Enhanced American College of Cardiology/American Heart Association Strategy for Prevention of Sudden Cardiac Death in High-Risk Patients with Hypertrophic Cardiomyopathy

Martin S. Maron, Ethan J. Rowin, Benjamin S. Wessler, Paula J. Mooney, Amber Fatima, Parth Patel, Benjamin C. Koethe, Mikhail Romashko, Mark Steven Link, Barry J. Maron

Research output: Contribution to journalArticle

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Abstract

Importance: Strategies for reliable selection of high-risk patients with hypertrophic cardiomyopathy (HCM) for prevention of sudden cardiac death (SCD) with implantable cardioverter/defibrillators (ICDs) are incompletely resolved. Objective: To assess the reliability of SCD prediction methods leading to prophylactic ICD recommendations to reduce the number of SCDs occurring in patients with HCM. Design, Setting, and Participants: In this observational longitudinal study, 2094 predominantly adult patients with HCM consecutively evaluated over 17 years in a large HCM clinical center were studied. All patients underwent prospective ICD decision making relying on individual major risk markers derived from the HCM literature and an enhanced American College of Cardiology/American Heart Association (ACC/AHA) guidelines-based risk factor algorithm with complete clinical outcome follow-up. Data were collected from June 2017 to February 2018, and data were analyzed from February to July 2018. Main Outcomes and Measures: Arrhythmic SCD or appropriate ICD intervention for ventricular tachycardia or ventricular fibrillation. Results: Of the 2094 study patients, 1313 (62.7%) were male, and the mean (SD) age was 51 (17) years. Of 527 patients with primary prevention ICDs implanted based on 1 or more major risk markers, 82 (15.6%) experienced device therapy-terminated ventricular tachycardia or ventricular fibrillation episodes, which exceeded the 5 HCM-related SCDs occurring among 1567 patients without ICDs (0.3%), including 2 who declined device therapy, by 49-fold (95% CI, 20-119; P =.001). Cumulative 5-year probability of an appropriate ICD intervention was 10.5% (95% CI, 8.0-13.5). The enhanced ACC/AHA clinical risk factor strategy was highly sensitive for predicting SCD events (range, 87%-95%) but less specific for identifying patients without SCD events (78%). The C statistic calculated for enhanced ACC/AHA guidelines was 0.81 (95% CI, 0.77-0.85), demonstrating good discrimination between patients who did or did not experience an SCD event. Compared with enhanced ACC/AHA risk factors, the European Society of Cardiology risk score retrospectively applied to the study patients was much less sensitive than the ACC/AHA criteria (34% [95% CI, 22-44] vs 95% [95% CI, 89-99]), consistent with recognizing fewer high-risk patients. Conclusions and Relevance: A systematic enhanced ACC/AHA guideline and practice-based risk factor strategy prospectively predicted SCD events in nearly all at-risk patients with HCM, resulting in prophylactically implanted ICDs that prevented many catastrophic arrhythmic events in this at-risk population.

Original languageEnglish (US)
JournalJAMA Cardiology
DOIs
StatePublished - Jan 1 2019

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Hypertrophic Cardiomyopathy
Sudden Cardiac Death
Implantable Defibrillators
American Heart Association
Cardiology
Ventricular Fibrillation
Ventricular Tachycardia
Guidelines
Equipment and Supplies
Primary Prevention
Practice Guidelines
Observational Studies
Longitudinal Studies
Decision Making
Outcome Assessment (Health Care)

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Enhanced American College of Cardiology/American Heart Association Strategy for Prevention of Sudden Cardiac Death in High-Risk Patients with Hypertrophic Cardiomyopathy. / Maron, Martin S.; Rowin, Ethan J.; Wessler, Benjamin S.; Mooney, Paula J.; Fatima, Amber; Patel, Parth; Koethe, Benjamin C.; Romashko, Mikhail; Link, Mark Steven; Maron, Barry J.

In: JAMA Cardiology, 01.01.2019.

