LONG-TERM FATE OF PATIENTS WITH CONGENITAL HEART DISEASE UNDERGOING CARDIAC RESYNCHRONIZATION THERAPY

Aim: To evaluate long-term impact of CRT in pts with congenital heart disease (CHD).
Patients and methods: 33 consecutive pts with structural CHD (N=32) or congenital AV block (N=1) aged median 13 (IQR 8-23) yrs were followed-up for median 50 (IQR 38-72) mos after primary (N=9) CRT-P (N=29) or CRT-P/D (N=4) implantation or upgrade from conventional pacing (N=24). CRT was applied for treatment of dyssynchronous heart failure (HF,N=29) or to prevent systemic ventricular desynchronization after pacemaker implantation (N=4). Response was defined as improvement of systemic ventricular EF or fractional area of change (FAC) by >10 points and improved or unchanged NYHA class at end of follow-up.
Results: Six adverse outcomes (18.2 %) yielded an actuarial probability of uneventful therapy continuation at 5 yrs of 77.0 %: 1 HF related and 1 sudden cardiac death, 1 heart transplant, 1 pat suffering from neurological damage due to exit block and asystole and 2 therapy terminations (lead fracture and infection, resp.). Among 29 pts with dyssynchronous HF (initial median EF or FAC=23, IQR 16-31 %, mean NYHA=2.3) CRT response was observed in 8/12 with systemic LV and 5/17 with systemic RV or single ventricle, P=0.07. Decrease in NYHA class and in the z-score of systemic ventricular end-diastolic dimension was larger in systemic LV (P=0.038 and 0.006, resp.). 8 surgical revisions had to be performed in 7 pts (generator replacement for ERI in 6/8) with a reintervention-free survival probability at 5 yrs of 81.6 %.
Conclusions: Long-term CRT led to sustained improvement of systolic function in ~2/3 of pts with systemic LV and ~1/3 of pts with systemic RV or single ventricle. It was associated with significant incidence of heart failure-related adverse outcomes and device complications, resp.. (Supported by the research project of University Hospital Motol MZOFNM2005)