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The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy.

Authors
  • Norrish, Gabrielle1, 2
  • Topriceanu, Cristian2
  • Qu, Chen3
  • Field, Ella1, 2
  • Walsh, Helen1
  • Ziółkowska, Lidia4
  • Olivotto, Iacopo5
  • Passantino, Silvia6
  • Favilli, Silvia6
  • Anastasakis, Aris7
  • Vlagkouli, Vasiliki7
  • Weintraub, Robert8, 9, 10
  • King, Ingrid9
  • Biagini, Elena11
  • Ragni, Luca11
  • Prendiville, Terrence12
  • Duignan, Sophie12
  • McLeod, Karen13
  • Ilina, Maria13
  • Fernández, Adrian14
  • And 31 more
  • 1 Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK.
  • 2 Institute of Cardiovascular Sciences, University College London, London, UK.
  • 3 Department of Statistical Science, University College London, London, UK.
  • 4 Department of Cardiology, The Children's Memorial Health Institute, Warsaw, Poland. , (Poland)
  • 5 Careggi University Hospital, Florence, Italy. , (Italy)
  • 6 Cardiology Unit, A Meyer Pediatric Hospital, Florence, Italy. , (Italy)
  • 7 Onassis Cardiac Surgery Center, Athens, Greece. , (Greece)
  • 8 The Royal Children's Hospital, Melbourne, Australia. , (Australia)
  • 9 The Murdoch Children's Research Institute.
  • 10 University of Melbourne, Australia. , (Australia)
  • 11 S. Orsola-Malpighi Hospital, Bologna, Italy. , (Italy)
  • 12 Our Lady's Children's Hospital, Dublin, Ireland. , (Ireland)
  • 13 Royal Hospital for Children, Glasgow, UK.
  • 14 Favaloro Foundation University Hospital, Buenos Aires, Argentina. , (Argentina)
  • 15 Leiden University Medical Center, Leiden, Netherlands. , (Netherlands)
  • 16 Bambino Gesu Hospital, Rome, Italy. , (Italy)
  • 17 University Hospital Motol, Prague, Czech Republic. , (Czechia)
  • 18 Royal Brompton and Harefield NHS Trust, London, UK.
  • 19 Arrhythmia and Inherited Cardiac Diseases Unit, Hospital Sant Joan de Déu, University of Barcelona, Spain. , (Spain)
  • 20 Medical Sciences Department, School of Medicine, University of Girona.
  • 21 Papa Giovanni XXIII hospital, Bergamo, Italy. , (Italy)
  • 22 Hospital General Universitario Gregorio Marañón, Madrid, Spain. , (Spain)
  • 23 University Hospital of Wales, Cardiff, UK.
  • 24 Val d'Hebron University Hospital, Barcelona, Spain. , (Spain)
  • 25 University Hospital Virgen de la Arrixaca, Murcia, Spain. , (Spain)
  • 26 Complexo Hospitalario Universitario A Coruña, CIBERCV, A Coruña, Spain. , (Spain)
  • 27 John Radcliffe Hospital, Oxford, UK.
  • 28 Southampton general Hospital, Southampton, UK.
  • 29 Hospital Universitario Puerta de Hierro Majadahonda, CIBERCV, Madrid, Spain. , (Spain)
  • 30 University Francisco de Vitoria, Pozuelo de Alarcon, Spain. , (Spain)
  • 31 Department of cardiology, Aarhus University Hospital, Aarhus, Denmark. , (Denmark)
  • 32 Alder Hey Children's hospital, Liverpool, UK.
  • 33 Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Japan. , (Japan)
  • 34 Odense University Hospital, Odense, Denmark. , (Denmark)
  • 35 The Freeman Hospital, Newcastle, UK.
  • 36 St Bartholomew's Centre for Inherited Cardiovascular Diseases, St Bartholomew's Hospital, West Smithfield, London, UK.
Type
Published Article
Journal
European Journal of Preventive Cardiology
Publisher
SAGE Publications
Publication Date
Mar 30, 2022
Volume
29
Issue
4
Pages
645–653
Identifiers
DOI: 10.1093/eurjpc/zwab046
PMID: 33772274
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

The 12-lead electrocardiogram (ECG) is routinely performed in children with hypertrophic cardiomyopathy (HCM). An ECG risk score has been suggested as a useful tool for risk stratification, but this has not been independently validated. This aim of this study was to describe the ECG phenotype of childhood HCM in a large, international, multi-centre cohort and investigate its role in risk prediction for arrhythmic events. Data from 356 childhood HCM patients with a mean age of 10.1 years (±4.5) were collected from a retrospective, multi-centre international cohort. Three hundred and forty-seven (97.5%) patients had ECG abnormalities at baseline, most commonly repolarization abnormalities (n = 277, 77.8%); left ventricular hypertrophy (n = 240, 67.7%); abnormal QRS axis (n = 126, 35.4%); or QT prolongation (n = 131, 36.8%). Over a median follow-up of 3.9 years (interquartile range 2.0-7.7), 25 (7%) had an arrhythmic event, with an overall annual event rate of 1.38 (95% CI 0.93-2.04). No ECG variables were associated with 5-year arrhythmic event on univariable or multivariable analysis. The ECG risk score threshold of >5 had modest discriminatory ability [C-index 0.60 (95% CI 0.484-0.715)], with corresponding negative and positive predictive values of 96.7% and 6.7. In a large, international, multi-centre cohort of childhood HCM, ECG abnormalities were common and varied. No ECG characteristic, either in isolation or combined in the previously described ECG risk score, was associated with 5-year sudden cardiac death risk. This suggests that the role of baseline ECG phenotype in improving risk stratification in childhood HCM is limited. © The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.

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