Older children presenting with coarctation of the aorta are usually asymptomatic. There may be a history of leg discomfort with exercise, headache, or epistaxis. Decreasedor absent lower extremity pulses, hypertension (upperextremity), or a murmur may be present. The murmur istypically best heard in the left interscapular area of the back.If significant collaterals have developed, continuous murmurs may be heard throughout the chest. An abnormal aortic valve is present approximately 50% of the time, causing asystolic ejection click and systolic ejection murmur of aorticstenosis.
The ECG and chest x-ray show evidence of right ventricular enlargement and hypertrophy in infantile coarctation with marked cardiomegaly and pulmonary edema. Echocardiography shows the site of coarctation and associated lesions. In older children, the ECG and chest x-ray usually show left ventricular hypertrophy and a mildly enlarged heart. Rib notching may also be seen in older children (>8 years of age) with large collaterals. Echocardiography shows the site and degree of coarctation, presence of left ventricular hypertrophy, and aortic valve morphology and function.
Management of an infant presenting with cardiac decompensation includes intravenous infusion of prostaglandin E1 (chemically opens the ductus arteriosus), inotropic agents, diuretics, and other supportive care. Balloon angioplastyhas been done, especially in critically ill infants, but surgical repair of the coarctation is most commonly performed. Ballooning and stenting of older patients with coarctation has become more accepted as primary therapy, but surgical repair remains a common form of management.
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Cyanotic congenital heart disease occurs when some of the systemic venous return crosses from the right side of the heart to the left and returns to the body without going through the lungs (righttoleft shunt). Cyanosis, the visible sign of this shunt, occurs when approximately 5 g/100 mL of reduced hemoglobin is present in systemic blood. Thus, a polycythemic patient appears cyanotic with a lower percentage of reduced hemoglobin. A patient with anemia requires a higher percentage of reduced hemoglobin for the recognition of cyanosis.
The most common cyanotic congenital heart defects are the five Ts: Tetralogy of FallotTransposition of the great arteriesTricuspid atresiaTruncus arteriosus Total anomalous pulmonary venous return
Other congenital heart defects that allow complete mixing of systemic and pulmonary venous return can present with cyanosis depending on the amount of pulmonary blood flow that is present. Many cyanotic heart lesions present in the neonatal period (Table 144-1).
Tetralogy of Fallot is the most common cyanotic congenital heart defect, representing about 10% of all congenital heart defects (Fig. 144-1). There are four structural defects: ventricular septal defect (VSD), pulmonary stenosis, overriding aorta, and right ventricular hypertrophy. Tetralogy of Fallot is due to abnormal septation of the truncus arteriosus into the aorta and pulmonary artery that occurs early in gestation (3to 4 weeks). The VSD is large and the pulmonary stenosis is most commonly subvalvular or infundibular. It may also be valvular, supravalvular, or, frequently, a combination of levels of obstruction.
Infants initially may be acyanotic. A pulmonary stenosis murmur is the usual initial abnormal finding. The amount of right-to-left shunting at the VSD (and the degree of cyanosis) increases as the degree of pulmonary stenosis increases. With increasing severity of pulmonary stenosis, the murmur becomes shorter and softer. In addition to varying degrees of cyanosis and a murmur, a single S2 and right ventricular impulse at the left sternal border are typical findings.