Myocardial infarction of hypertrophied right ventricle may occur. Prominent A wave of the jugular venous pulse is observed. Tricuspid regurgitation occasionally is present. This maldevelopment is characterized by fusion of leaflet commissures, resulting in a thickened and domed appearance to the valve. Aberrant flow patterns in utero also may be, in part, associated with maldevelopment of the pulmonary valve.
Family history is a risk factor.
Laboratory evaluation usually is not helpful. CXR demonstrates a prominent main pulmonary artery segment but a normal heart size Pulmonary vascular markings are usually normal but may be decreased in severe pulmonary stenosis. The sine qua non of diagnosis is two dimensional and Doppler echocardiography. Multiple views will be utilized to confirm the absence of coexistent congenital cardiac disease. Multiple views can confirm the absence of coexistent congenital cardiac disease.