The surgical anatomy of pulmonary atresia with ventricular septal defect: pseudotruncus.

This is a study of 172 heart specimens with pulmonary atresia and ventricular septal defect (VSD)--pseudotruncus. These are divided into simple and complicated types. In the simple type the following may be of importance from the surgical standpoint: (1) the size of the pulmonary arteries, (2) the origin of the aorta, (3) the amount of pulmonary flow, (4) the size of the left side of the heart, (5) the presence of bronchial and/or abnormal systemic supply to the lungs, (6) the coronary circulation, and (7) the presence of intra- and extracardiac associated abnormalities. The following factors may be important in the complicated types: (1) Complete transposition may accompany the complex; (2) instead of the VSD there may be a common atrioventricular (AV) orifice; (3) the pulmonary atresia with VSD may be associated with tricuspid or mitral atresia or with common or single ventricle; (4) the entity may be associated with abnormal position of the entire heart or some of its component chambers.