Cyanosis after Kawashima-Fontan: hybrid approach to mixing and matching flows.

Abstract

Surgical approaches to a single ventricle with absent infrahepatic segment of the inferior vena cava include a bidirectional Glenn and separate hepatic venous connection to the pulmonary arteries. If sites of insertion into the pulmonary arteries are widely separated, hepatic flow is directed to only one lung. Pulmonary arteriovenous malformations may form in the contralateral lung and result in severe cyanosis. We report a case of resolution of pulmonary arteriovenous malformations after successful rerouting of hepatic venous flow to the hemi-azygous vein in a 16-year-old patient. A dilatable pulmonary artery band reduced cyanosis by limiting flow to the affected lung until resolution of pulmonary arteriovenous malformations.