Importance of newer cardiac magnetic resonance–based risk markers for sudden death prevention in hypertrophic cardiomyopathy: An international multicenter study

Ethan J. Rowin; Martin S. Maron; Arnon Adler; Alfred J. Albano; Armanda M. Varnava; Danna Spears; Dana Marsy; Stephen B. Heitner; Emilie Cohen; Kevin M.W. Leong; Stephen L. Winters; Matthew W. Martinez; Benjamin C. Koethe; Harry Rakowski; Barry J. Maron

doi:10.1016/j.hrthm.2021.12.017

Importance of newer cardiac magnetic resonance–based risk markers for sudden death prevention in hypertrophic cardiomyopathy: An international multicenter study

Ethan J. Rowin, Martin S. Maron, Arnon Adler, Alfred J. Albano, Armanda M. Varnava, Danna Spears, Dana Marsy, Stephen B. Heitner, Emilie Cohen, Kevin M.W. Leong, Stephen L. Winters, Matthew W. Martinez, Benjamin C. Koethe, Harry Rakowski, Barry J. Maron

Research output: Contribution to journal › Article › peer-review

14 Scopus citations

Abstract

Background: The sudden death (SD) risk stratification algorithm in hypertrophic cardiomyopathy (HCM) has evolved, underscored recently by novel cardiac magnetic resonance (CMR)–based risk markers (left ventricular apical aneurysm, extensive late gadolinium enhancement, and end-stage disease with systolic dysfunction) incorporated into the 2020 American Heart Association (AHA)/American College of Cardiology (ACC) HCM guidelines. Objective: The purpose of this study was to assess the specific impact of newer, predominantly CMR-based risk markers in a large multicenter HCM population that underwent primary prevention implantable cardioverter-defibrillator (ICD) implants. Methods: Longitudinal study of 1149 consecutive HCM patients from 6 North American and European HCM centers prospectively judged to be at high SD risk based on ≥1 AHA/ACC individual risk markers and prophylactically implanted with an ICD was performed. European Society of Cardiology (ESC) risk score was retrospectively analyzed with respect to the known clinical outcome. Results: Of 1149 patients with an ICD, 162 (14%) experienced device therapy terminating ventricular tachycardia/ventricular fibrillation 4.6 ± 4.2 years after implant. CMR-based markers solely or in combination led to ICD implantation in 49 of the 162 patients (30%) experiencing device therapy. Particularly low ESC scores (<4%/5 years) would have excluded an ESC ICD recommendation for 67 patients who nevertheless experienced appropriate ICD therapy, including 26 with the CMR-based risk markers not part of the ESC formula. Conclusion: Identification and incorporation of novel guideline-supported CMR-based risk markers enhance selection of HCM patients for SD prevention with ICDs. Absence of CMR-based markers from the ESC risk score accounts, in part, for it not identifying many HCM patients with SD events. These data support inclusion of CMR as a routine part of HCM patient evaluation and risk stratification.

Original language	English
Pages (from-to)	782-789
Number of pages	8
Journal	Heart Rhythm
Volume	19
Issue number	5
DOIs	https://doi.org/10.1016/j.hrthm.2021.12.017
State	Published - May 2022
Externally published	Yes

Keywords

Cardiac magnetic resonance imaging
Hypertrophic cardiomyopathy
Implantable cardioverter-defibrillator
Risk stratification
Sudden death

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10.1016/j.hrthm.2021.12.017

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Rowin, E. J., Maron, M. S., Adler, A., Albano, A. J., Varnava, A. M., Spears, D., Marsy, D., Heitner, S. B., Cohen, E., Leong, K. M. W., Winters, S. L., Martinez, M. W., Koethe, B. C., Rakowski, H., & Maron, B. J. (2022). Importance of newer cardiac magnetic resonance–based risk markers for sudden death prevention in hypertrophic cardiomyopathy: An international multicenter study. Heart Rhythm, 19(5), 782-789. https://doi.org/10.1016/j.hrthm.2021.12.017

