ABSENT PULMONARY VALVE WITH VENTRICULAR SEPTAL DEFECT (APV WITH VSD)

Abstract

SA characteristic clinical picture, recognizable in the o first hours of life, consisting of extreme respiratory distress, cyanosis, a to-and-fro murmur at the upper left sternal border, and bilateral pneumothorax is described in two infants. This syndrome, the result of aneurysmal dilitation of the main and branch pulmonary arteries producing compression of the bronchi, is unusual but well-described in slightly older infants. Chest tube drainage and respirator support were required by 5 hours of age in both our patients. Immediate and lasting relief from respiratory obstruction was obtained by surgical reimplantation of the right pulmonary artery to main pulmonary artery, anterior to the ascending aorta, as described by Litwin, et al., in the second infant. Severe pulmonary valve insufficiency with intractable right heart failure and inadequate net pulmonary flow persisted, leading us to believe that this syndrome, if present at birth, is characteristic of the severest type of pulmonary valve insufficiency. Prompt recognition, relief of respiratory obstruction and, probably, total correction of the cardiac lesion with a valved pulmonary conduit will be necessary for management of these patients.

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