A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt.
10.3346/jkms.2014.29.4.604
- Author:
Jeong Eun YI
1
;
Hae Ok JUNG
;
Ho Joong YOUN
;
Jong Young CHOI
;
Ho Jong CHUN
;
Jae Young LEE
Author Information
1. Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea. hojheart@catholic.ac.kr
- Publication Type:Case Reports
- Keywords:
Congenital Extrahepatic Portocaval Shunt;
Pulmonary Arterial Hypertension;
Heart Septal Defect, Ventricular;
Multifocal Nodular Regenerative Hyperplasia of the Liver
- MeSH:
Echocardiography, Doppler;
Humans;
Hypertension, Pulmonary/*diagnosis/drug therapy;
Iloprost/therapeutic use;
Liver/blood supply/radiography;
Magnetic Resonance Imaging;
Male;
Thoracic Arteries/ultrasonography;
Tomography, X-Ray Computed;
Vasodilator Agents/therapeutic use;
Vena Cava, Inferior/*abnormalities/ultrasonography;
Young Adult
- From:Journal of Korean Medical Science
2014;29(4):604-608
- CountryRepublic of Korea
- Language:English
-
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 microg/day) with improved condition and walking test. Physicians should note that congenital portocaval shunt may cause pulmonary hypertension.
