Clinical outcomes and programming strategies of implantable cardioverter-defibrillator devices in paediatric hypertrophic cardiomyopathy: a UK National Cohort Study.

Europace : European Pacing, Arrhythmias, and Cardiac Electrophysiology : Journal of the Working Groups on Cardiac Pacing, Arrhythmias, and Cardiac Cellular Electrophysiology of the European Society of Cardiology
Gabrielle NorrishJuan Pablo Kaski

Abstract

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. 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 arrhythm...Continue Reading

References

Jun 27, 2006·Europace : European Pacing, Arrhythmias, and Cardiac Electrophysiology : Journal of the Working Groups on Cardiac Pacing, Arrhythmias, and Cardiac Cellular Electrophysiology of the European Society of Cardiology·Massimo Stefano SilvettiLucilla Ravà
May 18, 2012·Heart·Jeffrey P Moak, Juan Pablo Kaski
Mar 21, 2013·Pediatric Cardiology·Anna N KampDavid J Bradley
Jan 27, 2015·Heart Rhythm : the Official Journal of the Heart Rhythm Society·Jason M GarnreiterElizabeth V Saarel
Apr 28, 2016·Pacing and Clinical Electrophysiology : PACE·Brynn E DechertMartin J Lapage
Nov 20, 2016·Journal of Cardiovascular Electrophysiology·Pietro FranciaCamillo Autore
Feb 18, 2017·Journal of the American College of Cardiology·Ethan J RowinMartin S Maron
Mar 2, 2018·Circulation·Peta M A AlexanderUNKNOWN National Australian Childhood Cardiomyopathy Study
Apr 20, 2020·The American Journal of Cardiology·Kartik R KumarMark S Link

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