Published online Nov 27, 2014. doi: 10.4254/wjh.v6.i11.825
Revised: August 27, 2014
Accepted: September 16, 2014
Published online: November 27, 2014
Processing time: 163 Days and 7 Hours
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.
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.