PULMONARY VASCULAR DISEASE

Portopulmonary hypertension (PoPH), a serious complication of advanced liver disease, is defi ned as pulmonary arterial hypertension (PAH) associated with portal hypertension. 1,2 Although categorized by the fourth World Symposium on Pulmonary Hyper-tension (Dana Point, 2008) 2 within the same group of diseases causing pulmonary hypertension as idio-pathic PAH (IPAH) (group 1 based upon pathology and disease mechanisms), relatively little is known about the natural history, response to therapy, and prognosis of patients with PoPH. PoPH is characterized by pulmonary vascular obstruc-tion due to the abnormal proliferation of pulmonary Background: We evaluated survival and hospitalization rates in patients with group 1 portopulmonary hypertension (PoPH) in the Registry to Evaluate Early and Long-term Pulmonary Arterial Hyper-tension Disease Management (REVEAL Registry)