Abstract
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.
- © 2011 ERS
