Portopulmonary hypertension associated with congenital absence of the portal vein treated with bosentan.
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.
Postgraduate Clinical Training Center, Kawasaki Medical School Hospital Kurashaki, Japan. thino-kws@umin.ac.jp
Tomoko
T
Hino
Akihiro
A
Hayashida
Noriko
N
Okahashi
Nozomi
N
Wada
Nozomi
N
Watanabe
Kikuko
K
Obase
Yoji
Y
Neishi
Takahiro
T
Kawamoto
Hiroyuki
H
Okura
Kiyoshi
K
Yoshida
D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D002648:Child; D006801:Humans; D006975:Hypertension, Portal / Q000188:drug therapy; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology*; D008297:Male; D011169:Portal Vein / Q000002:abnormalities*; D013449:Sulfonamides / Q000627:therapeutic use*; D016896:Treatment Outcome
D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan