Congenital intrahepatic aorto-portal fistula presenting with cardiac failure

Abstract

Congenital intrahepatic arterio-portal fistulae (cIAPF) are rare, high-flow vascular malformations that usually present with portal hypertension. They almost never cause heart failure, unless there is associated congenital heart disease or the ductus venosus in patent. We present an unusual case of IAPF in an 11-day-old boy, who presented with features of cardiac failure associated with increased N-terminal pro-brain natriuretic peptide (NT pro-BNP). The IAPF arose directly from the aorta, separated from the hepatic artery and divided to separately supply both left and right portal veins. The ductus venosus was occluded. The IAPF was treated with embolization of the aorto-portal fistula, accessed through a direct percutaneous puncture of the fistula. Embolization was associated with an immediate clinical improvement and a rapid and sustained normalization of the NT pro-BNP level. A similar re-presentation was noted and treated with repeat embolization. The child is well on follow-up. To our knowledge, this is the first case of cIAPF, which was presented with cardiac failure when the ductus venosus has closed and has been treated successfully with direct, percutaneous transhepatic embolization of the fistula, twice. Serial clinical follow-up and ultrasonographical examinations have proven to be an effective strategy to detect recurrent fistulae.