title: Successful surgical pulmonary artery reconstruction in arterial tortuosity syndrome.
abstract: The outcomes of surgical repair for diffuse pulmonary artery stenoses due to arterial tortuosity syndrome are unknown. We report a 1-year-old girl with tortuosity syndrome who presented with suprasystemic right ventricular pressure due to distortion and stenoses of the pulmonary artery branches. We describe the operative findings of this unique pathology and the surgical techniques used to completely reconstruct the pulmonary arterial tree, with normalization of the right ventricular pressure.
authors.affiliation: Division of Cardiac Surgery, King Abdulaziz Medical City, Riyadh, Saudi Arabia. khaldiab@ngha.med.sa
authors.forename: Abdulaziz
authors.initials: A
authors.lastname: Al-Khaldi
authors.forename: Abdulmohsen
authors.initials: A
authors.lastname: Alharbi
authors.forename: Omar
authors.initials: O
authors.lastname: Tamimi
authors.forename: Yasser
authors.initials: Y
authors.lastname: Mohammed
mesh_terms: D000792:Angiography; D001157:Arterial Occlusive Diseases / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D003937:Diagnosis, Differential; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D011651:Pulmonary Artery / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery*; D015607:Stents; D013577:Syndrome; D054079:Vascular Malformations / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D014656:Vascular Surgical Procedures / Q000379:methods*