Pulmonary hypertension in scimitar syndrome in infancy.

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Abstract
Four infants with the scimitar syndrome presented with failure to thrive, cardiac failure and pulmonary hypertension. In all cases systemic arteries arose from the upper abdominal aorta to perfuse the lower part of the right lung, and a large unobstructed anomalous pulmonary vein drained venous blood from almost the entire right lung to the inferior vena cava. An additional small pulmonary vein joined the inferior vena cava or right atrium and in 3 of the 4 cases angiography showed intrapulmonary connections betwen the 2 anomalous veins. At necropsy, in all 4 cases the right lung was hypoplastic. The bronchi were abnormal in size or number in 3 cases, but there was no sequestrated tissue. Microscopical examination showed normal peripheral airway and alveolar development. The pulmonary arterial branching pattern was deficient in 3 patients. Postmortem arteriography, dissection and microscopical studies showed that in areas of lung not perfused by branches of the right pulmonary artery the systemic arteries anastomosed with intrapulmonary arteries to distribute blood to a dilated capillary bed. In all tissue examined arterial medial thickness was increased in both lungs while vein wall thickness was normal. For comparison a 5th patient, more typical of the syndrome, was included; this patient did not have pulmonary hypertension and died from septicemia at 2 yr of age. Symptomatic infants with pulmonary hypertension probably have the most severe form of the scimitar syndrome. Sick infants should have a 2 stage correction, systemic arteries being ligated at the 1st procedure and the anomalous vein being reimplanted in the left atrium at a 2nd and later procedure, with closure of an atrial septal defect if present.