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Portopulmonary Hypertension

Survival and Prognostic Factors

¹ Université Paris-Sud 11, UPRES 2705, Service de Pneumologie et Réanimation, Hôpital Antoine Béclère, Assistance Publique-Hôpitaux de Paris, Clamart, France; and ² INSERM U773, Centre de Recherche Biomédical Bichat-Beaujon, CRB3 and Service d'Hépatologie, Hôpital Beaujon, Assistance Publique-Hôpitaux de Paris, Clichy, France

Correspondence and requests for reprints should be addressed to Dr. Jérôme Le Pavec, M.D., Service de Pneumologie, Hôpital Antoine Béclère, 157 rue de la Porte de Trivaux, 92140 Clamart, France. E-mail: lepavec{at}gmail.com

Rationale: Portopulmonary hypertension (PoPH) can be defined as elevation of pulmonary arterial pressure and pulmonary vascular resistance in the setting of portal hypertension. Survival results in PoPH are contrasting, and prognostic factors need to be identified.

Objectives: To analyze long-term survival in a large cohort of patients with PoPH 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 PoPH between 1984 and 2004.

Measurements and Main Results: The study population comprised 154 patients; 57% male. Mean age at diagnosis was 49 ± 11 years, 60% of patients were in New York Heart Association functional class III-IV, and mean 6-minute 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/m², and pulmonary vascular resistance of 752 ± 377 dyn/s/cm⁵. 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 yr were 88, 75, and 68%, respectively. Multivariate regression analysis individualized the presence and severity of cirrhosis and cardiac index as major independent prognostic factors.

Conclusions: Prognosis in PoPH is 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 PoPH.

Key Words: pulmonary hypertension • portal hypertension • portopulmonary hypertension • cirrhosis • survival

AT A GLANCE COMMENTARY Scientific Knowledge on the Subject Portal hypertension is an identified risk factor for pulmonary arterial hypertension. Survival data for patients with portopulmonary hypertension are conflicting, and prognostic factors have not been identified. What This Study Adds to the Field Prognosis in portopulmonary hypertension (PoPH) is 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 PoPH.