Case Report
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World J Hepatol. Nov 27, 2014; 6(11): 825-829
Published online Nov 27, 2014. doi: 10.4254/wjh.v6.i11.825
Hemodynamic effects of ambrisentan-tadalafil combination therapy on progressive portopulmonary hypertension
Yu Yamashita, Ichizo Tsujino, Takahiro Sato, Asuka Yamada, Taku Watanabe, Hiroshi Ohira, Masaharu Nishimura
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
Author contributions: Yamashita Y contributed to the management of the patients, paper drafting; Tsujino I and Sato T contributed to the management of the patients, edition of the manuscript; Yamada A, Watanabe T and Ohira H contributed to the management of the patients; Nishimura M contributed to the edition and finalization of the manuscript.
Correspondence to: Ichizo Tsujino, MD, PhD, First Department of Medicine, Hokkaido University Hospital N14, W5, Kita-ku, Sapporo, Hokkaido 060-8648, Japan. itsujino@med.hokudai.ac.jp
Telephone: +81-11-7065911 Fax: +81-11-7067899
Received: June 10, 2014
Revised: August 27, 2014
Accepted: September 16, 2014
Published online: November 27, 2014
Processing time: 163 Days and 7 Hours
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.

Keywords: Portopulmonary hypertension; Ambrisentan; Tadalafil; Thrombocytopenia

Core tip: Advanced liver dysfunction and/or thrombocytopenia often hamper the use of intravenous epoprostenol in patients with portopulmonary hypertension (PoPH). However, recent progress in the oral treatment for pulmonary hypertension (PH) has enabled better clinical outcome in severe PH patients. Here we report two World Health Organization functional class IV patients with PoPH and thrombocytopenia who were successfully treated with ambrisentan and tadalafil. Oral vasodilator therapy using a combination of ambrisentan and tadalafil may be a safe and effective therapeutic option for patients with PoPH and advanced thrombocytopenia.