Clinical Course and Quality of Life in High-Risk Patients with Hypertrophic Cardiomyopathy and Implantable Cardioverter-Defibrillators

Barry J. Maron, Susan A. Casey, Iacopo Olivotto, Mark V. Sherrid, Christopher Semsarian, Camillo Autore, Aisha Ahmed, Giuseppe Boriani, Pietro Francia, Stephen Winters, Michael Giudici, Anna Koulova, Ross Garberich, Ethan J. Rowin, Samuel F. Sears, Martin S. Maron, Paolo Spirito

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

Background: High-risk patients with hypertrophic cardiomyopathy (HCM) are identified by contemporary risk stratification and effectively treated with implantable cardioverter-defibrillators (ICDs). However, long-term HCM clinical course after ICD therapy for ventricular tachyarrhythmias is incompletely understood. Methods and Results: Cohort of 486 high-risk HCM patients with ICDs was assembled from 8 international centers. Clinical course and device interventions were addressed, and survey questionnaires assessed patient anxiety level and psychological well-being related to ICD therapy. Of 486 patients, 94 (19%) experienced appropriate ICD interventions terminating ventricular tachycardia/ventricular fibrillation, 3.7% per year for primary prevention, over 6.4±4.7 years. Of 94 patients, 87 were asymptomatic or only mildly symptomatic at the time of appropriate ICD interventions; 74 of these 87 (85%) remained in classes I/II without significant change in clinical status over the subsequent 5.9±4.9 years (up to 22). Among the 94 patients, there was one sudden death (caused by device failure; 1.1%); 3 patients died from other HCM-related processes unrelated to arrhythmic risk (eg, end-stage heart failure). Post-ICD intervention, freedom from HCM mortality was 100%, 97%, and 92% at 1, 5, and 10 years, distinctly lower than in ischemic or nonischemic cardiomyopathy ICD trials. HCM patients with ICD interventions reported heightened anxiety in expectation of future shocks, but with intact general psychological well-being and quality of life. Conclusions: In HCM, unlike ischemic heart disease, prevention of sudden death with ICD therapy is unassociated with significant increase in cardiovascular morbidity or mortality, or transformation to heart failure deterioration. ICD therapy does not substantially impair overall psychological and physical well-being.

Original languageEnglish (US)
Article numbere005820
JournalCirculation: Arrhythmia and Electrophysiology
Volume11
Issue number4
DOIs
StatePublished - Apr 1 2018

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Implantable Defibrillators
Hypertrophic Cardiomyopathy
Quality of Life
Psychology
Sudden Death
Anxiety
Heart Failure
Equipment Failure
Mortality
Ventricular Fibrillation
Primary Prevention
Therapeutics
Ventricular Tachycardia
Cardiomyopathies
Tachycardia
Myocardial Ischemia
Shock
Morbidity

