Congenital pulmonary atresia with ventricular septal defect: Review of the clinical course of fifty patients with assessment of the results of palliative surgery

Abstract

Fifty patients with congenital pulmonary atresia and ventricular septal defect are separated into three relatively distinct physiologic, clinical and therapeutic categories, according to the amount of flow of pulmonary blood through aorticopulmonary communications. Clinical characteristics of the entire group are summarized, and additional findings that permitted separation of patients into one of three therapeutic groups are analyzed. Twenty-two infants with minimal aorticopulmonary communications had maximal hypoxia, and only those in whom successful palliative surgery was accomplished survived the first year of life. In 13 patients pulmonary flow was adequate during infancy but thereafter became sufficiently limited to require surgery. Fifteen patients had adequate pulmonary blood flow in infancy or childhood, and in 2 of these increased pulmonary flow through collaterals led to heart failure in early infancy. None of these 15 patients has required palliative surgery so far. The methods and results of palliative surgery in 28 infants and children are presented. An ascending aorta to right pulmonary artery shunt is the procedure of choice in the very young. Other types of systemic to pulmonary artery anastomosis may be utilized in older children. Successful total correction of patients with congenital pulmonary atresia and ventricular septal defect requires the presence of pulmonary arteries of adequate size. Half our patients had pulmonary arteries at least 5 mm. in diameter and therefore could be considered eligible for complete repair. Palliative surgery in infancy and early childhood, in addition to alleviating the catastrophic consequences of anoxia, also may promote sufficient growth of the pulmonary arteries to make definitive surgery more feasible at a later age.