Echocardiographic pulsed Doppler features of absent pulmonary valve syndrome in the neonate.

M mode echocardiography was used to evaluate nine neonates with absent pulmonary valve syndrome. Six were also studied with two dimensional echocardiography and two with pulsed Doppler echocardiography. M mode echocardiography demonstrated a large, overriding great artery and right ventricular dilation in all nine patients and abnormal septal motion in eight. Two dimensional echocardiography demonstrated aneurysmal dilation of the main pulmonary artery in all six patients studied. Pulsed Doppler echocardiography in the two infants studied demonstrated anterograde systolic and retrograde diastolic flow in the main pulmonary artery and right ventricular outflow tract. The echocardiographic features of absent pulmonary valve syndrome appear to be unique and allow the diagnosis to be made noninvasively, thus obviating or delaying the need for potentially high risk cardiac catheterization.