Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
CASE REPORTS
Portopulmonary Hypertension Associated with Congenital Absence of the Portal Vein Treated with Bosentan
Tomoko Hino, Akihiro Hayashida, Noriko Okahashi, Nozomi Wada, Nozomi Watanabe, Kikuko Obase, Yoji Neishi, Takahiro Kawamoto, Hiroyuki Okura, Kiyoshi Yoshida
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Keywords: congenital absence of the portal vein, portopulmonary hypertension, pulmonary hypertension, bosentan therapy
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2009 Volume 48 Issue 8 Pages 597-600

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Abstract

Portopulmonary hypertension (PPHTN) is pulmonary arterial hypertension (PAH) associated with portal hypertension. It is a common condition among liver transplantation candidates; however, its association with congenital absence of the portal vein (CAPV) has not yet been established. CAPV is a very rare developmental anomaly, which is usually accompanied by abnormal mesenteric drainage that bypasses the liver. Here, we report a rare case of severe PPHTN secondary to CAPV.

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© 2009 by The Japanese Society of Internal Medicine
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