Pulmonary Arterial Hypertension in France

TLDR

Pulmonary arterial hypertension is an orphan disease typically managed in specialized multidisciplinary centers. The study aimed to describe clinical and hemodynamic characteristics of PAH patients and estimate their prevalence using a standardized definition. A national registry was established in 17 university hospitals, enrolling all consecutive adults (≥18 yr) diagnosed between October 2002 and October 2003, yielding 674 patients. The registry revealed that 75 % of patients were NYHA class III/IV, had a mean 6‑min walk of 329 m, mean pulmonary artery pressure of 55 mm Hg, a low prevalence of 15.0 cases per million adults and incidence of 2.4 cases per million adults per year, and a one‑year survival of 88 %.

Abstract

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.