Absent Pulmonary Valve: Echocardiographic Features.

Congenital absence of the pulmonary valve, a rare anomaly, is characterized by absent or rudimentary pulmonary valve, often with annular stenosis, and aneurysmal dilatation of the pulmonary arteries. This defect is most commonly an accompaniment of tetralogy of Fallot but occasionally occurs alone. Four patients with this abnormality were examined by two-dimensional echocardiography at the Mayo Clinic. Doppler echocardiography provided hemodynamic assessment of the magnitude of outflow obstruction and valve regurgitation. The two-dimensional echocardiographic and Doppler features of absent or rudimentary pulmonary valve provided diagnostic information sufficient to proceed directly to surgical correction.