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Relationship between maximal left ventricular wall thickness and sudden cardiac death in childhood onset hypertrophic cardiomyopathy

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Item Type:Article
Title:Relationship between maximal left ventricular wall thickness and sudden cardiac death in childhood onset hypertrophic cardiomyopathy
Creators Name:Norrish, G. and Ding, T. and Field, E. and Cervi, E. and Ziółkowska, L. and Olivotto, I. and Khraiche, D. and Limongelli, G. and Anastasakis, A. and Weintraub, R. and Biagini, E. and Ragni, L. and Prendiville, T. and Duignan, S. and McLeod, K. and Ilina, M. and Fernandez, A. and Marrone, C. and Bökenkamp, R. and Baban, A. and Kubus, P. and Daubeney, P.E.F. and Sarquella-Brugada, G. and Cesar, S. and Klaassen, S. and Ojala, T.H. and Bhole, V. and Medrano, C. and Uzun, O. and Brown, E. and Gran, F. and Sinagra, G. and Castro, F.J. and Stuart, G. and Vignati, G. and Yamazawa, H. and Barriales-Villa, R. and Garcia-Guereta, L. and Adwani, S. and Linter, K. and Bharucha, T. and Garcia-Pavia, P. and Siles, A. and Rasmussen, T.B. and Calcagnino, M. and Jones, C.B. and De Wilde, H. and Kubo, T. and Felice, T. and Popoiu, A. and Mogensen, J. and Mathur, S. and Centeno, F. and Reinhardt, Z. and Schouvey, S. and O'Mahony, C. and Omar, R.Z. and Elliott, P.M. and Kaski, J.P.
Abstract:BACKGROUND: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort. METHODS: The study cohort comprised 1075 children (mean age, 10.2 years [±4.4]) diagnosed with HCM (1-16 years) from the International Paediatric Hypertrophic Cardiomyopathy Consortium. Anonymized, noninvasive clinical data were collected from baseline evaluation and follow-up, and 5-year estimated SCD risk was calculated (HCM Risk-Kids). RESULTS: MLVWT Z score was <10 in 598 (58.1%), ≥10 to <20 in 334 (31.1%), and ≥20 in 143 (13.3%). Higher MLVWT Z scores were associated with heart failure symptoms, unexplained syncope, left ventricular outflow tract obstruction, left atrial dilatation, and nonsustained ventricular tachycardia. One hundred twenty-two patients (71.3%) with MLVWT Z score ≥20 had coexisting risk factors for SCD. Over a median follow-up of 4.9 years (interquartile range, 2.3-9.3), 115 (10.7%) had an SCD event. Freedom from SCD event at 5 years for those with MLVWT Z scores <10, ≥10 to <20, and ≥20 was 95.6%, 87.4%, and 86.0, respectively. The estimated SCD risk at 5 years had a nonlinear, inverted U-shaped relationship with MLVWT Z score, peaking at Z score +23. The presence of coexisting risk factors had a summative effect on risk. CONCLUSIONS: In children with HCM, an inverted U-shaped relationship exists between left ventricular hypertrophy and estimated SCD risk. The presence of additional risk factors has a summative effect on risk. While MLVWT is important for risk stratification, it should not be used either as a binary variable or in isolation to guide implantable cardioverter defibrillator implantation decisions in children with HCM.
Keywords:Adult, Child, Sudden Death, Humans, Hypertrophic Cardiomyopathy
Source:Circulation Arrhythmia and Electrophysiology
Publisher:American Heart Association
Page Range:e010075
Date:May 2022
Official Publication:https://doi.org/10.1161/CIRCEP.121.010075
PubMed:View item in PubMed

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