Treatment Barriers in Portopulmonary Hypertension

Portopulmonary hypertension (PoPH) is a form of pulmonary arterial hypertension (PAH) that can develop as complication of portal hypertension. Treatment of PoPH includes PAH-specific therapies and in certain cases, such therapies are necessary to facilitate a successful liver transplantation. A significant number of barriers may limit the adequate treatment of patients with PoPH and explain the poorer survival of these patients when compared to other types of PAH. Until recently, only one randomized controlled trial has included PoPH patients and the majority of treatment data is derived from relatively small observational studies. In the present manuscript we review some of the barriers in the treatment of patients with PoPH and implications for liver transplantation.