RT Journal Article
SR Electronic
T1 Characteristics of patients with pulmonary arterial hypertension associated with congenital heart disease in the French PAH registry
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP p1527
VO 38
IS Suppl 55
A1 Jais, Xavier
A1 Bonnet, Damien
A1 Rottat, Laurence
A1 Szezepanski, Isabelle
A1 Lévy, Maryline
A1 Gressin, Virginie
A1 Humbert, Marc
A1 Sitbon, Olivier
A1 Simonneau, Gérald
YR 2011
UL http://erj.ersjournals.com/content/38/Suppl_55/p1527.abstract
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.