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Maron, Barry J. ; Casey, Susan A. ; Olivotto, Iacopo ; Sherrid, Mark V. ; Semsarian, Christopher ; Autore, Camillo ; Ahmed, Aisha ; Boriani, Giuseppe ; Francia, Pietro ; Winters, Stephen ; Giudici, Michael ; Koulova, Anna ; Garberich, Ross ; Rowin, Ethan J. ; Sears, Samuel F. ; Maron, Martin S. ; Spirito, Paolo. / Clinical Course and Quality of Life in High-Risk Patients with Hypertrophic Cardiomyopathy and Implantable Cardioverter-Defibrillators. In: Circulation: Arrhythmia and Electrophysiology. 2018 ; Vol. 11, No. 4.
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title = "Clinical Course and Quality of Life in High-Risk Patients with Hypertrophic Cardiomyopathy and Implantable Cardioverter-Defibrillators",
abstract = "Background: High-risk patients with hypertrophic cardiomyopathy (HCM) are identified by contemporary risk stratification and effectively treated with implantable cardioverter-defibrillators (ICDs). However, long-term HCM clinical course after ICD therapy for ventricular tachyarrhythmias is incompletely understood. Methods and Results: Cohort of 486 high-risk HCM patients with ICDs was assembled from 8 international centers. Clinical course and device interventions were addressed, and survey questionnaires assessed patient anxiety level and psychological well-being related to ICD therapy. Of 486 patients, 94 (19{\%}) experienced appropriate ICD interventions terminating ventricular tachycardia/ventricular fibrillation, 3.7{\%} per year for primary prevention, over 6.4±4.7 years. Of 94 patients, 87 were asymptomatic or only mildly symptomatic at the time of appropriate ICD interventions; 74 of these 87 (85{\%}) remained in classes I/II without significant change in clinical status over the subsequent 5.9±4.9 years (up to 22). Among the 94 patients, there was one sudden death (caused by device failure; 1.1{\%}); 3 patients died from other HCM-related processes unrelated to arrhythmic risk (eg, end-stage heart failure). Post-ICD intervention, freedom from HCM mortality was 100{\%}, 97{\%}, and 92{\%} at 1, 5, and 10 years, distinctly lower than in ischemic or nonischemic cardiomyopathy ICD trials. HCM patients with ICD interventions reported heightened anxiety in expectation of future shocks, but with intact general psychological well-being and quality of life. Conclusions: In HCM, unlike ischemic heart disease, prevention of sudden death with ICD therapy is unassociated with significant increase in cardiovascular morbidity or mortality, or transformation to heart failure deterioration. ICD therapy does not substantially impair overall psychological and physical well-being.",
author = "Maron, {Barry J.} and Casey, {Susan A.} and Iacopo Olivotto and Sherrid, {Mark V.} and Christopher Semsarian and Camillo Autore and Aisha Ahmed and Giuseppe Boriani and Pietro Francia and Stephen Winters and Michael Giudici and Anna Koulova and Ross Garberich and Rowin, {Ethan J.} and Sears, {Samuel F.} and Maron, {Martin S.} and Paolo Spirito",
year = "2018",
month = "4",
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Maron, BJ, Casey, SA, Olivotto, I, Sherrid, MV, Semsarian, C, Autore, C, Ahmed, A, Boriani, G, Francia, P, Winters, S, Giudici, M, Koulova, A, Garberich, R, Rowin, EJ, Sears, SF, Maron, MS & Spirito, P 2018, 'Clinical Course and Quality of Life in High-Risk Patients with Hypertrophic Cardiomyopathy and Implantable Cardioverter-Defibrillators', Circulation: Arrhythmia and Electrophysiology, vol. 11, no. 4, e005820. https://doi.org/10.1161/CIRCEP.117.005820

Clinical Course and Quality of Life in High-Risk Patients with Hypertrophic Cardiomyopathy and Implantable Cardioverter-Defibrillators. / Maron, Barry J.; Casey, Susan A.; Olivotto, Iacopo; Sherrid, Mark V.; Semsarian, Christopher; Autore, Camillo; Ahmed, Aisha; Boriani, Giuseppe; Francia, Pietro; Winters, Stephen; Giudici, Michael; Koulova, Anna; Garberich, Ross; Rowin, Ethan J.; Sears, Samuel F.; Maron, Martin S.; Spirito, Paolo.

In: Circulation: Arrhythmia and Electrophysiology, Vol. 11, No. 4, e005820, 01.04.2018.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Clinical Course and Quality of Life in High-Risk Patients with Hypertrophic Cardiomyopathy and Implantable Cardioverter-Defibrillators

AU - Maron, Barry J.

AU - Casey, Susan A.

AU - Olivotto, Iacopo

AU - Sherrid, Mark V.

AU - Semsarian, Christopher

AU - Autore, Camillo

AU - Ahmed, Aisha

AU - Boriani, Giuseppe

AU - Francia, Pietro

AU - Winters, Stephen

AU - Giudici, Michael

AU - Koulova, Anna

AU - Garberich, Ross

AU - Rowin, Ethan J.

