PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM

1. Babies with pulmonary atresia and intact ventricular septum rarely survive for more than a few weeks after birth. Prompt diagnosis and recent advances in surgical treatment now offer greatly improved possibilities for long term survival.2. Emergency angiocardiography is necessary for precise preoperative diagnosis.a. Venous or right atrial angiocardiograms are generally inadequate for accurate diagnosis.b. Selective right ventricular angiocardiography best shows the size and position of the right ventricle, the pulmonary valvular atresia and the intact ventricular septum, but poorly demonstrates the left heart and the lung vessels. Tricuspid atresia is ruled out.c. Selective left ventricular angiocardiography shows the left heart, aorta and pulmonary blood supply as well as the distal surface of the atretic pulmonary valve. The right coronary artery position permits an estimate of right ventricular size.d. A combination of both selective right and left ventricular angiocardiograms is ideal. Either exam...