title: Hemodynamic effects of ambrisentan-tadalafil combination therapy on progressive portopulmonary hypertension.
abstract: 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.
authors.affiliation: Yu Yamashita, Ichizo Tsujino, Takahiro Sato, Asuka Yamada, Taku Watanabe, Hiroshi Ohira, Masaharu Nishimura, First Department of Medicine, Hokkaido University Hospital, Sapporo, Hokkaido 060-8648, Japan.
authors.forename: Yu
authors.initials: Y
authors.lastname: Yamashita
authors.affiliation: Yu Yamashita, Ichizo Tsujino, Takahiro Sato, Asuka Yamada, Taku Watanabe, Hiroshi Ohira, Masaharu Nishimura, First Department of Medicine, Hokkaido University Hospital, Sapporo, Hokkaido 060-8648, Japan.
authors.forename: Ichizo
authors.initials: I
authors.lastname: Tsujino
authors.affiliation: Yu Yamashita, Ichizo Tsujino, Takahiro Sato, Asuka Yamada, Taku Watanabe, Hiroshi Ohira, Masaharu Nishimura, First Department of Medicine, Hokkaido University Hospital, Sapporo, Hokkaido 060-8648, Japan.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Sato
authors.affiliation: Yu Yamashita, Ichizo Tsujino, Takahiro Sato, Asuka Yamada, Taku Watanabe, Hiroshi Ohira, Masaharu Nishimura, First Department of Medicine, Hokkaido University Hospital, Sapporo, Hokkaido 060-8648, Japan.
authors.forename: Asuka
authors.initials: A
authors.lastname: Yamada
authors.affiliation: Yu Yamashita, Ichizo Tsujino, Takahiro Sato, Asuka Yamada, Taku Watanabe, Hiroshi Ohira, Masaharu Nishimura, First Department of Medicine, Hokkaido University Hospital, Sapporo, Hokkaido 060-8648, Japan.
authors.forename: Taku
authors.initials: T
authors.lastname: Watanabe
authors.affiliation: Yu Yamashita, Ichizo Tsujino, Takahiro Sato, Asuka Yamada, Taku Watanabe, Hiroshi Ohira, Masaharu Nishimura, First Department of Medicine, Hokkaido University Hospital, Sapporo, Hokkaido 060-8648, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ohira
authors.affiliation: Yu Yamashita, Ichizo Tsujino, Takahiro Sato, Asuka Yamada, Taku Watanabe, Hiroshi Ohira, Masaharu Nishimura, First Department of Medicine, Hokkaido University Hospital, Sapporo, Hokkaido 060-8648, Japan.
authors.forename: Masaharu
authors.initials: M
authors.lastname: Nishimura
keywords: Ambrisentan; Portopulmonary hypertension; Tadalafil; Thrombocytopenia