@article{fde4cc65066548419e6c36e7008f4996,

title = "Importance of newer cardiac magnetic resonance–based risk markers for sudden death prevention in hypertrophic cardiomyopathy: An international multicenter study",

abstract = "Background: The sudden death (SD) risk stratification algorithm in hypertrophic cardiomyopathy (HCM) has evolved, underscored recently by novel cardiac magnetic resonance (CMR)–based risk markers (left ventricular apical aneurysm, extensive late gadolinium enhancement, and end-stage disease with systolic dysfunction) incorporated into the 2020 American Heart Association (AHA)/American College of Cardiology (ACC) HCM guidelines. Objective: The purpose of this study was to assess the specific impact of newer, predominantly CMR-based risk markers in a large multicenter HCM population that underwent primary prevention implantable cardioverter-defibrillator (ICD) implants. Methods: Longitudinal study of 1149 consecutive HCM patients from 6 North American and European HCM centers prospectively judged to be at high SD risk based on ≥1 AHA/ACC individual risk markers and prophylactically implanted with an ICD was performed. European Society of Cardiology (ESC) risk score was retrospectively analyzed with respect to the known clinical outcome. Results: Of 1149 patients with an ICD, 162 (14%) experienced device therapy terminating ventricular tachycardia/ventricular fibrillation 4.6 ± 4.2 years after implant. CMR-based markers solely or in combination led to ICD implantation in 49 of the 162 patients (30%) experiencing device therapy. Particularly low ESC scores (<4%/5 years) would have excluded an ESC ICD recommendation for 67 patients who nevertheless experienced appropriate ICD therapy, including 26 with the CMR-based risk markers not part of the ESC formula. Conclusion: Identification and incorporation of novel guideline-supported CMR-based risk markers enhance selection of HCM patients for SD prevention with ICDs. Absence of CMR-based markers from the ESC risk score accounts, in part, for it not identifying many HCM patients with SD events. These data support inclusion of CMR as a routine part of HCM patient evaluation and risk stratification.",

keywords = "Cardiac magnetic resonance imaging, Hypertrophic cardiomyopathy, Implantable cardioverter-defibrillator, Risk stratification, Sudden death",

author = "Rowin, {Ethan J.} and Maron, {Martin S.} and Arnon Adler and Albano, {Alfred J.} and Varnava, {Armanda M.} and Danna Spears and Dana Marsy and Heitner, {Stephen B.} and Emilie Cohen and Leong, {Kevin M.W.} and Winters, {Stephen L.} and Martinez, {Matthew W.} and Koethe, {Benjamin C.} and Harry Rakowski and Maron, {Barry J.}",

note = "Publisher Copyright: {\textcopyright} 2021 Heart Rhythm Society",

year = "2022",

month = may,

doi = "10.1016/j.hrthm.2021.12.017",

language = "English",

volume = "19",

pages = "782--789",

journal = "Heart Rhythm",

issn = "1547-5271",

publisher = "Elsevier",

number = "5",

}

Rowin, EJ, Maron, MS, Adler, A, Albano, AJ, Varnava, AM, Spears, D, Marsy, D, Heitner, SB, Cohen, E, Leong, KMW, Winters, SL, Martinez, MW, Koethe, BC, Rakowski, H & Maron, BJ 2022, 'Importance of newer cardiac magnetic resonance–based risk markers for sudden death prevention in hypertrophic cardiomyopathy: An international multicenter study', Heart Rhythm, vol. 19, no. 5, pp. 782-789. https://doi.org/10.1016/j.hrthm.2021.12.017

TY - JOUR

T1 - Importance of newer cardiac magnetic resonance–based risk markers for sudden death prevention in hypertrophic cardiomyopathy

T2 - An international multicenter study

AU - Rowin, Ethan J.

AU - Maron, Martin S.

AU - Adler, Arnon

AU - Albano, Alfred J.

AU - Varnava, Armanda M.

AU - Spears, Danna

AU - Marsy, Dana

AU - Heitner, Stephen B.

AU - Cohen, Emilie

AU - Leong, Kevin M.W.

AU - Winters, Stephen L.

AU - Martinez, Matthew W.

AU - Koethe, Benjamin C.

AU - Rakowski, Harry

AU - Maron, Barry J.

