Objective: Cardiac resynchronization therapy (CRT) is increasingly used in children in a variety of anatomic and pathophysiologic conditions, but published data are scarce. This study records current practice and results of CRT in paediatric and congenital heart disease.
Design: Retrospective multi-centre European survey.
Setting: Paediatric cardiology and cardiac surgery centres.
Patients: One hundred nine patients aged 0.24 – 73.8 (median 16.9) years with structural congenital heart disease (N=87), congenital AV block (N=12) and dilated cardiomyopathy (N=10) with systemic left (N=69), right (N=36) or single (N=4) ventricular dysfunction and ventricular dyssynchrony during sinus rhythm (N=25) or associated with pacing (N=84).
Interventions: CRT for a median period of 7.5 months (concurrent cardiac surgery in 16/109).
Main outcome measures: Functional improvement and echocardiographic change in systemic ventricular function.
Results: The z-score of the systemic ventricular end-diastolic dimension decreased by median 1.1 (P<0.001). Ejection fraction (EF) or fractional area of change increased by a mean of 11.5±14.3 % (P<0.001) and New York Heart Association (NYHA) class improved by median 1.0 grade (P<0.001). Non-response to CRT (18.5 %) was multivariably predicted by the presence of primary dilated cardiomyopathy (P=0.002) and poor NYHA class (P=0.003). Presence of a systemic left ventricle (LV) was the strongest multivariable predictor of improvement in EF/fractional area of change (P<0.001). Results were independent of number of patients treated in each contributing centre.
Conclusion: Heart failure associated with ventricular pacing is the largest indication for CRT in paediatric and congenital heart disease. CRT efficacy varies widely with the underlying anatomic and pathophysiologic substrate.
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