Management of portopulmonary hypertension: new perspectives

Portopulmonary hypertension (PPHTN) is a known complication of cirrhosis. Moderate-to-severe PPHTN implies an extremely poor prognosis. It occurs in 5%-10% of patients referred for liver transplantation (LT), and probably with an higher incidence in patients with large portosystemic shunts. Patients with moderate-to-severe pulmonary hypertension have been previously excluded from LT because of the extremely high surgical risk and since the post-transplant outcome reported was poor. Recently, new perspectives in the management of patients with portopulmonary hypertension are emerging. In fact, some pulmonary vasoactive drugs have become routine in the treatment of patients with idiopathic pulmonary hypertension. These drugs, particularly epoprostenol, have been recently introduced in the treatment of patients with PPHTN, and have been shown to be effective in reducing pulmonary artery pressure as well as pulmonary vascular resistances. Furthermore, recent studies seem to demonstrate that treatment with pulmonary vasoactive drugs could allow liver transplantation with acceptable surgical risks and excellent survival. Although there are not large series nor prospective studies addressing this topic, the clinical scenario of patients with PPHTN seems to be positively changing.