ABSENT PULMONARY VALVE WITH INTACT VENTRICULAR SEPTUM PRESENTING AS CARDIORESPIRATORY FAILURE AT BIRTH

Absent pulmonary valve with intact ventricular septum is a rare congen ital anomaly, Most cases are not diagnosed until years after birth, al though several cases have been reported in the literature in which car diorespiratory distress developed during the neonatal period, We prese nt a patient who showed improvement after fairly conservative therapy to alleviate the pulmonary vascular resistance, with ultimate palliati ve surgery, The aneurysmal dilatation of the pulmonary artery was for the most part confined to the proximal portion of the trunk, possibly because the ductus arteriosus was patent, The literature is reviewed.