Abstract
Portopulmonary hypertension is an uncommon disease associated with high morbidity and mortality, so its early diagnosis and treatment are essential. We report here the case of a 57-year-old man with portopulmonary hypertension caused by chronic hepatosplenic schistosomiasis and also liver cirrhosis due to hepatitis C and alcoholism. As well as treating both diseases, portopulmonary hypertension was successfully managed with tadalafil and macitentan as maintenance therapy. This case reminds clinicians that pulmonary hypertension can be multifactorial, a good diagnosis and a multidisciplinary treatment can lead to improved prognosis.