Cross-sectional echocardiographic measurements of right ventricular size and growth in patients with pulmonary atresia and intact ventricular septum.

Fifteen patients with pulmonary atresia or critical pulmonary stenosis and intact ventricular septum were studied. All were operated on in the neonatal period, with valvotomy or a systemic to pulmonary arterial shunt, or both. In 12 patients, right ventricular to pulmonary arterial communication was established in the neonatal period. In three patients, only systemic to pulmonary arterial shunts were constructed. Six patients died. The median follow-up period for the surviving patients was 64 months (range, 12-87 months). Right and left atrial and ventricular dimensions and areas, the tricuspid annular diameter, and the cross-sectional area of the aortic root were measured in cross-sectional echocardiograms from the neonatal period, at the age of 1 year, and at the latest clinical follow-up. A classification of right ventricular morphology was made, based on identification of the inlet, the trabecular, and the outlet parts. Most of the patients had hypoplastic right ventricles at birth but at the latest follow-up, seven of nine surviving patients had right ventricles in the normal range. Right ventricular growth was better in patients who were given a right ventricular to pulmonary arterial communication in the neonatal period and those with complete right ventricular anatomy. The patients who died had severely hypoplastic right ventricles and small tricuspid valves.