TY - JOUR
T1 - Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy
AU - Norrish, Gabrielle
AU - Ding, Tao
AU - Field, Ella
AU - Cervi, Elena
AU - Ziółkowska, Lidia
AU - Olivotto, Iacopo
AU - Khraiche, Diala
AU - Limongelli, Giuseppe
AU - Anastasakis, Aris
AU - Weintraub, Robert
AU - Biagini, Elena
AU - Ragni, Luca
AU - Prendiville, Terrence
AU - Duignan, Sophie
AU - McLeod, Karen
AU - Ilina, Maria
AU - Fernández, Adrián
AU - Marrone, Chiara
AU - Bökenkamp, Regina
AU - Baban, Anwar
AU - Kubus, Peter
AU - Daubeney, Piers E F
AU - Sarquella-Brugada, Georgia
AU - Cesar, Sergi
AU - Klaassen, Sabine
AU - Ojala, Tiina H
AU - Bhole, Vinay
AU - Medrano, Constancio
AU - Uzun, Orhan
AU - Brown, Elspeth
AU - Gran, Ferran
AU - Sinagra, Gianfranco
AU - Castro, Francisco J
AU - Stuart, Graham
AU - Vignati, Gabriele
AU - Yamazawa, Hirokuni
AU - Barriales-Villa, Roberto
AU - Garcia-Guereta, Luis
AU - Adwani, Satish
AU - Linter, Katie
AU - Bharucha, Tara
AU - Garcia-Pavia, Pablo
AU - Siles, Ana
AU - Rasmussen, Torsten B
AU - Calcagnino, Margherita
AU - Jones, Caroline B
AU - De Wilde, Hans
AU - Kubo, Toru
AU - Felice, Tiziana
AU - Popoiu, Anca
AU - Mogensen, Jens
AU - Mathur, Sujeev
AU - Centeno, Fernando
AU - Reinhardt, Zdenka
AU - Schouvey, Sylvie
AU - O'Mahony, Costas
AU - Omar, Rumana Z
AU - Elliott, Perry M
AU - Kaski, Juan Pablo
PY - 2022/5
Y1 - 2022/5
N2 - 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.
AB - 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.
KW - Adult
KW - Cardiomyopathy, Hypertrophic/complications
KW - Child
KW - Death, Sudden, Cardiac/epidemiology
KW - Defibrillators, Implantable/adverse effects
KW - Heart Ventricles/diagnostic imaging
KW - Humans
KW - Hypertrophy, Left Ventricular/complications
KW - Retrospective Studies
KW - Risk Assessment
KW - Risk Factors
UR - http://www.scopus.com/inward/record.url?scp=85130641990&partnerID=8YFLogxK
U2 - 10.1161/CIRCEP.121.010075
DO - 10.1161/CIRCEP.121.010075
M3 - Journal article
C2 - 35491873
SN - 1941-3149
VL - 15
SP - e010075
JO - Circulation. Arrhythmia and Electrophysiology
JF - Circulation. Arrhythmia and Electrophysiology
IS - 5
M1 - e010075
ER -