| Title: |
Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy |
| Authors: |
Norrish, Gabrielle; Ding, Tao; Field, Ella; Cervi, Elena; Ziolkowska, Lidia; Olivotto, Iacopo; Khraiche, Diala; Limongelli, Giuseppe; Anastasakis, Aris; Weintraub, Robert; Biagini, Elena; Ragni, Luca; Prendiville, Terrence; Duignan, Sophie; McLeod, Karen; Ilina, Maria; Fernandez, Adrian; Marrone, Chiara; Bokenkamp, Regina; Baban, Anwar; Kubus, Peter; Daubeney, Piers E. F.; Sarquella-Brugada, Georgia; Cesar, Sergi; Klaassen, Sabine; Ojala, Tiina H.; Bhole, Vinay; Medrano, Constancio; Uzun, Orhan; Brown, Elspeth; Gran, Ferran; Sinagra, Gianfranco; Castro, Francisco J.; Stuart, Graham; Vignati, Gabriele; Yamazawa, Hirokuni; Barriales-Villa, Roberto; Garcia-Guereta, Luis; Adwani, Satish; Linter, Katie; Bharucha, Tara; Garcia-Pavia, Pablo; Siles, Ana; Rasmussen, Torsten B.; Calcagnino, Margherita; Jones, Caroline B.; De Wilde, Hans; Kubo, Toru; Felice, Tiziana; Popoiu, Anca; Mogensen, Jens; Mathur, Sujeev; Centeno, Fernando; Reinhardt, Zdenka; Schouvey, Sylvie; O'Mahony, Costas; Omar, Rumana Z.; Elliott, Perry M.; Kaski, Juan Pablo |
| Contributors: |
University of Helsinki; Clinicum; Children's Hospital; HUS Children and Adolescents |
| Publisher Information: |
Lippincott williams & wilkins |
| Publication Year: |
2022 |
| Collection: |
Helsingfors Universitet: HELDA – Helsingin yliopiston digitaalinen arkisto |
| Subject Terms: |
adult; child; death; sudden; humans; hypertrophic cardiomyopathy; LONG-TERM OUTCOMES; RISK-FACTORS; TASK-FORCE; CHILDREN; DIAGNOSIS; IDENTIFICATION; ADOLESCENTS; SURVIVAL; FEATURES; General medicine; internal medicine and other clinical medicine; Gynaecology and paediatrics |
| Description: |
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 to = 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 to = 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. ; Peer reviewed |
| Document Type: |
article in journal/newspaper |
| File Description: |
application/pdf |
| Language: |
English |
| Relation: |
This work was supported by the British Heart Foundation (grant number FS/16/72/32270) to Drs Norrish and Kaski. E. Field and Dr Kaski are supported by Max's Foundation and Great Ormond Street Hospital Children's Charity. Dr Kaski is supported by a Medical Research Council Clinical-National Institute for Health Research (NIHR) Clinical Academic Research Partnership award. This work is (partly) funded by the NIHR Great Ormond Street Hospital Biomedical Research Centre. Dr Omar and T.D. work at University College London Hospitals/University College London that received a proportion of funding from the UK Department of Health National Institute for Health Research Biomedical Research Centres funding scheme. This work was financially supported by the Foundation for Paediatric Research, Finland (Dr Ojala). Dr Fernandez has received speaker's fees from Sanofi-Genzyme.; https://hdl.handle.net/10138/344405; 000796080400004 |
| Availability: |
https://hdl.handle.net/10138/344405 |
| Rights: |
cc_by ; info:eu-repo/semantics/openAccess ; openAccess |
| Accession Number: |
edsbas.E1FDB2C7 |
| Database: |
BASE |