title: A case of pulmonary arterial hypertension associated with congenital extrahepatic portocaval shunt.
abstract: Congenital extrahepatic portocaval shunt (CEPS) is a rare anomaly of the mesenteric vasculature in which the intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava, the left hepatic vein or the left renal vein. This uncommon disease is frequently associated with other malformations and mainly affects females. Here we report a case of pulmonary arterial hypertension associated with CEPS (Abernethy type 1b shunt) in a 20-yr-old man who was incidentally diagnosed during evaluation of multiple nodules of the liver. The patient was treated by inhalation of iloprost (40 µg/day) with improved condition and walking test. Physicians should note that congenital portocaval shunt may cause pulmonary hypertension.
authors.affiliation: Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors.forename: Jeong-Eun
authors.identifier: 0000-0003-2614-6983
authors.initials: JE
authors.lastname: Yi
authors.affiliation: Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors.forename: Hae-Ok
authors.identifier: 0000-0002-5102-9212
authors.initials: HO
authors.lastname: Jung
authors.affiliation: Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors.forename: Ho-Joong
authors.initials: HJ
authors.lastname: Youn
authors.affiliation: Division of Hepatology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors.forename: Jong Young
authors.initials: JY
authors.lastname: Choi
authors.affiliation: Department of Radiology, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors.forename: Ho Jong
authors.initials: HJ
authors.lastname: Chun
authors.affiliation: Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors.forename: Jae Young
authors.initials: JY
authors.lastname: Lee
mesh_terms: D015150:Echocardiography, Doppler; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000188:drug therapy; D016285:Iloprost / Q000627:therapeutic use; D008099:Liver / Q000098:blood supply / Q000000981:diagnostic imaging; D008279:Magnetic Resonance Imaging; D008297:Male; D013895:Thoracic Arteries / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed; D014665:Vasodilator Agents / Q000627:therapeutic use; D014682:Vena Cava, Inferior / Q000002:abnormalities* / Q000000981:diagnostic imaging; D055815:Young Adult
chemical_list: D014665:Vasodilator Agents; D016285:Iloprost
keywords: Congenital Extrahepatic Portocaval Shunt; Heart Septal Defect, Ventricular; Multifocal Nodular Regenerative Hyperplasia of the Liver; Pulmonary Arterial Hypertension
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