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)
Sí
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.
Estimación del efecto: 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.