteriosus. Opening the ductus and its aortic ampulla
provides some relief of the aortic obstruction and allows pulmonary artery blood to increase
perfusion of the descending aorta through the ductus, improving systemic perfusion and
reversing metabolic acidosis. IV cardioactive drugs (eg, milrinone Some Trade Names
PRIMACOR
Click for Drug Monograph
, dopamine Some Trade Names
INTROPIN
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, dobutamine Some Trade Names
DOBUTREX
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), diuretics, and O2 are used to treat HF.

In nonemergent situations, patients with hypertension may be treated with β-blockers; ACE
inhibitors may adversely affect renal function. After repair of the coarctation, hypertension may
persist or develop years after repair and can be treated with β-blockers, ACE inhibitors,
angiotensin II receptor blockers, or Ca channel blockers.

The preferred definitive treatment is controversial. Some centers prefer balloon angioplasty with
or without stent placement, but most prefer surgical correction and reserve the balloon procedure
for recoarctation after surgical correction or for primary treatment of discrete coarctation in older
children or adolescents. Initial success rate after balloon angioplasty is 80 to 90%; subsequent
catheterization can dilate the stent as children grow.

Surgical options include resection and end-to-end anastomosis, patch aortoplasty, and left
subclavian flap aortoplasty. In severe coarctation manifesting early in life, the transverse aorta
and isthmus are often hypoplastic, and this region of the aorta may need to be surgically
enlarged. Choice of surgical technique depends on anatomy and center preference. Surgical
mortality rate is < 5% for symptomatic infants and < 1% for older children. Residual coarctation
is common (6 to 33%). Rarely, paraplegia results from cross-clamping of the aorta during
surgery.

Endocarditis prophylaxis is not needed preoperatively and is required only for the first 6 mo after
repair or if there is a residual defect adjacent to a surgical patch.

Mortality/Morbidity

Past autopsy studies suggest that the mortality rate in patients in whom coarctation of the aorta is
not surgically repaired is 90% by age 50 years, with a mean age of 35 years.[16] In the current era,
coarctation of the aorta mortality is often determined by patient age, patient size, and associated
major cardiovascular anomalies.

Associated problems that may contribute to death or morbidity include hypertension, intracranial
hemorrhage, aortic rupture or dissection, endocarditis, and CHF.

Race

No definitive racial differences have been documented in coarctation of the aorta, although some
authors have suggested that coarctation of the aorta is less common in Asians.[15]

Sex

The male-to-female ratio is 2:1, although this ratio is not valid in abdominal coarctation of the
aorta, in which this rare lesion predominantly affects females. The ratio of abdominal-to-thoracic
coarctation is approximately 1:1000. The male preponderance observed in older patients is not
seen in infants with coarctation of the aorta.

Age

Generally, patients with coarctation of the aorta present early in life with CHF or later in life
with hypertension. Studies continue to document that coarctation of the aorta is often missed in
the first year of life,[17, 18] and the median age of referral to a pediatric cardiologist in one study
was 5 years. Among 2192 patients reported to the Pediatric Cardiac Care Consortium from 1985-
1993, 1337 were infants, 824 were children, and 31 were adults.[19]
Coarctation of the Aorta

Definition
Coarctation of the aorta is caused by the discrete narrowing of the distal segment of the aorta beyond
the left subclavian artery arising from the aortic arch. This includes the deformity of the media of the
aorta and is represented by a curtain-like infolding of the outer wall of the aorta which causes a
narrowing. The coarctation is due to an inbalance in fetal circulation within the aorta and pulmonary
arteries that interferes with normal hemodynamic molding of these arteries.
Coarctation of the aorta occurs in approximately 8% of congenital heart defects.

Anatomy
The coarctation occurs after the left subclavian which arises from the aortic arch. It begins as an
indentation of the posterior wall. When the ductus arteriosus is patent, the aorta is not significantly
obstructed; however, if the ductus arteriosus begins to close after birth the aorta is no longer widened
by it and this results in aortic obstruction.
The coarctation may be preductal, where the narrowing is proximal to the ductus arteriosus or
ligamentum arteriosum.
The coarctation may be postductal, where the narrowing is distal to the ductus arteriosus or
ligamentum arteriosum.
Collateral circulation connects the proximal and the distal aspects of the vessels over time. This
collateral circulation will develop mainly from the subclavian, scapular, internal thoracic, and
intercostal arteries. Collateral circulation is divided into anterior and posterior systems.
In the anterior system, the internal mammary arteries and the epigastric arteries join to form
collaterals which supply the abdominal wall and the lower extremities.
In the posterior system, the parascapular arteries connect with the intercostal arteries to form
collaterals which supply the distal aortic compartment and primarily the abdominal viscera.
The left subclavian may form collaterals through linkage to the IMA's and intercostals to give blood
supply distally. The right subclavian will join with the vertebral, spinal, cervical, and scapular
branches, and will eventually provide blood supply to the intercostal circuit.
The coarctation of the aorta may also involve the whole contour of the aorta which is known as
hypoplasia segmentaria.

