• Decreased systemic blood flow to lower extremities after ductal closure
• Increased resistance to left ventricular outflow causes systolic hypertension and LV hypertrophy.
• If the coarctation of the aorta (CoA) is severe, left ventricular dysfunction and congestive heart failure result, with low cardiac output and increased left ventricular end-diastolic pressure.
• Decreased myocardial perfusion may be present in cases of very low output.

• Multifactorial; occurs in 35% of patients with Turner syndrome (XO)
• Has been described in cases of monozygotic twins

• Systemic hypertension
• Shock
• Congestive heart failure
• Cerebrovascular accident

• Residual or recurrent coarctation
• Hypertension (rest and exercise)
• Aortic aneurysm
• Associated intracardiac lesions

• Patent ductus arteriosus (PDA)
• Ventricular septal defect
• Valvar or subvalvar aortic stenosis
• Bicuspid aortic valve occurs in 85% of patients with CoA.
• Mitral stenosis: often associated with structural mitral valve abnormalities (i.e., supravalvar mitral ring, thickening of mitral leaflet, single papillary muscle with parachute deformity, or short dysplastic chordae tendinae)
• Shone syndrome: multiple left-sided obstructive lesions, including mitral stenosis, subaortic membrane, aortic valve stenosis, and coarctation
• Berry aneurysm of the circle of Willis
• Renal artery stenosis associated with abdominal coarctation

• Other left-heart obstructive lesions
• Hypoplastic left-heart syndrome
• Critical aortic stenosis

• An infant with congestive heart failure or shock—“A small, pale, irritable child in respiratory distress.” It is caused by closure of PDA and is more common in infants with complex CoA (20%–30%).
  • Poor feeding
  • Dyspnea
  • Diaphoresis
  • Poor weight gain
  • Oliguria
• An otherwise asymptomatic child with systolic hypertension and/or a heart murmur (70%–80%)
  • Lower extremity claudication
  • Headaches

• Tachypnea and tachycardia
• Discrepant arterial pulses and systolic blood pressure in the upper and lower extremities
• Weak “thready” pulses
• Grades 2 to 3/6 systolic ejection murmur
• Gallop rhythm in an infant with congestive heart failure
• Ejection click from bicuspid aortic valve
  • The most important finding: decreased or absent lower extremity pulses. (Always put your fingers on femoral pulses.) Are pulses present? Is there a delay between the brachial and femoral pulses?
  • Heart murmur: best heard at the upper LSB, at the base and radiating to the left interscapular area posteriorly
  • An infant with critical CoA with PDA: “differential cyanosis” The lower part of the body appears cyanotic because the descending aortic flow is provided by the right ventricle through PDA (check postductal saturation).

• ECG: Right ventricular hypertrophy is usually present in symptomatic infants. ECG is often normal in children. Left ventricular hypertrophy is apparent with more severe coarctation or coarctation of longer standing.
• Chest x-ray: in the infant, moderate-to-severe cardiomegaly with increased pulmonary vascular markings (PVM). In an asymptomatic child, the heart size is often normal with normal PVM. Rib notching may be seen in older children secondary to erosion of the ribs by dilated intercostal collateral vessels.
• Echocardiography: localization and degree of CoA and associated findings; PDA, isthmus hypoplasia. Assessment of associated left-sided obstruction (severity of which may be underestimated in patients with congestive heart failure, low cardiac output, or the presence of a PDA): mitral valve abnormality, left ventricular outflow obstruction, and aortic stenosis (bicuspid aortic valve)
• MRI: clearly defines the location and severity of CoA. May be useful for serial follow-up postoperatively (especially aortic aneurysms)
• Cardiac catheterization and angiography: usually not indicated unless there are further questions to be answered and/or a planned intervention.

• Prostaglandin infusion: 0.05 µg/kg/min
• Inotropes: digoxin
• Diuretics for pulmonary venous hypertension or pulmonary edema: furosemide
• Correction of metabolic disturbances caused by systemic hypoperfusion
• Surgical intervention should follow as soon as possible.

• Surgical repair of CoA and associated intracardiac anomalies
• The surgical mortality rate for infants with CoA and a large VSD ranges from 5% to 15% and is higher for children with more complex intracardiac anomalies.

• Elective CoA repair between ages 3 and 5 years in asymptomatic children without severe upper extremity hypertension. Later repair is associated with increased risk of sustained hypertension and atherosclerosis.

• End-to-end anastomosis
• Subclavian flap aortoplasty
• Prosthetic patch aortoplasty
• Bypass graft

• Percutaneous balloon angioplasty of native CoA is still controversial because of concern about recurrent stenosis and aneurysm formation.

• Bleeding
• Postcoarctectomy syndrome/mesenteric arteritis
• Paradoxical hypertension
• Spinal cord ischemia (0.4%)
• Residual coarctation
• Chylothorax
• Stridor
• Diaphragm paralysis
• Subclavian steal
• Aortic aneurysm or dissection

• Reexamine every 6 to 12 months.
• Residual or recurrent CoA (25%–60%); occurs most commonly after repair in infancy: Percutaneous balloon angioplasty can be performed as early as 2 months postoperatively.
• Residual systemic hypertension; most commonly in patients whose CoA repair is delayed beyond late childhood
• Aneurysm formation
• Cerebrovascular accidents
• Monitor four-extremity blood pressure for recoarctation or residual hypertension.
• Antibiotic prophylaxis to prevent endocarditis or endarteritis
• May have hypertension with exercise, even if normotensive at rest
• Exercise-induced hypertension without anatomic stenosis may respond to beta-blocker therapy.

• The most reliable clinical findings to diagnose CoA are the presence of pressure differences in upper and lower extremities and decreased or absent femoral pulses. Palpable pulses do not exclude coarctation. What one palpates is pulse pressure, not absolute systolic pressure.
• Four-extremity blood pressure measurement is very important in assessing infants and children with possible congenital heart disease. Proper cuff size must be used.

Q: When is the most appropriate time to perform surgical repair of simple CoA?
A: Currently, it is recommended that elective CoA repair be performed between 3 and 5 years of age in asymptomatic children without severe upper extremity hypertension, based on the risk of recoarctation (higher incidence under 3 years, especially under 1 year, of age) and residual hypertension and subsequent atherosclerotic cardiovascular disease.

Q: What is the incidence of residual hypertension after surgical repair of CoA?
A: The incidence of late residual hypertension may be reduced to approximately 6% if CoA repair is performed under 5 years of age, as opposed to 30% to 50% in patients whose CoA is repaired at an older age.

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Chang AC, Hanley FL, Wernovsky G, Wessel DL. Pediatric cardiac intensive care. Baltimore: Williams & Wilkins, 1998:247–254.

Fyler DC. Nadas’ pediatric cardiology. Philadelphia: Hanley and Belfur, Inc., 1992:535–556.

Liberthson RR. Coarctation of the aorta: review of 234 patients and clarification of management problems. Am J Cardiol 1979;43:835–840.

Morriss MJH, McNamara DG. Coarctation of the aorta and interrupted aortic arch. In: Gasson A, Jr., Bricker JT, Fisher DJ, Neish SR, eds. The science and practice of pediatric cardiology. Philadelphia: Lea &Febiger, 1990: 1353–1381.