Unusual vascular anomalies causing persistent pulmonary hypertension in a newborn

A unique case of pulmonary vascular anomalies causing persistent pulmonary hypertension in a newborn is described. The child died 3 days after birth. Necropsy revealed marked hypoplasia of the right and left pulmonary arteries with a normal main pulmonary artery, patent ductus arteriosus, bilateral systemic arteries to the lungs from the abdominal aorta, and partial anomalous pulmonary venous connection. Quantitative morphometric techniques demonstrated slight abnormalities of alveolar development and severe arterial medial hypertrophy with abnormal extension of muscle into small peripheral arteries. Bronchopulmonary development appeared relatively normal in spite of the vascular abnormalities.