PY - 2022/5

Y1 - 2022/5

N2 - Background: The sudden death (SD) risk stratification algorithm in hypertrophic cardiomyopathy (HCM) has evolved, underscored recently by novel cardiac magnetic resonance (CMR)–based risk markers (left ventricular apical aneurysm, extensive late gadolinium enhancement, and end-stage disease with systolic dysfunction) incorporated into the 2020 American Heart Association (AHA)/American College of Cardiology (ACC) HCM guidelines. Objective: The purpose of this study was to assess the specific impact of newer, predominantly CMR-based risk markers in a large multicenter HCM population that underwent primary prevention implantable cardioverter-defibrillator (ICD) implants. Methods: Longitudinal study of 1149 consecutive HCM patients from 6 North American and European HCM centers prospectively judged to be at high SD risk based on ≥1 AHA/ACC individual risk markers and prophylactically implanted with an ICD was performed. European Society of Cardiology (ESC) risk score was retrospectively analyzed with respect to the known clinical outcome. Results: Of 1149 patients with an ICD, 162 (14%) experienced device therapy terminating ventricular tachycardia/ventricular fibrillation 4.6 ± 4.2 years after implant. CMR-based markers solely or in combination led to ICD implantation in 49 of the 162 patients (30%) experiencing device therapy. Particularly low ESC scores (<4%/5 years) would have excluded an ESC ICD recommendation for 67 patients who nevertheless experienced appropriate ICD therapy, including 26 with the CMR-based risk markers not part of the ESC formula. Conclusion: Identification and incorporation of novel guideline-supported CMR-based risk markers enhance selection of HCM patients for SD prevention with ICDs. Absence of CMR-based markers from the ESC risk score accounts, in part, for it not identifying many HCM patients with SD events. These data support inclusion of CMR as a routine part of HCM patient evaluation and risk stratification.

AB - Background: The sudden death (SD) risk stratification algorithm in hypertrophic cardiomyopathy (HCM) has evolved, underscored recently by novel cardiac magnetic resonance (CMR)–based risk markers (left ventricular apical aneurysm, extensive late gadolinium enhancement, and end-stage disease with systolic dysfunction) incorporated into the 2020 American Heart Association (AHA)/American College of Cardiology (ACC) HCM guidelines. Objective: The purpose of this study was to assess the specific impact of newer, predominantly CMR-based risk markers in a large multicenter HCM population that underwent primary prevention implantable cardioverter-defibrillator (ICD) implants. Methods: Longitudinal study of 1149 consecutive HCM patients from 6 North American and European HCM centers prospectively judged to be at high SD risk based on ≥1 AHA/ACC individual risk markers and prophylactically implanted with an ICD was performed. European Society of Cardiology (ESC) risk score was retrospectively analyzed with respect to the known clinical outcome. Results: Of 1149 patients with an ICD, 162 (14%) experienced device therapy terminating ventricular tachycardia/ventricular fibrillation 4.6 ± 4.2 years after implant. CMR-based markers solely or in combination led to ICD implantation in 49 of the 162 patients (30%) experiencing device therapy. Particularly low ESC scores (<4%/5 years) would have excluded an ESC ICD recommendation for 67 patients who nevertheless experienced appropriate ICD therapy, including 26 with the CMR-based risk markers not part of the ESC formula. Conclusion: Identification and incorporation of novel guideline-supported CMR-based risk markers enhance selection of HCM patients for SD prevention with ICDs. Absence of CMR-based markers from the ESC risk score accounts, in part, for it not identifying many HCM patients with SD events. These data support inclusion of CMR as a routine part of HCM patient evaluation and risk stratification.

KW - Cardiac magnetic resonance imaging

KW - Hypertrophic cardiomyopathy

KW - Implantable cardioverter-defibrillator

KW - Risk stratification

KW - Sudden death

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U2 - 10.1016/j.hrthm.2021.12.017

DO - 10.1016/j.hrthm.2021.12.017

M3 - Article

C2 - 34933112

AN - SCOPUS:85123070674

SN - 1547-5271

VL - 19

SP - 782

EP - 789

JO - Heart Rhythm

JF - Heart Rhythm

IS - 5

ER -

Importance of newer cardiac magnetic resonance–based risk markers for sudden death prevention in hypertrophic cardiomyopathy: An international multicenter study

Abstract

Keywords

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