International external validation study of the 2014 European society of cardiology guidelines on sudden cardiac death prevention in hypertrophic cardiomyopathy (EVIDENCE-HCM)

Constantinos O'Mahony, Fatima Jichi, Steve R. Ommen, Imke Christiaans, Eloisa Arbustini, Pablo Garcia-Pavia, Franco Cecchi, Iacopo Olivotto, Hiroaki Kitaoka, Israel Gotsman, Gerald Carr-White, Jens Mogensen, Loizos Antoniades, Saidi A. Mohiddin, Mathew S. Maurer, Hak Chiaw Tang, Jeffrey B. Geske, Konstantinos C. Siontis, Karim D. Mahmoud, Alexa VermeerArthur Wilde, Valentina Favalli, Oliver P. Guttmann, Maria Gallego-Delgado, Fernando Dominguez, Ilaria Tanini, Toru Kubo, Andre Keren, Teofila Bueser, Sarah Waters, Issa F. Issa, James Malcolmson, Tom Burns, Neha Sekhri, Christopher W. Hoeger, Rumana Z. Omar, Perry M. Elliott

Research output: Contribution to journalArticlepeer-review


BACKGROUND: Identification of people with hypertrophic cardiomyopathy (HCM) who are at risk of sudden cardiac death (SCD) and require a prophylactic implantable cardioverter defibrillator is challenging. In 2014, the European Society of Cardiology proposed a new risk stratification method based on a risk prediction model (HCM Risk-SCD) that estimates the 5-year risk of SCD. The aim was to externally validate the 2014 European Society of Cardiology recommendations in a geographically diverse cohort of patients recruited from the United States, Europe, the Middle East, and Asia. METHODS: This was an observational, retrospective, longitudinal cohort study. RESULTS: The cohort consisted of 3703 patients. Seventy three (2%) patients reached the SCD end point within 5 years of follow-up (5-year incidence, 2.4% [95% confidence interval {CI}, 1.9-3.0]). The validation study revealed a calibration slope of 1.02 (95% CI, 0.93-1.12), C-index of 0.70 (95% CI, 0.68-0.72), and D-statistic of 1.17 (95% CI, 1.05-1.29). In a complete case analysis (n= 2147; 44 SCD end points at 5 years), patients with a predicted 5-year risk of <4% (n=1524; 71%) had an observed 5-year SCD incidence of 1.4% (95% CI, 0.8-2.2); patients with a predicted risk of ≥6% (n=297; 14%) had an observed SCD incidence of 8.9% (95% CI, 5.96-13.1) at 5 years. For every 13 (297/23) implantable cardioverter defibrillator implantations in patients with an estimated 5-year SCD risk ≥6%, 1 patient can potentially be saved from SCD. CONCLUSIONS: This study confirms that the HCM Risk-SCD model provides accurate prognostic information that can be used to target implantable cardioverter defibrillator therapy in patients at the highest risk of SCD.

Original languageEnglish
Pages (from-to)1015-1023
Number of pages9
Issue number10
Publication statusPublished - Mar 1 2018


  • Cardiac
  • Cardiomyopathy
  • Death
  • Defibrillators
  • Forecasting
  • Hypertrophic
  • Implantable
  • Risk assessment
  • Sudden

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)


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