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1 The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy
  1. Constantin-Cristian Topriceanu1,
  2. Gabrielle Norrish2,
  3. Chen Qu3,
  4. Ella Fiend2,
  5. Helen Walsh2,
  6. Lidia Ziółkowska4,
  7. Iacopo Olivotto5,
  8. Silvia Passantino6,
  9. Silvia Favilli6,
  10. Aris Anastasakis7,
  11. Vasiliki Vlagkouli7,
  12. Robert Weintraub8,
  13. Ingrid King9,
  14. Elena Biagini10,
  15. Lucaa Ragni10,
  16. Terence Prendiville11,
  17. Sophie Duignan11,
  18. Karen McLeod12,
  19. Maria Ilina12,
  20. Adrian Fernández13,
  21. Regina Bökenkamp14,
  22. Anwar Baban15,
  23. Drago Drago15,
  24. Peter Kubuš16,
  25. Piers Daubeney17,
  26. Sian Chivers17,
  27. Georgia Sarquella-Brugada18,
  28. Sergi Cesar18,
  29. Chiara Marrone19,
  30. Constancio Medrano20,
  31. Alvarez Garcia-Roves Reyes20,
  32. Orhan Uzun21,
  33. Ferran Gran Ipina22,
  34. FJ Castro Garcia23,
  35. Juan Ramón Gimeno23,
  36. Roberto Barriales-Villa24,
  37. Fernando Rueda24,
  38. Satish Adwani25,
  39. Jonathan Searle25,
  40. Tara Bharucha26,
  41. Ana Siles27,
  42. Ana Usano27,
  43. Torsten Bloch Rasmussen28,
  44. Caroline Jones29,
  45. T Kubo30,
  46. Jens Mogensen31,
  47. Zdenka Reinhardt32,
  48. Elena Cervi2,
  49. Perry Elliott33,
  50. Rumana Omar3,
  51. Juan Kaski2
  1. 1Institute of Cardiovascular Science, University College London, UK
  2. 2Centre for Inherited Cardiovascular diseases, Great Ormond Street Hospital, London, UK
  3. 3Department of Statistical science, University College London
  4. 4Department of Cardiology, The Children’s Memorial Health Institute, Warsaw, Poland
  5. 5Careggi University Hospital, Florence, Italy
  6. 6Cardiology Unit, A Meyer Pediatric Hospital, Florence, Italy
  7. 7Onassis Cardiac surgery center, Athens, Greece
  8. 8The Royal Children’s Hospital, Melbourne, Australia
  9. 9The Murdoch Children’s Research Institute
  10. 10S. Orsola-Malpighi Hospital, Bologna, Italy
  11. 11Our Lady’s Children’s Hospital, Dublin, Ireland
  12. 12Royal Hospital for Children, Glasgow
  13. 13Favaloro Foundation University Hospital, Buenos Aires, Argentina
  14. 14Leiden University Medical Center, Leiden, Netherlands
  15. 15Bambino Gesu Hospital, Rome, Italy
  16. 16University Hospital Motol, Prague, Czech Republic
  17. 17Royal Brompton and Harefield NHS Trust, London, UK
  18. 18Arrhythmia and Inherited Cardiac Diseases Unit, Hospital Sant Joan de Déu, University of Barcelona
  19. 19Papa Giovanni XXIII hospital, Bergamo, Italy
  20. 20Hospital General Universitario Gregorio Marañón, Madrid, Spain
  21. 21University Hospital of Wales, Cardiff, UK
  22. 22Val d’Hebron University Hospital, Barcelona, Spain
  23. 23University Hospital Virgen de la Arrixaca, Murcia, Spain
  24. 24Complexo Hospitalario Universitario A Coruña, CIBERCV. A Coruña, Spain
  25. 25John Radcliffe Hospital, Oxford, UK
  26. 26Southampton general Hospital, Southampton, UK
  27. 27Hospital Universitario Puerta de Hierro Majadahonda, CIBERCV Madrid, Spain
  28. 28Department of cardiology, Aarhus University Hospital, Aarhus, Denmark
  29. 29Alder Hey Children’s hospital, Liverpool, UK
  30. 30Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Japan
  31. 31Odense University Hospital, Odense, Denmark
  32. 32The Freeman Hospital, Newcastle, UK
  33. 33St Bartholomew’s Centre for Inherited Cardiovascular Diseases, St Bartholomew’s Hospital, West Smith


Introduction Sudden cardiac death is the most common cause of mortality in childhood onset hypertrophic cardiomyopathy. Identifying individuals at highest risk is therefore an essential part of clinical care but remains challenging. The 12 lead electrocardiogram (ECG) has been proposed as a useful tool for risk stratification and an ECG risk score has been proposed. However, this has not been independently validated and the ECG phenotype of childhood HCM has not been previously described. The 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 Participants with an available baseline resting 12-lead ECG were identified from a large, international, multi-centre, retrospective cohort of patients aged less than 16 years fulfilling the diagnostic criteria for HCM (n=1029). Resting baseline ECG was evaluated and ECG variables were extracted. In addition, the ECG risk score based on 8 parameters (deviation in QRS axis, pathological T-wave inversion in limb or precordial leads, ST-segment depression, dominant S-wave in V4, limb-lead amplitude sum, 12-lead amplitude duration product and QTc) was calculated as previously described. The primary study endpoint was a composite outcome of major cardiac events (MACE) defined as SCD, resuscitated cardiac arrest, appropriate implantable cardioverter defibrillator therapy, or sustained ventricular tachycardia (VT) with haemodynamic compromise. The discriminatory performance of using an ECG risk score >5 to identify patients at increased risk of MACE at 5 years was determined using Harrell’s C-index.

Results Of 356 patients with childhood HCM (68.9% male, mean age at presentation 10.1 ± 4.5 years), 347 (97.5%) had baseline ECG abnormalities such as: repolarization abnormalities (n=277, 77.8%), left ventricular hypertrophy (n=240. 67.6%), abnormal QRS axis (n=126, 35.4%) or QT prolongation (n=131, 36.8%). Over a median follow up of 3.9 years (IQR 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 MACE on univariable or multivariable Cox regression analysis. Of the 164 participants with an ECG score >5, 153 (93.3%) did not have a MACE within 5 years. Harrell’s C-index (the probability of correctly distinguishing between high and low risk patients using an ECG risk score threshold of >5) was 0.60 (95% CI 0.484-0.715) at 5 years. The corresponding positive and negative predictive values were 6.7% (95% CI 4.7 – 9.4%) and 96.9% (95% CI 94.2 – 98.4%).

Conclusions In a large, international, multi-centre cohort of children with HCM, ECG abnormalities are common. No ECG characteristic, either in isolation or combined in the ECG risk score, was associated with 5-year MACE risk. This suggests that the role of the baseline ECG phenotype in improving risk stratification in childhood HCM is limited.

Conflict of Interest None

  • paediatric hypertrophic cardiomyopathy
  • resting electrocardiogram
  • sudden cardiac death

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