Congenital hepatic arteriovenous malformation presenting with severe persistent pulmonary hypertension.

Congenital hepatic arteriovenous malformation is a rarely seen vascular malformation with persistent pulmonary hypertension in neonates. The authors report a full-term female newborn presenting with intractable heart failure and respiratory distress soon after birth. Investigation by echocardiography showed severe persistent pulmonary hypertension of the newborn and patent ductus arteriosus. The hepatic angiogram revealed congenital hepatic arteriovenous malformation; therefore, secondary pulmonary artery hypertension complicated with 'steal' phenomenon was conclusively diagnosed.