title: Fontan Operation in a Patient with Severe Hypoplastic Right Pulmonary Artery, Single Ventricle, and Heterotaxy Syndrome.
abstract: Assessment of the pulmonary circulation status including pressure, resistance, size, and absence of anatomical distortion, is crucial to the successful Fontan operation. Most patients are found to have acceptable pulmonary arteries after previous palliation, although some degree of distortion is not uncommon. However, in rare instances, some patients have only one functioning lung with another pulmonary artery seriously hypoplastic or atretic. For theses patients, completion of a Fontan operation will be challenging. We reported a 17-year-old girl with a single ventricle and heterotaxy syndrome and only her left lung functioning, who underwent one-lung Fontan operation with a satisfactory result.
authors.affiliation: Department of Cardiovascular Surgery.
authors.forename: Jun-Yen
authors.initials: JY
authors.lastname: Pan
authors.affiliation: Department of Pediatrics, Kaohsiung Veterans General Hospital.
authors.forename: Chu-Chuan
authors.initials: CC
authors.lastname: Lin
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan.
authors.forename: Jen-Ping
authors.initials: JP
authors.lastname: Chang
keywords: Anomaly; Cardiovascular surgery; Congenital heart disease