Some infants arrive at the emergency department with respiratory distress and are found on examination to have findings suggestive of congenital heart disease, usually with evidence of congestive heart failure. These infants have near normal blood pressure and usually display normal skin perfusion, but may have cyanosis. Several congenital cardiac conditions may present in this fashion.
TRUNCUS ARTERIOSUS This congenital anomaly is characterized by a single, large arterial trunk originating from the ventricular portion of the heart. This common vascular trunk supplies blood to both the systemic and the pulmonary circulation. A large VSD is usually present and may account for the murmur that is often heard. Because of the large amount of flow from both ventricles into the single large arterial conduit, flow to the pulmonary tree is greatly enhanced due to decreased flow resistance. This results in little or no cyanosis until pulmonary resistance increases and then cyanosis appears.
Clinically, these infants present with signs of increased pulmonary blood flow, dyspnea, and, occasionally, frank congestive heart failure. Chest x-ray demonstrates cardiomegaly and increased pulmonary vascularity or pulmonary edema. The ECG is initially normal until pulmonary vascular resistance increases and then signs of strain, left ventricular hypertrophy, or biventricular hypertrophy are evident.
VENTRICULAR SEPTAL DEFECT This is the most common cardiac defect, and symptoms displayed depend on the size of the defect. Small defects are often found on routine physical examination. More than 60 percent of these close spontaneously in older childhood.
Moderate-sized VSDs cause elevated right ventricular pressure and subsequent increased pulmonary artery pressure. Infants with this defect present with increased cough with mild upper respiratory tract infections and may have mild increase in pulmonary vascularity and early congestive heart failure. Typically, the chest radiograph is interpreted as mild congestive heart failure, and treatment consists of furosemide and, occasionally, digoxin.
Large VSDs present with congestive heart failure early in infancy, resulting in early and severe pulmonary artery pressure that, if uncorrected, will result in pulmonary hypertension. Such pulmonary hypertension will result in reversal of left-to-right shunt and frank cyanosis in a condition known as the Eisenmenger complex. Originally felt to be surgically irreversible, some centers now are routinely repairing such defects in adolescents who had inadequate management in infancy. 10
COARCTATION OF THE AORTA This represents localized narrowing of the aortic lumen, most often distal to the origin of the left subclavian artery and in close proximity to the ductus arteriosus or its postnatal remnant, the ligamentum arteriosus.
In infancy, symptomatic infants present with congestive heart failure and feeding difficulty. Decreased pulse amplitude and duration are noted in the lower extremities, and hypertension is noted in the upper extremities. Cardiac examination reveals a systolic ejection murmur at the cardiac base, with interscapular radiation.
Older children present with decreased exercise tolerance and, occasionally, claudication to the lower extremities. In children older than 6 to 7 years of age, a characteristic rib notching of the inferior border of posterior ribs is evident. Such notching is bilateral and usually caused by hypertrophied collateral vessels. 11
Was this article helpful?
Do You Suffer From High Blood Pressure? Do You Feel Like This Silent Killer Might Be Stalking You? Have you been diagnosed or pre-hypertension and hypertension? Then JOIN THE CROWD Nearly 1 in 3 adults in the United States suffer from High Blood Pressure and only 1 in 3 adults are actually aware that they have it.