Pulmonary arterial hypertension in France: results from a national registry.
BACKGROUND
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 (> or = 18 yr) patients seen between October 2002 and October 2003 were to be included.


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/m(2), and 20.5 +/- 10.2 mm Hg/L/min/m(2), 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.
Humbert
Marc
M
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. marc.humbert@abc.aphp.fr
Sitbon
Olivier
O
Chaouat
Ari
A
Bertocchi
Michèle
M
Habib
Gilbert
G
Gressin
Virginie
V
Yaici
Azzedine
A
Weitzenblum
Emmanuel
E
Cordier
Jean-François
JF
Chabot
François
F
Dromer
Claire
C
Pison
Christophe
C
Reynaud-Gaubert
Martine
M
Haloun
Alain
A
Laurent
Marcel
M
Hachulla
Eric
E
Simonneau
Gérald
G
D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D017079:Exercise Tolerance / Q000502:physiology; D005260:Female; D005602:France / Q000453:epidemiology; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology; D015994:Incidence; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012042:Registries; D015996:Survival Rate