TY - JOUR
T1 - Risk factors for lethal arrhythmic events in children and adolescents with hypertrophic cardiomyopathy and an implantable defibrillator
T2 - An international multicenter study
AU - Balaji, Seshadri
AU - DiLorenzo, Michael P.
AU - Fish, Frank A.
AU - Etheridge, Susan P.
AU - Aziz, Peter F.
AU - Russell, Mark W.
AU - Tisma, Svjetlana
AU - Pflaumer, Andreas
AU - Sreeram, Narayanswami
AU - Kubus, Peter
AU - Law, Ian H.
AU - Kantoch, Michal J.
AU - Kertesz, Naomi J.
AU - Strieper, Margaret
AU - Erickson, Christopher C.
AU - Moore, Jeremy P.
AU - Nakano, Stephanie J.
AU - Singh, Harinder R.
AU - Chang, Philip
AU - Cohen, Mitchell
AU - Fournier, Anne
AU - Ilina, Maria V.
AU - Smith, Richard T.
AU - Zimmerman, Frank
AU - Horndasch, Michaela
AU - Li, Walter
AU - Batra, Anjan
AU - Liberman, Leonardo
AU - Hamilton, Robert
AU - Janson, Christopher M.
AU - Sanatani, Shubhayan
AU - Zeltser, Ilana
AU - McDaniel, George
AU - Blaufox, Andrew D.
AU - Garnreiter, Jason M.
AU - Katcoff, Hannah
AU - Shah, Maully
N1 - Funding Information:
This study was supported by Medtronic External Research Protocol number CR-1651.Dr Balaji has received consultancy fee from MyoKardia and serves on the advisory board of yoR labs. Dr Kubus was supported by the Ministry of Health, Czech Republic. Dr Erickson has received consultancy fee from Medtronic. Dr Moore has received research grants from Biotronik. The rest of the authors report no conflicts of interest.
Publisher Copyright:
© 2019 Heart Rhythm Society
PY - 2019/10
Y1 - 2019/10
N2 - Background: Predictors of risk of lethal arrhythmic events (LAE) is poorly understood and may differ from adults in children with hypertrophic cardiomyopathy (HCM). Objective: The purpose of this study was to determine predictors of LAE in children with HCM. Methods: A retrospective data collection was performed on 446 children and teenagers 20 years and younger (290 [65%] male; mean age 10.1 ± 5.7 years) with idiopathic HCM from 35 centers. Patients were classified as group 1 (HCM with LAE) if having a secondary prevention implantable cardioverter-defibrillator (ICD) or primary prevention ICD with appropriate interventions or group 2 (HCM without LAE) if having a primary prevention ICD without appropriate interventions. Results: There were 152 children (34%) in group 1 and 294 (66%) in group 2. Risk factors for group 1 by univariate analysis were septal thickness, posterior left ventricular (LV) wall thickness, lower LV outflow gradient, and Q wave > 3 mm in inferior electrocardiographic leads. Factors not associated with LAE were family history of SCD, abnormal blood pressure response to exercise, and ventricular tachycardia on ambulatory electrocardiographic monitoring. Risk factors for SCD by multivariate analysis were age at ICD placement (hazard ratio [HR] 0.9; P =.0025), LV posterior wall thickness z score (HR 1.02; P <.005), and LV outflow gradient < 30 mm Hg (HR 2.0; P <.006). LV posterior wall thickness z score ≥ 5 was associated with LAE. Conclusion: Risk factors for LAE appear different in children compared to adults. Conventional adult risk factors were not significant in children. Further prospective studies are needed to improve risk stratification for LAE in children with HCM.
AB - Background: Predictors of risk of lethal arrhythmic events (LAE) is poorly understood and may differ from adults in children with hypertrophic cardiomyopathy (HCM). Objective: The purpose of this study was to determine predictors of LAE in children with HCM. Methods: A retrospective data collection was performed on 446 children and teenagers 20 years and younger (290 [65%] male; mean age 10.1 ± 5.7 years) with idiopathic HCM from 35 centers. Patients were classified as group 1 (HCM with LAE) if having a secondary prevention implantable cardioverter-defibrillator (ICD) or primary prevention ICD with appropriate interventions or group 2 (HCM without LAE) if having a primary prevention ICD without appropriate interventions. Results: There were 152 children (34%) in group 1 and 294 (66%) in group 2. Risk factors for group 1 by univariate analysis were septal thickness, posterior left ventricular (LV) wall thickness, lower LV outflow gradient, and Q wave > 3 mm in inferior electrocardiographic leads. Factors not associated with LAE were family history of SCD, abnormal blood pressure response to exercise, and ventricular tachycardia on ambulatory electrocardiographic monitoring. Risk factors for SCD by multivariate analysis were age at ICD placement (hazard ratio [HR] 0.9; P =.0025), LV posterior wall thickness z score (HR 1.02; P <.005), and LV outflow gradient < 30 mm Hg (HR 2.0; P <.006). LV posterior wall thickness z score ≥ 5 was associated with LAE. Conclusion: Risk factors for LAE appear different in children compared to adults. Conventional adult risk factors were not significant in children. Further prospective studies are needed to improve risk stratification for LAE in children with HCM.
KW - Arrhythmia
KW - Children
KW - Defibrillator
KW - Hypertrophic cardiomyopathy
KW - Sudden cardiac death
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U2 - 10.1016/j.hrthm.2019.04.040
DO - 10.1016/j.hrthm.2019.04.040
M3 - Article
C2 - 31026510
AN - SCOPUS:85071009550
SN - 1547-5271
VL - 16
SP - 1462
EP - 1467
JO - Heart Rhythm
JF - Heart Rhythm
IS - 10
ER -