A full-term baby presented at 2 weeks of life with severe congestive heart failure. On clinical examination a 2/6 holosystolic murmur was found, bounding femoral pulses and hepatomegaly. Echocardiography found massive dilatation of both ventricles with severe atrioventricular regurgitation and pulmonary hypertension ( Fig. 1 ). No intracardiac shunt was found but an abnormal right pulmonary artery arising from the ascending aorta was diagnosed ( Figs. 2 et 3 ). On Doppler investigation of the aortic arch, diastolic reflux from the aorta to the right pulmonary artery was found ( Fig. 4 ). The patient underwent immediate and successful complete surgical repair, consisting of detachment of the anomalous pulmonary artery from the ascending aorta with direct anastomosis to the main pulmonary artery.
