title: Redirection of hepatic drainage for treatment of pulmonary arteriovenous malformations following the Fontan procedure.
abstract: The development of unilateral pulmonary arteriovenous malformations in patients after total cavopulmonary connection with an extracardiac conduit has been reported. Unequal distribution of hepatic venous flow to the lung is theorized to be the causative factor. We report the surgical management of pulmonary arteriovenous malformations in a patient with heterotaxy syndrome, single ventricle, and interrupted inferior vena cava. The patient had previously undergone a total cavopulmonary connection with an extracardiac conduit draining hepatic venous flow to the right branch pulmonary artery. In the subsequent operation, we redirected the extracardiac conduit to the innominate vein. This operation provided the affected lung with hepatic venous blood without exposing the patient to the morbidity associated with cardiopulmonary bypass.
authors.affiliation: Department of Cardiothoracic Surgery, Mount Sinai School of Medicine, One Gustave L. Levy Place, PO Box 1028, New York, NY 10029, USA. ihuiwu@ha.mc.ntu.edu.tw
authors.forename: I-Hui
authors.initials: IH
authors.lastname: Wu
authors.forename: Khanh H
authors.initials: KH
authors.lastname: Nguyen
mesh_terms: D001165:Arteriovenous Malformations / Q000503:physiopathology / Q000601:surgery*; D002648:Child; D004322:Drainage; D018729:Fontan Procedure; D018610:Heart Bypass, Right; D006330:Heart Defects, Congenital / Q000601:surgery; D006503:Hepatic Veins / Q000002:abnormalities* / Q000503:physiopathology; D006801:Humans; D008102:Liver Circulation; D008297:Male; D011183:Postoperative Complications / Q000209:etiology / Q000503:physiopathology / Q000601:surgery; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000503:physiopathology; D011652:Pulmonary Circulation; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000503:physiopathology; D012086:Reoperation