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Published ahead of print on February 2, 2006, doi:10.1164/rccm.200510-1668OC

Am. J. Respir. Crit. Care Med., Volume 173, Number 9, May 2006, 1023-1030

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Submitted on October 26, 2005
Accepted on February 2, 2006

Pulmonary Arterial Hypertension in France : Results from a National Registry

Marc Humbert1*, Olivier Sitbon1, Ari Chaouat2, Michele Bertocchi3, Gilbert Habib4, Virginie Gressin5, Azzedine Yaici1, Emmanuel Weitzenblum2, Jean-Francois Cordier3, Francois Chabot6, Claire Dromer7, Christophe Pison8, Martine Reynaud-Gaubert9, Alain Haloun10, Marcel Laurent11, Eric Hachulla12, and Gerald Simonneau1

1 Service de Pneumologie, Centre des Maladies Vasculaires Pulmonaires, UPRES EA2705, Hopital Antoine-Beclere, Assistance-Publique-Hopitaux de Paris, Univeriste Paris-Sud, Clamart, France, 2 Service de Pneumologie, Hopital Hautepierre, Strasbourg, France, 3 Service de Pneumologie, Hopital Louis-Pradel, Lyon, France, 4 Service de Cardiologie, Hopital de la Timone, Marseille, France, 5 Actelion Pharmaceuticals France, Paris, France, 6 Service de Pneumologie, Hopital de Brabois, Vandoeuvre-les-Nancy, France, 7 Service de Chirurgie Thoracique, Hopital du Haut Levesque, Bordeaux, France, 8 Departement Medecine Aigue Specialisee, Hopital Michallon, Grenoble, France, 9 Service de Pneumologie, UPRES EA3287, Hopital Sainte Marguerite, Marseille, France, 10 Service de Pneumologie, Hopital Laennec, Nantes, France, 11 Service de Cardiologie, Hopital Pontchaillou, Rennes, France, 12 Service de Medecine Interne, Hopital Claude Huriez, Lille, France

* To whom correspondence should be addressed. E-mail: marc.humbert{at}abc.aphp.fr.

Rationale. Pulmonary arterial hypertension 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 pulmonary arterial hypertension according to a standardised definition. Methods. The registry was initiated in 17 university hospitals following at least 5 newly diagnosed patients per year. All consecutive adult (≥ 18 years) patients seen between October 2002 and October 2003 were to be included. Main Results. 674 patients (mean±SD age of 50±15 years (range 18-85)) were entered in the registry. Idiopathic, familial, anorexigen, connective tissue diseases, congenital heart diseases, portal hypertension and human immunodeficiency virus infection-associated pulmonary arterial hypertension 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 meters. Mean pulmonary artery pressure, cardiac index and pulmonary vascular resistance index were 55±15 mmHg, 2.5±0.8 L/min/m2, and 20.5±10.2 mmHg/L/min/m2, respectively. The low estimates of prevalence and incidence of pulmonary arterial hypertension in France were 15.0 cases/million of adult inhabitants and 2.4 cases/million of adult inhabitants/year. One-year survival was 88% in the incident cohort. Conclusions. This contemporary registry highlights current practice and shows that pulmonary arterial hypertension is still 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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