PT  - JOURNAL ARTICLE
AU  - Olivier Sitbon
AU  - Azzedine Yaïci
AU  - Laurence Rottat
AU  - Vincent Cottin
AU  - Emmanuel Bergot
AU  - Grégoire Prévot
AU  - Virginie Gressin
AU  - Marc Humbert
AU  - Gérald Simonneau
TI  - The changing picture of patients with pulmonary arterial hypertension (PAH) in France
DP  - 2011 Sep 01
TA  - European Respiratory Journal
PG  - 4913
VI  - 38
IP  - Suppl 55
4099  - http://erj.ersjournals.com/content/38/Suppl_55/4913.short
4100  - http://erj.ersjournals.com/content/38/Suppl_55/4913.full
SO  - Eur Respir J2011 Sep 01; 38
AB  - The PH Registry provides current demographics of patients (pts) with PAH in France. We describe clinical characteristics, haemodynamics and survival of pts newly diagnosed with PAH between Nov 06 and Oct 09.Methods: Clinical and haemodynamic characteristics of all consecutive newly diagnosed pts (i.e. incident cases) belonging to groups 1 (PAH) and 1' (pulmonary veno-occlusive disease [PVOD]) were collected at the time of diagnosis. Survival was analysed from PAH diagnosis to a cut-off date of 1 Nov 10.Results: A total of 751 pts were analysed. F/M sex-ratio was 1.26, mean (±SD) age was 56±15 yrs (18–86) and 24% of pts were > 70 yr. Idiopathic (I), heritable (H), anorexigen (An), connective tissue disease, congenital heart disease, portal hypertension, HIV and chronic haemolytic anaemia-associated PAH accounted for 38.3%, 2.4%, 4.8%, 19.6%, 4.7%, 16.6%, 4% and 0.7% of the population, respectively. Thirty five pts (4.7%) had 2 risk factors for PAH. PVOD was diagnosed in 4.3% of pts. At diagnosis, 74% of pts were in NYHA class III or IV. Mean 6-min walk was 335±123 m. Mean PAP, cardiac index and PVR were 47±12 mmHg, 2.6±0.9 L/min/m2 and 741±413 dyn.s.cm-5, respectively. PAH-specific therapies were initiated in 620 pts (82.6%). One-, 2- and 3-year survival estimates were 88.5%, 78.2% and 69.2% in the overall population, and 88.3%, 77.3% and 67.7% in the subgroup (n=350) with IPAH, HPAH or An-PAH, respectively.Conclusions: PAH is still diagnosed late in the course of the disease. Furthermore, pts are older and the proportion of males is higher than that observed in other registries. Finally, there is a trend for an improved survival in pts with IPAH, HPAH or An-PAH compared to our previous findings.