Research output: Contribution to journalArticle

Maron, Martin S. ; Rowin, Ethan J. ; Wessler, Benjamin S. ; Mooney, Paula J. ; Fatima, Amber ; Patel, Parth ; Koethe, Benjamin C. ; Romashko, Mikhail ; Link, Mark Steven ; Maron, Barry J. / Enhanced American College of Cardiology/American Heart Association Strategy for Prevention of Sudden Cardiac Death in High-Risk Patients with Hypertrophic Cardiomyopathy. In: JAMA Cardiology. 2019.
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abstract = "Importance: Strategies for reliable selection of high-risk patients with hypertrophic cardiomyopathy (HCM) for prevention of sudden cardiac death (SCD) with implantable cardioverter/defibrillators (ICDs) are incompletely resolved. Objective: To assess the reliability of SCD prediction methods leading to prophylactic ICD recommendations to reduce the number of SCDs occurring in patients with HCM. Design, Setting, and Participants: In this observational longitudinal study, 2094 predominantly adult patients with HCM consecutively evaluated over 17 years in a large HCM clinical center were studied. All patients underwent prospective ICD decision making relying on individual major risk markers derived from the HCM literature and an enhanced American College of Cardiology/American Heart Association (ACC/AHA) guidelines-based risk factor algorithm with complete clinical outcome follow-up. Data were collected from June 2017 to February 2018, and data were analyzed from February to July 2018. Main Outcomes and Measures: Arrhythmic SCD or appropriate ICD intervention for ventricular tachycardia or ventricular fibrillation. Results: Of the 2094 study patients, 1313 (62.7{\%}) were male, and the mean (SD) age was 51 (17) years. Of 527 patients with primary prevention ICDs implanted based on 1 or more major risk markers, 82 (15.6{\%}) experienced device therapy-terminated ventricular tachycardia or ventricular fibrillation episodes, which exceeded the 5 HCM-related SCDs occurring among 1567 patients without ICDs (0.3{\%}), including 2 who declined device therapy, by 49-fold (95{\%} CI, 20-119; P =.001). Cumulative 5-year probability of an appropriate ICD intervention was 10.5{\%} (95{\%} CI, 8.0-13.5). The enhanced ACC/AHA clinical risk factor strategy was highly sensitive for predicting SCD events (range, 87{\%}-95{\%}) but less specific for identifying patients without SCD events (78{\%}). The C statistic calculated for enhanced ACC/AHA guidelines was 0.81 (95{\%} CI, 0.77-0.85), demonstrating good discrimination between patients who did or did not experience an SCD event. Compared with enhanced ACC/AHA risk factors, the European Society of Cardiology risk score retrospectively applied to the study patients was much less sensitive than the ACC/AHA criteria (34{\%} [95{\%} CI, 22-44] vs 95{\%} [95{\%} CI, 89-99]), consistent with recognizing fewer high-risk patients. Conclusions and Relevance: A systematic enhanced ACC/AHA guideline and practice-based risk factor strategy prospectively predicted SCD events in nearly all at-risk patients with HCM, resulting in prophylactically implanted ICDs that prevented many catastrophic arrhythmic events in this at-risk population.",
author = "Maron, {Martin S.} and Rowin, {Ethan J.} and Wessler, {Benjamin S.} and Mooney, {Paula J.} and Amber Fatima and Parth Patel and Koethe, {Benjamin C.} and Mikhail Romashko and Link, {Mark Steven} and Maron, {Barry J.}",
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T1 - Enhanced American College of Cardiology/American Heart Association Strategy for Prevention of Sudden Cardiac Death in High-Risk Patients with Hypertrophic Cardiomyopathy

AU - Maron, Martin S.

AU - Rowin, Ethan J.

AU - Wessler, Benjamin S.

AU - Mooney, Paula J.

AU - Fatima, Amber

AU - Patel, Parth

AU - Koethe, Benjamin C.

AU - Romashko, Mikhail

AU - Link, Mark Steven

AU - Maron, Barry J.

