Abstract 2841: Efficacy of Implantable Cardioverter Defibrillator in Children With Dilated Cardiomyopathy
Background: Sudden cardiac death (SCD) is a well-known risk in patients with dilated cardiomyopathy (DCM). The use and efficacy of implantable cardioverter defibrillator (ICD) in pediatrics has not been well defined. We hypothesize that ICD may prevent SCD in children with DCM.
Methods: Medical records of 99 children with DCM were reviewed to determine arrhythmia incidence, left ventricular systolic function (LVEF), left ventricular diastolic diameter indexed by BSA (LVDDz), and outcome.
Results: The mean age of all patients was 6.8 +/− 6.5 years. All patients were on standard treatment for heart failure including 81% on beta-blockers. Mean follow up was 4.7 +/− 4/6 years. Arrhythmias were detected in 32 patients (38%): ventricular tachycardia (VT) in 27 and supraventricular tachycardia in five. ICD was placed in 20 patients (20%). Patients requiring ICD had lower LVEF at presentation: 18.3 +/− 35.4% versus 36.6 +/− 13.6% for non-ICD group (p=0.027). From presentation to last follow up, only patients without ICD showed significant improvement in LVEF from 35.4 +/− 12.8% to 51.1 +/− 13.0% (p<0.0001) and LVDDz from 3.8 +/− 3.2 to 1.99 +/− 2.9 (p=0.0003). In patients with ICD, LVEF improved but not significantly. For the ICD group, over a mean follow up of 7.1 +/− 5.4 years, there were 37 appropriate shocks in 8 patients (0.26 shocks/patient/year) and 5 inappropriate shocks in 4 patients (0.04 shocks/patient/year). In the ICD group there were 11 transplants and one death (non-SCD). In the non-ICD group there were 23 transplants and nine deaths. Three patients without ICD had SCD; two of these patients had history of atrial fibrillation.
Conclusions: VT is the most common arrhythmia in children with DCM. ICD can effectively rescue patients from VT; the number of inappropriate shocks is minimal. Use of ICD may be considered in patients with DCM and atrial fibrillation. Patients requiring ICD have lower LVEF at presentation and show no significant improvement at follow up, which my be reflective of more severe disease.