Echocardiographic diagnosis of pulmonary atresia with intact ventricular septum

The echocardiographic features of pulmonary valvar atresia with intact ventricular septum are described. The pulmonary valve fails to open during systole: there is a large pre-systolic 'a' wave dip, but the pulmonary valve membrane then returns to its closed diastolic position where it remains for the remainder of the cardiac cycle. The echocardiogram also provides valuable information about right ventricular size, the tricuspid valve, and aorta-mitral and aorta-septal relations.