title: Severe peripheral pulmonary artery stenosis is not a contraindication to liver transplantation in Alagille syndrome.
abstract: We described a case of Alagille syndrome with severe peripheral pulmonary artery stenosis and very high right ventricular pressure that underwent successful living-related liver transplantation without any peri-operative and mid-term postoperative complication because of this cardiac malformation. The aim of this report is to point out that the severe pulmonary artery stenosis may be a risk factor but not a contraindication to liver transplantation in patients with Alagille syndrome.
authors.affiliation: Department of Pediatric Gastroenterology, Hepatology and Nutrition, Başkent University Medical Faulty, Ankara, Turkey. fozcay@yahoo.com
authors.forename: Figen
authors.initials: F
authors.lastname: Ozçay
authors.forename: Birgül
authors.initials: B
authors.lastname: Varan
authors.forename: Kürşad
authors.initials: K
authors.lastname: Tokel
authors.forename: Ilker
authors.initials: I
authors.lastname: Cetin
authors.forename: Aydin
authors.initials: A
authors.lastname: Dalgiç
authors.forename: Mehmet
authors.initials: M
authors.lastname: Haberal
mesh_terms: D016738:Alagille Syndrome / Q000150:complications / Q000601:surgery*; D000792:Angiography; D001157:Arterial Occlusive Diseases / Q000150:complications* / Q000000981:diagnostic imaging; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D008297:Male; D011651:Pulmonary Artery; D012720:Severity of Illness Index