A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt
Journal of Korean Medical Science
;
: 604-608, 2014.
Article
in English
| WPRIM
| ID: wpr-65538
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Thoracic Arteries
/
Vasodilator Agents
/
Vena Cava, Inferior
/
Magnetic Resonance Imaging
/
Echocardiography, Doppler
/
Tomography, X-Ray Computed
/
Iloprost
/
Hypertension, Pulmonary
/
Liver
Limits:
Humans
/
Male
Language:
English
Journal:
Journal of Korean Medical Science
Year:
2014
Type:
Article
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