Background and Aims: Portopulmonary hypertension can be defined as elevation of pulmonary arterial pressure and pulmonary vascular resistance in the setting of portal hypertension. Survival results in portopulmonary hypertension are contrasting and prognostic factors need to be identified. This study analyzed long-term survival in a large cohort of patients with portopulmonary hypertension with the aim of determining the independent variables affecting survival. Methods: We retrospectively analyzed charts of all patients referred to the French Referral Center for pulmonary arterial hypertension with the diagnosis of portopulmonary hypertension between 1984 and 2004. Results: The study population comprised 154 patients, with a male predominance (57%). Mean age at diagnosis was 49±11 years, 60% of patients were in New York Heart Association functional class III-IV, and mean 6-min walk distance was 326 ±116 m. Hemodynamic measurements showed a mean pulmonary arterial pressure of 53±13 mm Hg, cardiac index of 2.9±0.9 L.min^-1.m^-2 and pulmonary vascular resistance of 752±377 dyn.s.cm^-5. Portal hypertension was related to cirrhosis in 136 patients, with a severity assessed as follows: Child-Pugh class A 51%, Child-Pugh class B 38% Child-Pugh class C 11%. Overall survival rates at 1, 3, and 5 years were 88%, 75%, and 68%, respectively. Multivariate regression analysis individualized presence and severity of cirrhosis, and cardiac index as major independent prognostic factors. Conclusion: Prognosis in portopulmonary hypertension in mainly related to the presence and severity of cirrhosis and to cardiac function. The place of pulmonary arterial hypertension specific therapies remains to be determined in the setting of portopulmonary hypertension.