[Pulmonary atresia with intact interventricular septum]

Ten cases with pumonary atresia with intact interventricular septum were studied; they were divided in type I (6 cases) and type II (4 cases). In the cases with necropsy no distinct characteristics were observed in comparison to those reported in the literature. Cyanosis, heart failure, cardiomegaly and pulmonary oligohaemia were present in all cases. No correlation was found neither between right atrial middle pressure and the P wave amplitude (r = 0.014), nor between the AQRS and the right ventricular systolic pressure (r = 0.039). All the cases from type II showed hypertrophy of the two ventricles and only one case from type I showed right ventricular hypertrophy. In nine cases right ventricular hypertension was registered. Right atrial middle pressure and right ventricular telediastolic pressure were found increased in nine cases; the other one showed normal values (type II). The angiocardiographic study is a necessary diagnostic procedure. By means of it is possible to determine the ventricular cavity size, the right ventricular infundibulum characteristics and the atresic segment size.