• Olivier Sitbon, Marc Humbert, Hilario Nunes, Florence Parent, Gilles Garcia, Philippe Hervé, Maurizio Rainisio, and Gérald Simonneau.
• Service de Pneumologie et Réanimation, UPRES EA 2705 on Pulmonary Vascular Diseases, Hôpital Antoine Béclère, Université Paris-Sud, Clamart, France. olivier.sitbon@abc.ap-hop-paris.fr
• J. Am. Coll. Cardiol. 2002 Aug 21; 40 (4): 780-8.

ObjectivesWe sought to determine the factors associated with long-term survival in patients with primary pulmonary hypertension (PPH) treated with continuous epoprostenol infusion.BackgroundEpoprostenol improves survival in patients with PPH in New York Heart Association (NYHA) functional class III or IV. However, some patients do not benefit from epoprostenol and must be considered for lung transplantation. The best timing for listing these patients on a lung transplantation program is currently unknown.MethodsBetween December 1992 and January 2001, 178 patients with PPH in NYHA functional class III or IV were treated with epoprostenol. The 6-min walk test (WT) and right-sided heart catheterization were performed at baseline, after three months on epoprostenol and thereafter once a year.ResultsOverall survival rates at one, two, three, and five years were 85%, 70%, 63%, and 55%, respectively. On univariate analysis, the baseline variables associated with a poor outcome were a history of right-sided heart failure, NYHA functional class IV, 6-min WT or=12 mm Hg, and mean pulmonary artery pressure <65 mm Hg. On multivariate analysis, including both baseline variables and those measured after three months on epoprostenol, a history of right-sided heart failure, persistence of NYHA functional class III or IV at three months, and the absence of a fall in total pulmonary resistance of >30%, relative to baseline, were associated with poor survival.ConclusionsSurvival of patients with PPH treated with epoprostenol depends on the severity at baseline, as well as the three-month response to therapy. These findings suggest that lung transplantation should be considered in a subset of patients who remain in NYHA functional class III or IV or in those who cannot achieve a significant hemodynamic improvement after three months of epoprostenol therapy, or both.

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