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International External Validation Study of the 2014 European Society of Cardiology Guidelines on Sudden Cardiac Death Prevention in Hypertrophic Cardiomyopathy (EVIDENCE-HCM)

O’Mahony, Constantinos, Jichi, Fatima, Ommen, Steve R., Christiaans, Imke, Arbustini, Eloisa, Garcia-Pavia, Pablo, Cecchi, Franco, Olivotto, Iacopo, Kitaoka, Hiroaki, Gotsman, Israel, Carr-White, Gerald, Mogensen, Jens, Antoniades, Loizos, Mohiddin, Saidi A., Maurer, Mathew S., Tang, Hak Chiaw, Geske, Jeffrey B., Siontis, Konstantinos C., Mahmoud, Karim D., Vermeer, Alexa, Wilde, Arthur, Favalli, Valentina, Guttmann, Oliver P., Gallego-Delgado, Maria, Dominguez, Fernando, Tanini, Ilaria, Kubo, Toru, Keren, Andre, Bueser, Teofila, Waters, Sarah, Issa, Issa F., Malcolmson, James, Burns, Tom, Sekhri, Neha, Hoeger, Christopher W., Omar, Rumana Z., Elliott, Perry M.·2018·Circulation

Abstract

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.

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Authors
O’Mahony, C., Jichi, F., Ommen, S. R., Christiaans, I., Arbustini, E., Garcia-Pavia, P., Cecchi, F., Olivotto, I., Kitaoka, H., Gotsman, I., Carr-White, G., Mogensen, J., Antoniades, L., Mohiddin, S. A., Maurer, M. S., Tang, H. C., Geske, J. B., Siontis, K. C., Mahmoud, K. D., Vermeer, A., Wilde, A., Favalli, V., Guttmann, O. P., Gallego-Delgado, M., Dominguez, F., Tanini, I., Kubo, T., Keren, A., Bueser, T., Waters, S., Issa, I. F., Malcolmson, J., Burns, T., Sekhri, N., Hoeger, C. W., Omar, R. Z., & Elliott, P. M.
Venue
Circulation
Published
2018-03-06
Volume
137
Issue
10
Pages
1015-1023
ISSN
  • 0009-7322
  • 1524-4539
Cited by
201
References
6
Language
en

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