Aim: To evaluate long-term impact of cardiac resynchronization therapy (CRT) in pts with congenital heart disease (CHD).
Methods: Thirty consecutive pts with CHD (N=28) or congenital atrioventricular block (N=2) aged median 12.9 (IQR 6.5-18.2) years at CRT-P implantation were followed for median 9.0 (IQR 4.5-11.4) years on CRT. CRT was performed for systemic left ventricular (LV, N=12) and right (RV) or single (SV) ventricular (N=18) failure and was associated with additional cardiac surgery in 13 pts. CRT response was defined as an increase in systemic ventricular ejection fraction or fractional area of change (FAC) by >10 points and improved/unchanged NYHA class at the end of follow-up. Actuarial survival probability was calculated for 5 and 10 years after CRT implantation.
Results: Freedom from cardiovascular death/heart failure hospitalization was 92.0 and 82.3%, resp.. No patient underwent heart transplant. Freedom from complications leading to surgical revision (elective generator replacement excluded) or therapy termination was 81.4 and 70.1% at 5 and 10 years, resp. Overall probability of an uneventful therapy continuation was 74.6 and 56.3%, resp. Upgrade to CRT-D was performed in 1 patient. Ejection fraction or FAC changed from median 29.5% (IQR 22.3-35.0) before CRT to median 40.3% (IQR 32.0-54.0; p<0.001) at the end of follow-up. NYHA class improved from median 2.0 to 1.5 (p=0.002). CRT response tended to be more frequent in systemic LV (9/12 pts) than RV or SV (6/18 pts, p=0.060).
Conclusion: Long-term CRT in patients with CHD is associated with acceptable survival and sustained improvement of systemic ventricular function in 50 % of patients. Probability of device complications necessitating surgical revision or therapy termination is however high.(Supported by MH CZ - DRO, University Hospital Motol, Prague, Czech Republic 00064203)