Pathophysiology
The coarctation is due to intracardiac abnormalities where the increase in pulmonary artery and
ductal flow and the decrease in aortic flow are present. The area of the aorta which is affected
remains under development and narrows. This usually develops into the preductal type.
When there is a defect which is only significant in the fetal state (i.e. narrow foramen ovale), and
ductal flow exceeds the flow across the aortic valve, the flow to the ductus arteriosus will begin to
supply a portion of the distal brachiocephalic circulation. This biforcated circulation causes an
indentation in the aorta beyond the ductal opening. The ductus will continue to close and cause
infolds in the lumen, producing a smaller opening. This usually results in the postductal type.
The principle cardiac anatomy is left ventricular hypertrophy due to increased afterload, since tension
is increased when the heart tries to pump against it causing the ventricle to become hypertrophied. It
is usually accompinied with venous congestion. Dilatations of the aorta above and below the
coarctation are common. Aneurysms of the aortic arch, the Circle of Willis, and the descending aorta
are common. The rupture of these aneurysms is the primary cause of death in adult patients with
coarctation of the aorta.

Shunts

Left to right shunt: occurs when a postductal coarctation and a PDA are found together.
Manifestations include right diastolic overload, varying degrees of pulmonary hypertension, right
ventricular hypertrophy, and eventually Eisenmeiger Syndrome. In this case, there is good formation
of collateral circulation at birth. This case is also more benign than preductal coarctation in association
with a PDA.
Right to left shunt: allows for a continuous blood flow from the pulmonary artery to the aorta. Occurs
when a preductal coarctation and a PDA are found together. Manifestations include right ventricular
hypertrophy, pulmonary hypertension, aortic and left ventricular hypoplasia. Most of these patients
die within a few months of birth due to poor collateral circulation at birth causing heart failure,
pulmonary hypertension, and the effects associated when deoxygenated blood reaches descending
aorta.
When a postductal COA is found with a closed ductus arteriosus the extent of the problems depends
upon the severity of the stenosis and the development of the collateral circulation.
When a preductal COA is found with a closed PDA there is usually good collateral circulation present.
The closing of the PDA causes changes in pulmonary arterial blood flow that increase diastolic
overload and cause LV failure from the sudden overload of blood in the left ventricle. This leads to
88% mortality rate.

Associated Abnormalities

1) Bicuspid Aortic Valve (85%)

2) Mitral Valve Malformation (30%)
3) PDA (22%)
4) Congestive Heart Failure (10%)
5) Bacterial Endocarditis (may result in cerebral vascular accident due to increased turbulence of
blood)

Hemodynamics
Arterial Hypertension: 150/100 due to systolic overload.
Pulmonary Hypertension: large PDA, left to right shunt.
Distal Arterial Hypotension: deficit of blood flow distally to COA.
Local Blood Turbulence: bacterial endocarditis.
Aortic and LV Hypoplasia: pulmonary hypertension, RV hypertrophy.

Clinical Manifestations
LV and Systolic Overload: dyspnea, CHF, left to right shunt.
Abnormal Hemodynamics: aortic aneurysms.
Large PDA and Pulmonary Hypertension: dyspnea, RV failure.

Prognosis and Medical Intervention

Medical management is important for the treatment or prevention of bacterial endocarditis. It is also
important to treat newborns who present with severe heart failure with intravenous prostaglandins
to reopen or keep ductus arteriosus in a patent state. However, in case of closed PDA, surgical
intervention should be given an immediate consideration. Optional surgery may be postponed until
15 years of age when the aorta is fully developed.
Surgical treatment results in end to end anastomosis of the aorta. If this is not possible due to the
presentation of tubular hypoplasia, a dacron tube prosthesis may be used. If the aorta has
degenerative lesions and resection is hazardous due to a tortuous aorta, the prosthesis should be
implanted around the area of the coarctation. In cases of localized coarctation, the area of the
infolded