Regression of a large congenital hepatic arteriovenous malformation

Abstract

Congenital hepatic arteriovenous malformations are rarely seen in association with persistent neonatal pulmonary hypertension. We report the case of a full-term female newborn who presented with heart failure and respiratory distress soon after birth. Echocardiographic investigation revealed severe persistent pulmonary hypertension and patent ductus arteriosus. Here we report spontaneous regression in size of both the feeder vessel and the vascular bed of the congenital hepatic arteriovenous malformation. We postulate that our conservative use of oral heart failure therapy, in the form of diuretic agents and captopril, decreased the congestion and diameter of the affected vessels.

Keywords: Arteriovenous malformations/diagnosis/hepatic/therapy; captopril/therapeutic use; diagnosis, differential; diuretics/therapeutic use; heart failure/etiology/drug therapy; infant, newborn; pulmonary hypertension, persistent; remission, spontaneous; venous thrombosis.

Fig. 1

Computed tomograms (3-dimensional volume-rendered) in A) left lateral and B) anteroposterior views show a large, undefined (but well-circumscribed) vascular structure (arrows)—subdiaphragmatic and epigastric. The celiac trunk and right internal mammary artery (RIMA) supply this vascular structure. The left hepatic vein is prominent and appears to be draining the anomaly. IVC = inferior vena cava

Fig. 2

Four months later, computed tomograms (3-dimensional volume-rendered) in A) left lateral and B) anteroposterior views show that the left hepatic vein (arrow) draining the anomaly is still prominent, but that the vascular lesion itself (arrowhead) is markedly reduced in size.