Article Text

Download PDFPDF
Original research article
Effectiveness of the 2014 European Society of Cardiology guideline on sudden cardiac death in hypertrophic cardiomyopathy: a systematic review and meta-analysis
  1. Constantinos O’Mahony1,2,3,
  2. Mohammed Majid Akhtar1,3,2,
  3. Zacharias Anastasiou4,
  4. Oliver P Guttmann1,3,2,
  5. Pieter A Vriesendorp5,
  6. Michelle Michels5,
  7. Damiano Magrì6,
  8. Camillo Autore6,
  9. Adrián Fernández7,
  10. Juan Pablo Ochoa7,8,9,
  11. Kevin M W Leong10,
  12. Amanda M Varnava10,
  13. Lorenzo Monserrat9,11,
  14. Aristides Anastasakis12,
  15. Pablo Garcia-Pavia3,13,14,15,
  16. Claudio Rapezzi16,
  17. Elena Biagini16,
  18. Juan Ramon Gimeno3,17,
  19. Giuseppe Limongelli3,18,
  20. Rumana Z Omar4,
  21. Perry M Elliott1,3,2
  1. 1 The Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew’s Hospital, London, UK
  2. 2 UCL Centre for Heart Muscle Disease, Institute of Cardiovascular Science, University College London, London, UK
  3. 3 European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart (ERN GUARD-HEART)
  4. 4 Department of Statistical Science, University College London, London, UK
  5. 5 Department of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands
  6. 6 Clinical and Molecular Medicine, University ‘La Sapienza’, Rome, Italy
  7. 7 Department of Cardiology, Favaloro Foundation University Hospital, Institute of Cardiology and Cardiovascular Surgery, Buenos Aires, Argentina
  8. 8 GRINCAR (Cardiovascular Research Group), University of A Coruña, A Coruña, Spain
  9. 9 Scientific Department, Health In Code, A Coruña, Spain
  10. 10 Imperial College Healthcare NHS Trust, London, UK
  11. 11 Cardiology Department and Research Unit, A Coruña University Hospital, Galician Health Service, A Coruña, Spain
  12. 12 Unit of Inherited Cardiovascular Diseases, First Department of Cardiology, University of Athens, Athens, Greece
  13. 13 Heart Failure and Inherited Cardiac Diseases Unit, Department of Cardiology, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain
  14. 14 University Francisco de Vitoria (UFV), Madrid, Spain
  15. 15 Centro de Investigacion Biomedica en Red en Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain
  16. 16 Department of Specialised, Experimental and Diagnostic Medicine, Institute of Cardiology, University of Bologna, Bologna, Italy
  17. 17 Cardiac Department, University Hospital Virgen Arrixaca, La Alberca (Murcia), Spain
  18. 18 Monaldi Hospital, Second University of Naples, Naples, Italy
  1. Correspondence to Dr Constantinos O’Mahony, The Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew’s Hospital, London EC1A 7BE, UK; c.omahony{at}


Objective In 2014, the European Society of Cardiology (ESC) recommended the use of a novel risk prediction model (HCM Risk-SCD) to guide use of implantable cardioverter defibrillators (ICD) for the primary prevention of sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). We sought to determine the performance of HCM Risk-SCD by conducting a systematic review and meta-analysis of articles reporting on the prevalence of SCD within 5 years of evaluation in low, intermediate and high-risk patients as defined by the 2014 guidelines (predicted risk <4%, 4%–<6% and ≥6%, respectively).

Methods The protocol was registered with PROSPERO (registration number: CRD42017064203). MEDLINE and manual searches for papers published from October 2014 to December 2017 were performed. Longitudinal, observational cohorts of unselected adult patients, without history of cardiac arrest were considered. The original HCM Risk-SCD development study was included a priori. Data were pooled using a random effects model.

Results Six (0.9%) out of 653 independent publications identified by the initial search were included. The calculated 5-year risk of SCD was reported in 7291 individuals (70% low, 15% intermediate; 15% high risk) with 184 (2.5%) SCD endpoints within 5 years of baseline evaluation. Most SCD endpoints (68%) occurred in patients with an estimated 5-year risk of ≥4% who formed 30% of the total study cohort. Using the random effects method, the pooled prevalence of SCD endpoints was 1.01% (95% CI 0.52 to 1.61) in low-risk patients, 2.43% (95% CI 1.23 to 3.92) in intermediate and 8.4% (95% CI 6.68 to 10.25) in high-risk patients.

Conclusions This meta-analysis demonstrates that HCM Risk-SCD provides accurate risk estimations that can be used to guide ICD therapy in accordance with the 2014 ESC guidelines.

Registration number PROSPERO CRD42017064203;Pre-results.

  • hypertrophic cardiomyopathy
  • implanted cardiac defibrillators
  • ventricular fibrillation

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.


  • Contributors COM and PME designed the study and drafted the manuscript. The search strategy was developed by COM. Two independent reviewers (OPG and MMA) performed the literature search and checked the eligibility of each study. Disagreement between the two reviewing authors was resolved by a third author (COM). Data were extracted by COM and independently verified by MMA. Postpublication data required for the completion of the systematic review/meta-analysis were provided by PAV, MM, DM, CA, AF, JPO, KMWL and AMV. RZO and ZA provided statistical expertise. The quality of the included studies was assessed by two reviewers (MMA, COM). PME is the guarantor. All authors read, critically appraised, provided feedback and approved the final manuscript.

  • Funding This work was undertaken at University College London Hospitals/University College London and St Bartholomew’s Hospital who received a proportion of funding from the UK Department of Health’s National Institute for Health Research Biomedical Research Centres funding scheme. OPG received research support from the British Heart Foundation (FS/12/86/29841) and the National Institute for Health Research University College London Hospitals Biomedical Research Centre. The present work was partially supported by a research grant from ’Sapienza' University of Rome to DM. KMWL was supported by a British Heart Foundation Grant (PG/15/20/31339) and The Dan Bagshaw Memorial Trust Fund. LM received a Carlos III Health Institute grant, Madrid, Spain (PI11/0260). GL received support from a Progetto ministeriale (MIUR), Italy (PRIN 2008). PGP was supported by Institutode Salud Carlos III (grants PI14/0967 and RD012/0042/0066) through the Plan Estatal de I+D+I 2013-2016–European Regional Development Fund (FEDER): ’A way of making Europe'. JRG received support from the Cardiovascular Research Network (RIC) from the Carlos III Health Institute, Madrid, Spain (RD12/0042/0049).

  • Competing interests MMA reports being supported by an unrestricted educational grant from Sanofi Genzyme, outside the submitted work. LM is an employee and a stakeholder of Health in Code SL. All other authors have nothing to disclose.

  • Patient consent Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement Not applicable.