PT  - JOURNAL ARTICLE
AU  - Jais, Xavier
AU  - Bonnet, Damien
AU  - Rottat, Laurence
AU  - Szezepanski, Isabelle
AU  - Lévy, Maryline
AU  - Gressin, Virginie
AU  - Humbert, Marc
AU  - Sitbon, Olivier
AU  - Simonneau, Gérald
TI  - Characteristics of patients with pulmonary arterial hypertension associated with congenital heart disease in the French PAH registry
DP  - 2011 Sep 01
TA  - European Respiratory Journal
PG  - p1527
VI  - 38
IP  - Suppl 55
4099  - http://erj.ersjournals.com/content/38/Suppl_55/p1527.short
4100  - http://erj.ersjournals.com/content/38/Suppl_55/p1527.full
SO  - Eur Respir J2011 Sep 01; 38
AB  - Background: Epidemiological data relative to pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) are scarce. In the first French PAH registry conducted in 2002-2003, CHD accounted for 11.3%.Objective: To analyze PAH associated with CHD in patients enrolled in the second prospective PAH registry initiated in 2006.Methods: PAH-related clinical and outcome data were reviewed and analyzed from the registry.Patients and results: 2585 patients with PAH were enrolled in 26 PAH centers. CHD-PAH (n = 255) accounted for 9.8%, including 95 isolated pre-tricuspid shunts (mainly ASD), 134 isolated post-tricuspid shunts (mainly VSD), 11 combined pre-and post-tricuspid shunts and 15 complex CHD. 60% of patients were females and mean age at diagnosis was 37 years. The diagnosis of PAH was done simultaneously with the diagnosis of CHD in 37% of the cases and in 60% PAH appeared during the follow-up of CHD. At study entry, 52% of patients were in NYHA functional class (FC) III or IV, 6MWD was 370±105 m and pulmonary hemodynamics were: mPAP= 59±20 mmHg, CI= 2,7±1.1 L/min/m2 and PVR= 12.5±10.3 WU. 47% of NYHA II and 43% of NYHA III patients were not receiving PAH-specific therapies. In treated patients (n = 164), NYHA FC improved (59% in NYHA FC I-II at last follow-up). During the 3-year follow-up period, 20 patients died and 7 patients were transplanted.Conclusions: PAH is a complication of a previously known CHD in 60% of cases. ASD is the main CHD that is diagnosed concomitantly or after PAH. Less than a half of NYHA III patients are offered PAH-specific therapies. Mortality was low during the short period of follow-up.