Pulmonary atresia with intact ventricular septum: a quantitative cineventriculographic study of the right and left ventricular function.

Quantitative cineventriculographic measurements (ventricular volumes, ejection fraction, and myocardial mass) were obtained in 15 neonates with pulmonary atresia and intact ventricular septum. There was a wide dimensional range for the right ventricle, from a reduced through normal to enlarged. A restrictive tricuspid valve (less than 12 mm) was associated with a small or diminutive right ventricle. A normal function of the right ventricle was present only in those cases with normal-sized chambers. The left ventricular end-diastolic volume was always greater than normal. The ejection fraction was normal only if a normal myocardial mass was present. Additional abnormalities were frequent in the myocardium of both ventricles, such as extreme thinning of the wall of the right ventricle and hypoplasia or fibroelastosis of the left ventricle. All these factors can lead to a poor surgical prognosis despite good anatomical correction.