Congenital Hepatic Arteriovenous Malformation: Often Missed Cause for Neonatal Pulmonary Arterial Hypertension

Abstract

Congenital hepatic arteriovenous malformations (AVM) are extremely rare, with an incidence of less than 1 in 1 hundred thousand, and the literature is limited to only a few case reports. They are characterised by an abnormal arterial connection to a fistulous venous connection within the liver. This results in high flow, low resistance circulation that causes high output cardiac failure. We report a late-preterm male newborn who presented with respiratory distress and signs of cardiac failure on day 1 of life. The newborn is diagnosed with hepatic AVM as the cause of severe pulmonary arterial hypertension (PAH) and cardiac failure. This case report, along with the literature review, emphasises the need for a high index of suspicion to look for hepatic AVM in a newborn presenting with unexplained PAH and cardiac failure and also discusses different management strategies for hepatic AVM.