Resolution of cavopulmonary shunt-associated pulmonary arteriovenous malformation after heart transplantation

A child with heterotaxia, azygous continuation of an interrupted inferior vena cava, single ventricle, and pulmonary atresia underwent the Kawashima modification of the Fontan procedure, which excluded hepatic venous return from the pulmonary blood flow. After the operation, the patient had development of pulmonary arteriovenous malformations, increasing cyanosis, and ventricular dysfunction. He underwent orthotopic heart transplantation at 7 years of age with prompt resolution of the pulmonary arteriovenous malformations.