While the Fontan operation is a reliable treatment option for many complex congenital heart defects, the development of pulmonary arteriovenous malformations (PAVMs) remains a problematic outcome for some Fontan patients. Pulmonary arteriovenous malformations stem from an imbalance of hepatic blood flow in the pulmonary system. Balancing this hepatic flow has shown promising results in the treatment of PAVMs. We report the clinical course of a young patient with heterotaxy syndrome and an unbalanced right dominant atrioventricular septal defect. This patient developed PAVMs following a Fontan procedure, however, the PAVMs were resolved following the revision of the original Fontan conduit to a bifurcated conduit.
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