RT Journal Article
SR Electronic
T1 The changing picture of patients with pulmonary arterial hypertension (PAH) in France
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 4913
VO 38
IS Suppl 55
A1 Olivier Sitbon
A1 Azzedine Yaïci
A1 Laurence Rottat
A1 Vincent Cottin
A1 Emmanuel Bergot
A1 Grégoire Prévot
A1 Virginie Gressin
A1 Marc Humbert
A1 Gérald Simonneau
YR 2011
UL http://erj.ersjournals.com/content/38/Suppl_55/4913.abstract
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.