In a national cohort of paediatric hypertrophic cardiomyopathy patients, appropriate ICD therapies occurred at a rate of 4.7 per 100 patient years, with no specific device or programming strategy associated with inappropriate therapies or complications.
Cohort (n=90)
Yes
Does implantable cardioverter-defibrillator (ICD) programming strategy affect the incidence of appropriate therapies, inappropriate therapies, and complications in pediatric patients with hypertrophic cardiomyopathy?
In pediatric patients with hypertrophic cardiomyopathy, ICDs provide life-saving therapy but are associated with significant complication rates, and no specific programming strategy was found to reduce inappropriate therapies or complications, suggesting simpler devices may be preferable.
Effect estimate: incidence rate 4.7 per 100 patient years (95% CI 2.9-7.6)
AIMS: Sudden cardiac death (SCD) is the most common mode of death in paediatric hypertrophic cardiomyopathy (HCM). This study describes the implant and programming strategies with clinical outcomes following implantable cardioverter-defibrillator (ICD) insertion in a well-characterized national paediatric HCM cohort. METHODS AND RESULTS: Data from 90 patients undergoing ICD insertion at a median age 13 (±3.5) for primary (n = 67, 74%) or secondary prevention (n = 23, 26%) were collected from a retrospective, longitudinal multi-centre cohort of children (<16 years) with HCM from the UK. Seventy-six (84%) had an endovascular system 14 (18%) dual coil, 3 (3%) epicardial, and 11 (12%) subcutaneous system. Defibrillation threshold (DFT) testing was performed at implant in 68 (76%). Inadequate DFT in four led to implant adjustment in three patients. Over a median follow-up of 54 months (interquartile range 28-111), 25 (28%) patients had 53 appropriate therapies ICD shock n = 45, anti-tachycardia pacing (ATP) n = 8, incidence rate 4.7 per 100 patient years (95% CI 2.9-7.6). Eight inappropriate therapies occurred in 7 (8%) patients (ICD shock n = 4, ATP n = 4), incidence rate 1.1/100 patient years (95% CI 0.4-2.5). Three patients (3%) died following arrhythmic events, despite a functioning device. Other device complications were seen in 28 patients (31%), including lead-related complications (n = 15) and infection (n = 10). No clinical, device, or programming characteristics predicted time to inappropriate therapy or lead complication. CONCLUSION: In a large national cohort of paediatric HCM patients with an ICD, device and programming strategies varied widely. No particular strategy was associated with inappropriate therapies, missed/delayed therapies, or lead complications.
Norrish et al. (Fri,) conducted a cohort in paediatric hypertrophic cardiomyopathy (n=90). Implantable cardioverter-defibrillator (ICD) was evaluated on Appropriate ICD therapies (incidence rate 4.7 per 100 patient years, 95% CI 2.9-7.6). In a national cohort of paediatric hypertrophic cardiomyopathy patients, appropriate ICD therapies occurred at a rate of 4.7 per 100 patient years, with no specific device or programming strategy associated with inappropriate therapies or complications.