Cardiac Resynchronization Therapy in Children and Adults with Congenital Heart Disease

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US Cardiology, 2006;3(2):1-3

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Cardiac resynchronization therapy (CRT) using biventricular pacing has been proven to be effective in adult patients with left ventricular (LV) systolic dysfunction and QRS prolongation. In this group of patients, CRT improves exercise tolerance, symptoms of heart failure and all-cause mortality.1 In addition, there is growing evidence that inter- and intra-ventricular dyssynchrony induced by conventional right ventricular (RV) apical pacing has deleterious effects on LV function.2-4

CRT in children and patients with congenital heart disease (CHD) could be a very useful therapy for heart failure, but data to support this are still limited.To date, CRT studies in children have all been performed retrospectively and are mostly case reports and small case series.5-11 Only one large study was reported by Dubin et al. retrospectively evaluating the outcome of 103 CRT patients from 22 different institutions.12 Randomized clinical trials are still lacking because the group of patients is relatively small and heterogeneous. The diversity of the CHD population makes it difficult to automatically extrapolate the results from the large randomized CRT trials in adults. For example, in CHD, right bundle branch block and RV failure is a more common problem than LV failure and left bundle branch block (LBBB). Furthermore, the CHD population also includes patients with failing systemic right ventricles and different types of univentricular hearts. These different issues of CRT in CHD will be reviewed.

LV Dysfunction and CRT

The child or CHD patient with LV dysfunction and QRS prolongation appears to bear a resemblance with the typical adult CRT patient. This group consists of CHD patients with LV failure after congenital heart surgery, children with cardiomyopathies, and dilated cardiomyopathy (DCM) following conventional RV pacing. The first two pediatric case reports demonstrated the longer-term benefits of CRT in CHD children with LV failure. These reports describe the outcome of epicardial CRT in an infant with a left sided obstructive lesion and endocardial CRT in a six-year-old boy with multiple ventricular septal defects (VSDs) and mitral valve replacement. In both children symptoms of heart failure improved and LV ejection fractions (EFs) increased.

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References
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