Open AccessHemodynamic effects of ambrisentan-tadalafil combination therapy on progressive portopulmonary hypertension
Author(s) -
Yu Yamashita,
Ichizo Tsujino,
Takahiro Sato,
Asuka Yamada,
Taku Watanabe,
Hiroshi Ohira,
Masaharu Nishimura
Publication year - 2014
Publication title -
world journal of hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.913
H-Index - 55
ISSN - 1948-5182
DOI - 10.4254/wjh.v6.i11.825
Subject(s) - ambrisentan , portopulmonary hypertension , tadalafil , medicine , combination therapy , pulmonary hypertension , hemodynamics , cardiology , portal hypertension , sildenafil , cirrhosis , bosentan , receptor , endothelin receptor
Intravenous epoprostenol is recommended for World Health Organization functional class (WHO-FC) IV patients with pulmonary arterial hypertension (PAH) in the latest guidelines. However, in portopulmonary hypertension (PoPH) patients, advanced liver dysfunction and/or thrombocytopenia often makes the use of intravenous epoprostenol challenging. Here we report the cases of two WHO-FC IV PoPH patients who were successfully treated with a combination of two oral vasodilators used to treat PAH: ambrisentan and tadalafil. Oral vasodilator therapy using a combination of ambrisentan and tadalafil may be a safe and effective therapeutic option for WHO-FC IV PoPH patients and should be considered for selected patients with severe and rapidly progressing PoPH.