Early and late results in pulmonary atresia

Abstract

The early and long-term results following surgical treatment of 24 infants with pulmonary atresia with an intact ventricular septum were reviewed. Pulmonary valvotomy was the operation most often performed, and we came to realize that this was effective when the preoperative right ventricular angiogram had shown an open conus up to the atretic valve. By contrast, no infant without a patent conus survived pulmonary valvotomy. We suggest that this subgroup of patients (8 of the 24 in this series) should have a systemic-pulmonary shunt followed later by reconstruction of the right ventricular outflow tract. Diminutive right ventricles will grow and dilate if a reasonable passageway is formed from right ventricle to pulmonary artery. Thus the long-term results have been excellent when this has been carried out, with normal right ventricular pressures and appearance on angiography in 7 patients followed up to 13 years.