PY - 2019/1/1

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N2 - Importance: Strategies for reliable selection of high-risk patients with hypertrophic cardiomyopathy (HCM) for prevention of sudden cardiac death (SCD) with implantable cardioverter/defibrillators (ICDs) are incompletely resolved. Objective: To assess the reliability of SCD prediction methods leading to prophylactic ICD recommendations to reduce the number of SCDs occurring in patients with HCM. Design, Setting, and Participants: In this observational longitudinal study, 2094 predominantly adult patients with HCM consecutively evaluated over 17 years in a large HCM clinical center were studied. All patients underwent prospective ICD decision making relying on individual major risk markers derived from the HCM literature and an enhanced American College of Cardiology/American Heart Association (ACC/AHA) guidelines-based risk factor algorithm with complete clinical outcome follow-up. Data were collected from June 2017 to February 2018, and data were analyzed from February to July 2018. Main Outcomes and Measures: Arrhythmic SCD or appropriate ICD intervention for ventricular tachycardia or ventricular fibrillation. Results: Of the 2094 study patients, 1313 (62.7%) were male, and the mean (SD) age was 51 (17) years. Of 527 patients with primary prevention ICDs implanted based on 1 or more major risk markers, 82 (15.6%) experienced device therapy-terminated ventricular tachycardia or ventricular fibrillation episodes, which exceeded the 5 HCM-related SCDs occurring among 1567 patients without ICDs (0.3%), including 2 who declined device therapy, by 49-fold (95% CI, 20-119; P =.001). Cumulative 5-year probability of an appropriate ICD intervention was 10.5% (95% CI, 8.0-13.5). The enhanced ACC/AHA clinical risk factor strategy was highly sensitive for predicting SCD events (range, 87%-95%) but less specific for identifying patients without SCD events (78%). The C statistic calculated for enhanced ACC/AHA guidelines was 0.81 (95% CI, 0.77-0.85), demonstrating good discrimination between patients who did or did not experience an SCD event. Compared with enhanced ACC/AHA risk factors, the European Society of Cardiology risk score retrospectively applied to the study patients was much less sensitive than the ACC/AHA criteria (34% [95% CI, 22-44] vs 95% [95% CI, 89-99]), consistent with recognizing fewer high-risk patients. Conclusions and Relevance: A systematic enhanced ACC/AHA guideline and practice-based risk factor strategy prospectively predicted SCD events in nearly all at-risk patients with HCM, resulting in prophylactically implanted ICDs that prevented many catastrophic arrhythmic events in this at-risk population.

AB - Importance: Strategies for reliable selection of high-risk patients with hypertrophic cardiomyopathy (HCM) for prevention of sudden cardiac death (SCD) with implantable cardioverter/defibrillators (ICDs) are incompletely resolved. Objective: To assess the reliability of SCD prediction methods leading to prophylactic ICD recommendations to reduce the number of SCDs occurring in patients with HCM. Design, Setting, and Participants: In this observational longitudinal study, 2094 predominantly adult patients with HCM consecutively evaluated over 17 years in a large HCM clinical center were studied. All patients underwent prospective ICD decision making relying on individual major risk markers derived from the HCM literature and an enhanced American College of Cardiology/American Heart Association (ACC/AHA) guidelines-based risk factor algorithm with complete clinical outcome follow-up. Data were collected from June 2017 to February 2018, and data were analyzed from February to July 2018. Main Outcomes and Measures: Arrhythmic SCD or appropriate ICD intervention for ventricular tachycardia or ventricular fibrillation. Results: Of the 2094 study patients, 1313 (62.7%) were male, and the mean (SD) age was 51 (17) years. Of 527 patients with primary prevention ICDs implanted based on 1 or more major risk markers, 82 (15.6%) experienced device therapy-terminated ventricular tachycardia or ventricular fibrillation episodes, which exceeded the 5 HCM-related SCDs occurring among 1567 patients without ICDs (0.3%), including 2 who declined device therapy, by 49-fold (95% CI, 20-119; P =.001). Cumulative 5-year probability of an appropriate ICD intervention was 10.5% (95% CI, 8.0-13.5). The enhanced ACC/AHA clinical risk factor strategy was highly sensitive for predicting SCD events (range, 87%-95%) but less specific for identifying patients without SCD events (78%). The C statistic calculated for enhanced ACC/AHA guidelines was 0.81 (95% CI, 0.77-0.85), demonstrating good discrimination between patients who did or did not experience an SCD event. Compared with enhanced ACC/AHA risk factors, the European Society of Cardiology risk score retrospectively applied to the study patients was much less sensitive than the ACC/AHA criteria (34% [95% CI, 22-44] vs 95% [95% CI, 89-99]), consistent with recognizing fewer high-risk patients. Conclusions and Relevance: A systematic enhanced ACC/AHA guideline and practice-based risk factor strategy prospectively predicted SCD events in nearly all at-risk patients with HCM, resulting in prophylactically implanted ICDs that prevented many catastrophic arrhythmic events in this at-risk population.

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