Hepatic Venous Blood and the Development of Pulmonary Arteriovenous Malformations in Congenital Heart Disease

Abstract

Background Pulmonary arteriovenous malformations (PAVMs) are a known complication after some types of cavopulmonary anastomoses (CVPAs). Their cause is unknown, but they may be related to the absence of pulsatile flow or the presence or absence of circulating factors. These PAVMs are diffuse and are presumed to be progressive and irreversible. Methods and Results All patients with congenital heart disease (CHD) seen at Children’s Hospital, Boston, Mass, between 1970 and 1993 were reviewed. We report on the 10 patients with CHD who were found to have developed PAVMs, as diagnosed by cardiac catheterization. Diagnoses included heterotaxy syndrome/polysplenia, with interrupted inferior vena cava and hepatic veins draining to the right atrium (n=6); heterotaxy/asplenia (n=1); corrected transposition with pulmonary stenosis (n=1); and biliary atresia and associated CHD (n=2). PAVMs were diagnosed 0.1 to 7.0 years (median, 3.5 years) after creation of a CVPA that resulted in exclusion of hepatic venous flow fro...