title: Staged Fontan operation for children with heterotaxy syndrome, bilateral ductus arteriosus, and nonconfluent pulmonary artery.
abstract: We report two cases of successful Fontan operation in children with heterotaxy syndrome associated with univentricular physiology and absent and nonconfluent central pulmonary arteries with both distal pulmonary arteries directly connected to the ipsilateral ductus arteriosus. After unilateral systemic-pulmonary shunt, the central pulmonary artery was reconstructed with a polytetrafluoroethylene prosthetic graft concomitantly with bidirectional cavopulmonary shunt. Finally, extracardiac total cavopulmonary connection was performed as an off-pump procedure. Children with bilateral ductus arteriosus and a nonconfluent pulmonary artery with univentricular physiology present a particular challenge in regard to completing Fontan operations. Careful attention should be directed at ensuring balanced growth of the bilateral distal pulmonary arteries. When planning reconstruction of the central pulmonary artery with a prosthesis, late reconstruction may be beneficial, as it enables utilization of a larger-caliber graft, obviating the need for replacement during a subsequent Fontan operation.
authors.affiliation: Department of Cardiovascular Surgery, Tsukuba University Hospital, 2-1-1 Amakubo, Tsukuba, Ibaraki 305-8576, Japan. s-kane@mail.hosp.tsukuba.ac.jp
authors.forename: Shinya
authors.initials: S
authors.lastname: Kanemoto
authors.forename: Masakazu
authors.initials: M
authors.lastname: Abe
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Ban
authors.forename: Seigo
authors.initials: S
authors.lastname: Gomi
mesh_terms: D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D019917:Blood Vessel Prosthesis Implantation; D013505:Digestive System Surgical Procedures; D004374:Ductus Arteriosus, Patent / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D006439:Hemodynamics; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D011859:Radiography; D013577:Syndrome; D016896:Treatment Outcome