title: Modification of Hepatic Venous Conduit to Manage Pulmonary Arteriovenous Malformations.
abstract: 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.
authors.affiliation: Department of Surgery, Section of Pediatric Cardiothoracic Surgery, University of Utah, Salt Lake City, UT, USA robert.mcrae@hsc.utah.edu.
authors.forename: Robert O
authors.initials: RO
authors.lastname: McRae
authors.affiliation: Division of Pediatric Cardiothoracic Surgery, Primary Children's Hospital, Salt Lake City, UT, USA.
authors.forename: Linda M
authors.initials: LM
authors.lastname: Lambert
authors.affiliation: Department of Pediatrics, Division of Cardiology, University of Utah, Salt Lake City, UT, USA.
authors.forename: Richard V
authors.initials: RV
authors.lastname: Williams
authors.affiliation: Department of Pediatrics, Division of Cardiology, University of Utah, Salt Lake City, UT, USA.
authors.forename: Mary H
authors.initials: MH
authors.lastname: Martin
authors.affiliation: Department of Surgery, Section of Pediatric Cardiothoracic Surgery, University of Utah, Salt Lake City, UT, USA.
authors.forename: Phillip T
authors.initials: PT
authors.lastname: Burch
mesh_terms: D001164:Arteriovenous Fistula / Q000601:surgery*; D018729:Fontan Procedure / Q000379:methods*; D006503:Hepatic Veins / Q000002:abnormalities*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000601:surgery
keywords: Fontan operation; bifurcated conduit; hepatic venous conduit; heterotaxy; pulmonary arteriovenous malformations