Pulmonary arterial hypertension in France: results from a national registry
| Autor: | Olivier Sitbon, Marc Humbert, François Chabot, Claire Dromer, Gérald Simonneau, Azzedine Yaici, Christophe Pison, Jean-François Cordier, Marcel Laurent, Ari Chaouat, Gilbert Habib, Eric Hachulla, Virginie Gressin, Emmanuel Weitzenblum, Alain Haloun, Michèle Bertocchi, Martine Reynaud-Gaubert |
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| Rok vydání: | 2006 |
| Předmět: |
Pulmonary and Respiratory Medicine
Adult Male medicine.medical_specialty Adolescent Hypertension Pulmonary Population Critical Care and Intensive Care Medicine Cohort Studies Age Distribution Intensive care Internal medicine medicine Prevalence Humans Registries education Associated Pulmonary Arterial Hypertension Aged Aged 80 and over education.field_of_study Portopulmonary hypertension Exercise Tolerance business.industry Incidence (epidemiology) Incidence Hemodynamics Middle Aged medicine.disease Pulmonary hypertension Surgery Survival Rate Portal hypertension Female France business Cohort study |
| Zdroj: | American journal of respiratory and critical care medicine. 173(9) |
| ISSN: | 1073-449X |
| Popis: | 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.To describe clinical and hemodynamic parameters and to provide estimates for the prevalence of patients diagnosed for PAH according to a standardized definition.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.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.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. |
| Databáze: | OpenAIRE |
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