AU - Sears, Samuel F.

AU - Maron, Martin S.

AU - Spirito, Paolo

PY - 2018/4/1

Y1 - 2018/4/1

N2 - Background: High-risk patients with hypertrophic cardiomyopathy (HCM) are identified by contemporary risk stratification and effectively treated with implantable cardioverter-defibrillators (ICDs). However, long-term HCM clinical course after ICD therapy for ventricular tachyarrhythmias is incompletely understood. Methods and Results: Cohort of 486 high-risk HCM patients with ICDs was assembled from 8 international centers. Clinical course and device interventions were addressed, and survey questionnaires assessed patient anxiety level and psychological well-being related to ICD therapy. Of 486 patients, 94 (19%) experienced appropriate ICD interventions terminating ventricular tachycardia/ventricular fibrillation, 3.7% per year for primary prevention, over 6.4±4.7 years. Of 94 patients, 87 were asymptomatic or only mildly symptomatic at the time of appropriate ICD interventions; 74 of these 87 (85%) remained in classes I/II without significant change in clinical status over the subsequent 5.9±4.9 years (up to 22). Among the 94 patients, there was one sudden death (caused by device failure; 1.1%); 3 patients died from other HCM-related processes unrelated to arrhythmic risk (eg, end-stage heart failure). Post-ICD intervention, freedom from HCM mortality was 100%, 97%, and 92% at 1, 5, and 10 years, distinctly lower than in ischemic or nonischemic cardiomyopathy ICD trials. HCM patients with ICD interventions reported heightened anxiety in expectation of future shocks, but with intact general psychological well-being and quality of life. Conclusions: In HCM, unlike ischemic heart disease, prevention of sudden death with ICD therapy is unassociated with significant increase in cardiovascular morbidity or mortality, or transformation to heart failure deterioration. ICD therapy does not substantially impair overall psychological and physical well-being.

AB - Background: High-risk patients with hypertrophic cardiomyopathy (HCM) are identified by contemporary risk stratification and effectively treated with implantable cardioverter-defibrillators (ICDs). However, long-term HCM clinical course after ICD therapy for ventricular tachyarrhythmias is incompletely understood. Methods and Results: Cohort of 486 high-risk HCM patients with ICDs was assembled from 8 international centers. Clinical course and device interventions were addressed, and survey questionnaires assessed patient anxiety level and psychological well-being related to ICD therapy. Of 486 patients, 94 (19%) experienced appropriate ICD interventions terminating ventricular tachycardia/ventricular fibrillation, 3.7% per year for primary prevention, over 6.4±4.7 years. Of 94 patients, 87 were asymptomatic or only mildly symptomatic at the time of appropriate ICD interventions; 74 of these 87 (85%) remained in classes I/II without significant change in clinical status over the subsequent 5.9±4.9 years (up to 22). Among the 94 patients, there was one sudden death (caused by device failure; 1.1%); 3 patients died from other HCM-related processes unrelated to arrhythmic risk (eg, end-stage heart failure). Post-ICD intervention, freedom from HCM mortality was 100%, 97%, and 92% at 1, 5, and 10 years, distinctly lower than in ischemic or nonischemic cardiomyopathy ICD trials. HCM patients with ICD interventions reported heightened anxiety in expectation of future shocks, but with intact general psychological well-being and quality of life. Conclusions: In HCM, unlike ischemic heart disease, prevention of sudden death with ICD therapy is unassociated with significant increase in cardiovascular morbidity or mortality, or transformation to heart failure deterioration. ICD therapy does not substantially impair overall psychological and physical well-being.

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U2 - 10.1161/CIRCEP.117.005820

DO - 10.1161/CIRCEP.117.005820

M3 - Article

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VL - 11

JO - Circulation: Arrhythmia and Electrophysiology

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