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 journalArticle

21 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1015-1023
Number of pages9
JournalCirculation
Volume137
Issue number10
DOIs
Publication statusPublished - Mar 1 2018

Fingerprint

Validation Studies
Hypertrophic Cardiomyopathy
Sudden Cardiac Death
Cardiology
Guidelines
Confidence Intervals
Implantable Defibrillators
Incidence
Middle East
Far East
Calibration
Longitudinal Studies
Cohort Studies

Keywords

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

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

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.

In: Circulation, Vol. 137, No. 10, 01.03.2018, p. 1015-1023.

Research output: Contribution to journalArticle

O'Mahony, C, Jichi, F, Ommen, SR, Christiaans, I, Arbustini, E, Garcia-Pavia, P, Cecchi, F, Olivotto, I, Kitaoka, H, Gotsman, I, Carr-White, G, Mogensen, J, Antoniades, L, Mohiddin, SA, Maurer, MS, Tang, HC, Geske, JB, Siontis, KC, Mahmoud, KD, Vermeer, A, Wilde, A, Favalli, V, Guttmann, OP, Gallego-Delgado, M, Dominguez, F, Tanini, I, Kubo, T, Keren, A, Bueser, T, Waters, S, Issa, IF, Malcolmson, J, Burns, T, Sekhri, N, Hoeger, CW, Omar, RZ & Elliott, PM 2018, 'International external validation study of the 2014 European society of cardiology guidelines on sudden cardiac death prevention in hypertrophic cardiomyopathy (EVIDENCE-HCM)', Circulation, vol. 137, no. 10, pp. 1015-1023. https://doi.org/10.1161/CIRCULATIONAHA.117.030437
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. / International external validation study of the 2014 European society of cardiology guidelines on sudden cardiac death prevention in hypertrophic cardiomyopathy (EVIDENCE-HCM). In: Circulation. 2018 ; Vol. 137, No. 10. pp. 1015-1023.
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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.",
keywords = "Cardiac, Cardiomyopathy, Death, Defibrillators, Forecasting, Hypertrophic, Implantable, Risk assessment, Sudden",
author = "Constantinos O'Mahony and Fatima Jichi and Ommen, {Steve R.} and Imke Christiaans and Eloisa Arbustini and Pablo Garcia-Pavia and Franco Cecchi and Iacopo Olivotto and Hiroaki Kitaoka and Israel Gotsman and Gerald Carr-White and Jens Mogensen and Loizos Antoniades and Mohiddin, {Saidi A.} and Maurer, {Mathew S.} and Tang, {Hak Chiaw} and Geske, {Jeffrey B.} and Siontis, {Konstantinos C.} and Mahmoud, {Karim D.} and Alexa Vermeer and Arthur Wilde and Valentina Favalli and Guttmann, {Oliver P.} and Maria Gallego-Delgado and Fernando Dominguez and Ilaria Tanini and Toru Kubo and Andre Keren and Teofila Bueser and Sarah Waters and Issa, {Issa F.} and James Malcolmson and Tom Burns and Neha Sekhri and Hoeger, {Christopher W.} and Omar, {Rumana Z.} and Elliott, {Perry M.}",
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TY - JOUR

T1 - International external validation study of the 2014 European society of cardiology guidelines on sudden cardiac death prevention in hypertrophic cardiomyopathy (EVIDENCE-HCM)

AU - O'Mahony, Constantinos

AU - Jichi, Fatima

AU - Ommen, Steve R.

AU - Christiaans, Imke

AU - Arbustini, Eloisa

AU - Garcia-Pavia, Pablo

AU - Cecchi, Franco

AU - Olivotto, Iacopo

AU - Kitaoka, Hiroaki

AU - Gotsman, Israel

AU - Carr-White, Gerald

AU - Mogensen, Jens

AU - Antoniades, Loizos

AU - Mohiddin, Saidi A.

AU - Maurer, Mathew S.

AU - Tang, Hak Chiaw

AU - Geske, Jeffrey B.

AU - Siontis, Konstantinos C.

AU - Mahmoud, Karim D.

AU - Vermeer, Alexa

AU - Wilde, Arthur

AU - Favalli, Valentina

AU - Guttmann, Oliver P.

AU - Gallego-Delgado, Maria

AU - Dominguez, Fernando

AU - Tanini, Ilaria

AU - Kubo, Toru

AU - Keren, Andre

AU - Bueser, Teofila

AU - Waters, Sarah

AU - Issa, Issa F.

AU - Malcolmson, James

AU - Burns, Tom

AU - Sekhri, Neha

AU - Hoeger, Christopher W.

AU - Omar, Rumana Z.

AU - Elliott, Perry M.

PY - 2018/3/1

Y1 - 2018/3/1

N2 - 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.

AB - 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.

KW - Cardiac

KW - Cardiomyopathy

KW - Death

KW - Defibrillators

KW - Forecasting

KW - Hypertrophic

KW - Implantable

KW - Risk assessment

KW - Sudden

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