January 1996

Pathological Case of the Month

Arch Pediatr Adolesc Med. 1996;150(1):101-102. doi:10.1001/archpedi.1996.02170260105019

A 1450-G WHITE MALE infant was delivered by cesarean section after 30 weeks' gestation. Fetal ultrasound showed polyhydramnios and possible complex congenital heart disease.

At delivery, the infant required intubation and significant peak inflating pressures with 100% oxygen to achieve good color. Severe respiratory distress, hepatomegaly, and decreased perfusion developed. An echocardiogram revealed a dilated right ventricle, patent ductus arteriosus, and decreased cardiac function.

Surfactant instillation, high-frequency ventilation, and tube thoracostomy were administered. Cardiac function was marginal despite inotropes. Subsequent improvement in the cardiorespiratory status allowed extubation on the 13th day of life. Intermittent hypertension of unclear origin was first noted on the 10th day of life, which was minimally responsive to diuretics and angiotensin-converting enzyme inhibitors.

On the 25th day of life, a chest radiograph revealed cardiomegaly, pulmonary edema, and, for the first time, probable calcification of the abdominal aorta (Figure 1). An echocardiogram showed a thick, poorly functioning

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