J Korean Med Sci.
2014 Apr;29(4):604-608. 10.3346/jkms.2014.29.4.604.
A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt
- Affiliations
-
- 1Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea. hojheart@catholic.ac.kr
- 2Division of Hepatology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
- 3Department of Radiology, College of Medicine, The Catholic University of Korea, Seoul, Korea.
- 4Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea.
- KMID: 1774470
- DOI: http://doi.org/10.3346/jkms.2014.29.4.604
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.
Keyword
MeSH Terms
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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
Iloprost
Vasodilator Agents
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