Portopulmonary Hypertension: Contemporary Paradigms in Diagnostic and Therapeutic Care

ABSTRACT

Portopulmonary hypertension (PoPH) is a pulmonary vascular complication of chronic liver disease. The condition is defined as precapillary pulmonary arterial hypertension (PAH) in the context of portal hypertension. Characterized by vasoconstriction and vascular remodeling of pulmonary arterial vessels, PoPH results in increased pulmonary vascular resistance and right ventricular failure. If left untreated, PoPH has poor prognosis indicated by a 5‐year survival of 4%–14%. The mainstay of treatment consists of vasomodulator therapy, although additional clinical trials in this specific patient population are needed. Liver transplantation should be considered only in carefully selected patients. This review summarizes current knowledge on PoPH pathogenesis and provides an update on screening, diagnosis and management.