The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy
Por:
Norrish G, Topriceanu C, Qu C, Field E, Walsh H, Ziólkowska L, Olivotto I, Passantino S, Favilli S, Anastasakis A, Vlagkouli V, Weintraub R, King I, Biagini E, Ragni L, Prendiville T, Duignan S, McLeod K, Ilina M, Fernández A, Bökenkamp R, Baban A, Drago F, Kubuš P, Daubeney PEF, Chivers S, Sarquella-Brugada G, César-Díaz S, Marrone C, Medrano C, Alvarez Garcia-Roves R, Uzun O, Gran F, Castro FJ, Gimeno JR, Barriales-Villa R, Rueda F, Adwani S, Searle J, Bharucha T, Siles A, Usano A, Rasmussen TB, Jones CB, Kubo T, Mogensen J, Reinhardt Z, Cervi E, Elliott PM, Omar RZ and Kaski JP
Publicada:
30 mar 2022
Ahead of Print:
1 mar 2021
Resumen:
Aims 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. Methods and results 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% Conclusion 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.
Filiaciones:
Norrish G:
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK
Institute of Cardiovascular Sciences, University College London, London, UK
Topriceanu C:
Institute of Cardiovascular Sciences, University College London, London, UK
Qu C:
Department of Statistical Science, University College London, London, UK
Field E:
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK
Institute of Cardiovascular Sciences, University College London, London, UK
Walsh H:
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK
Ziólkowska L:
Department of Cardiology, The Children's Memorial Health Institute, Warsaw, Poland
Olivotto I:
Careggi University Hospital, Florence, Italy
Passantino S:
Cardiology Unit, A Meyer Pediatric Hospital, Florence, Italy
Favilli S:
Cardiology Unit, A Meyer Pediatric Hospital, Florence, Italy
Anastasakis A:
Onassis Cardiac Surgery Center, Athens, Greece
Vlagkouli V:
Onassis Cardiac Surgery Center, Athens, Greece
Weintraub R:
The Royal Children's Hospital, Melbourne, Australia
The Murdoch Children's Research Institute
University of Melbourne, Australia
King I:
The Murdoch Children's Research Institute
Biagini E:
S. Orsola-Malpighi Hospital, Bologna, Italy
Ragni L:
S. Orsola-Malpighi Hospital, Bologna, Italy
Prendiville T:
Our Lady's Children's Hospital, Dublin, Ireland
Duignan S:
Our Lady's Children's Hospital, Dublin, Ireland
McLeod K:
Royal Hospital for Children, Glasgow, UK
Ilina M:
Royal Hospital for Children, Glasgow, UK
Fernández A:
Favaloro Foundation University Hospital, Buenos Aires, Argentina
Bökenkamp R:
Leiden University Medical Center, Leiden, Netherlands
Baban A:
Bambino Gesu Hospital, Rome, Italy
Drago F:
Bambino Gesu Hospital, Rome, Italy
Kubuš P:
University Hospital Motol, Prague, Czech Republic
Daubeney PEF:
Royal Brompton and Harefield NHS Trust, London, UK
Chivers S:
Royal Brompton and Harefield NHS Trust, London, UK
Sarquella-Brugada G:
Arrhythmia and Inherited Cardiac Diseases Unit, Hospital Sant Joan de Déu, University of Barcelona, Spain
Medical Sciences Department, School of Medicine, University of Girona
César-Díaz S:
Arrhythmia and Inherited Cardiac Diseases Unit, Hospital Sant Joan de Déu, University of Barcelona, Spain
Marrone C:
Papa Giovanni XXIII hospital, Bergamo, Italy
Medrano C:
Hospital General Universitario Gregorio Marañón, Madrid, Spain
Alvarez Garcia-Roves R:
Hospital General Universitario Gregorio Marañón, Madrid, Spain
Uzun O:
University Hospital of Wales, Cardiff, UK
Gran F:
Val d'Hebron University Hospital, Barcelona, Spain
Castro FJ:
University Hospital Virgen de la Arrixaca, Murcia, Spain
Gimeno JR:
University Hospital Virgen de la Arrixaca, Murcia, Spain
Barriales-Villa R:
Complexo Hospitalario Universitario A Coruña, CIBERCV, A Coruña, Spain
Rueda F:
Complexo Hospitalario Universitario A Coruña, CIBERCV, A Coruña, Spain
Adwani S:
John Radcliffe Hospital, Oxford, UK
Searle J:
John Radcliffe Hospital, Oxford, UK
Bharucha T:
Southampton general Hospital, Southampton, UK
Siles A:
Hospital Universitario Puerta de Hierro Majadahonda, CIBERCV, Madrid, Spain
University Francisco de Vitoria, Pozuelo de Alarcon, Spain
Usano A:
Hospital Universitario Puerta de Hierro Majadahonda, CIBERCV, Madrid, Spain
University Francisco de Vitoria, Pozuelo de Alarcon, Spain
Rasmussen TB:
Department of cardiology, Aarhus University Hospital, Aarhus, Denmark
Jones CB:
Alder Hey Children's Hospital, Liverpool, UK
Kubo T:
Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Japan
Mogensen J:
Odense University Hospital, Odense, Denmark
Reinhardt Z:
The Freeman Hospital, Newcastle, UK
Cervi E:
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK
Institute of Cardiovascular Sciences, University College London, London, UK
Elliott PM:
Institute of Cardiovascular Sciences, University College London, London, UK
St Bartholomew's Centre for Inherited Cardiovascular Diseases, St Bartholomew's Hospital, West Smithfield, London, UK
Omar RZ:
Department of Statistical Science, University College London, London, UK
Kaski JP:
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK
Institute of Cardiovascular Sciences, University College London, London, UK
Green Submitted, hybrid
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