Diagnosis & Treatment

Physical examination may reveal a pale child with some degree of dyspnea. On examination of the heart rhythm using a stethoscope, infants with coarctation of the aorta usually have an abnormal “gallop” heart rhythm, and 50 percent of children also have heart murmurs . Sometimes excessive arterial pulses can be seen in the arteries of the neck (carotid and suprasternal notch arteries), indicating increased pressure in these arteries, while the major artery of the legs (femoral artery) may have a weak pulse or none can be detected. The systolic pressure is higher in the arms than in the legs. Enlargement of the heart can be seen in x rays and congestion of the blood vessels in the lungs. Similar symptoms may be seen in older children and adults. A 10 mm Hg (mercury) pressure difference between the upper and lower extremities is diagnostic for coarctation of the aorta. For some individuals, the systolic pressure difference is observed only during exercise . Infants frequently have an abnormal electrocardiogram (ECG) that indicates that the right or both ventricles are enlarged, while in older children the ECG may be normal or show that the left ventricle is enlarged. The site and the extent of coarctation may also be detected using color-flow Doppler ultrasonography (echocardiology).

Asymptomatic children may have a normal heart size or only slight enlargement. Differences in blood pressure between the arms and legs may be noted. Hypertension is less likely and, if noted, may be less marked than in symptomatic children with other heart defects. The bicuspid aortic valve is usually present. Color-flow Doppler studies may show a reduced blood flow below the coarctation.

Treatment

The goal of treatment in children is to reopen the ductus arteriosus and restore blood flow to the descending aorta. Congestive heart failure may be treated simultaneously with anticongestive medications. Drug therapy is used first to treat hypertension and heart failure in children and adults who have coarctation of the aorta. Surgery may be required for infants who have severe coarctation of the aorta and is usually recommended for those who have associated cardiac defects or those infants who do not respond to drug therapy. Surgery may also be indicated for infants whose early symptoms do not indicate immediate surgery but who develop severe hypertension during the first several months of life. Older children and adults are advised to avoid vigorous exercise prior to surgical correction of the coarctation. Surgery may involve resection of the coarctation segment or opening and patching the aorta where the coarctation occurred. Balloon angioplasty is sometimes performed on infants who are not ideal candidates for repair surgery because of higher risk; this procedure involves passing a catheter with an attached deflated balloon through the femoral artery in the groin and inflating the balloon to open the coarctation segment of the aorta. Recoarctation can occur in some individuals, even after corrective surgery. Recurrence is higher with balloon angioplasty than with repair surgery.