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Published ahead of print on February 2, 2006, doi:10.1164/rccm.200510-1668OC
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American Journal of Respiratory and Critical Care Medicine Vol 173. pp. 1023-1030, (2006)
© 2006 American Thoracic Society
doi: 10.1164/rccm.200510-1668OC

Original Article

Pulmonary Arterial Hypertension in France

Results from a National Registry

Marc Humbert, Olivier Sitbon, Ari Chaouat, Michèle Bertocchi, Gilbert Habib, Virginie Gressin, Azzedine Yaici, Emmanuel Weitzenblum, Jean-François Cordier, François Chabot, Claire Dromer, Christophe Pison, Martine Reynaud-Gaubert, Alain Haloun, Marcel Laurent, Eric Hachulla and Gérald Simonneau

Service de Pneumologie, Centre des Maladies Vasculaires Pulmonaires, Hôpital Antoine Béclère, Assistance-Publique–Hôpitaux de Paris, Université Paris-Sud, Clamart, France; Service de Pneumologie, Hôpital Hautepierre, Strasbourg; Service de Pneumologie, Hôpital Louis-Pradel, Lyon; Service de Cardiologie, Hôpital de la Timone; Service de Pneumologie, Hôpital Sainte Marguerite, Marseille; Actelion Pharmaceuticals France, Paris; Service de Pneumologie, Hôpital de Brabois, Vandoeuvre-les-Nancy; Service de Chirurgie Thoracique, Hôpital du Haut Levesque, Bordeaux; Département Médecine Aiguë Spécialisée, Hôpital Michallon, Grenoble; Service de Pneumologie, Hôpital Laennec, Nantes; Service de Cardiologie, Hôpital Pontchaillou, Rennes; and Service de Médecine Interne, Hôpital Claude Huriez, Lille, France

Correspondence and requests for reprints should be addressed to Pr. Marc Humbert, M.D., Ph.D., Centre National de Référence de l'Hypertension Artérielle Pulmonaire, UPRES EA 2705, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Assistance Publique–Hôpitaux de Paris, Université Paris-Sud, 157 rue de la Porte de Trivaux, 92140 Clamart, France. E-mail: marc.humbert{at}abc.aphp.fr

Rationale: Pulmonary arterial hypertension (PAH) is an orphan disease for which the trend is for management in designated centers with multidisciplinary teams working in a shared-care approach.

Objective: To describe clinical and hemodynamic parameters and to provide estimates for the prevalence of patients diagnosed for PAH according to a standardized definition.

Methods: The registry was initiated in 17 university hospitals following at least five newly diagnosed patients per year. All consecutive adult (>= 18 yr) patients seen between October 2002 and October 2003 were to be included.

Main Results: A total of 674 patients (mean ± SD age, 50 ± 15 yr; range, 18–85 yr) were entered in the registry. Idiopathic, familial, anorexigen, connective tissue diseases, congenital heart diseases, portal hypertension, and HIV-associated PAH accounted for 39.2, 3.9, 9.5, 15.3, 11.3, 10.4, and 6.2% of the population, respectively. At diagnosis, 75% of patients were in New York Heart Association functional class III or IV. Six-minute walk test was 329 ± 109 m. Mean pulmonary artery pressure, cardiac index, and pulmonary vascular resistance index were 55 ± 15 mm Hg, 2.5 ± 0.8 L/min/m2, and 20.5 ± 10.2 mm Hg/L/min/m2, respectively. The low estimates of prevalence and incidence of PAH in France were 15.0 cases/million of adult inhabitants and 2.4 cases/million of adult inhabitants/yr. One-year survival was 88% in the incident cohort.

Conclusions: This contemporary registry highlights current practice and shows that PAH is detected late in the course of the disease, with a majority of patients displaying severe functional and hemodynamic compromise.

Key Words: cardiopulmonary hemodynamics • prevalence • pulmonary arterial hypertension registry • six-minute walk test




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