Contemporary strategies for risk stratification and prevention of sudden death with the implantable defibrillator in hypertrophic cardiomyopathy

Maron, Barry J.; Maron, Martin S.

doi:10.1016/j.hrthm.2015.12.048

Cited by 69 publications

(62 citation statements)

References 88 publications

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“…31 Our findings in HCM generally support the principle of patient satisfaction with the ICD initiative, focused on preservation of life in high-risk patients with this complex genetic disease. 1,3,4,6,8,9 Attention to patient concerns, and apprehension related to ICD shocks, could ultimately mitigate the modestly elevated levels of shock anxiety identified in this study.…”

Section: Discussionmentioning

confidence: 95%

“…First, data from our international multicenter HCM population reinforces the principle that device therapy is efficacious in terminating VT/VF, restoring sinus rhythm, and preventing sudden death. [1][2][3][4][5][6][7][8][9][10][11] As a result of this >15-year ICD initiative, 3,9 substantial numbers of patients prophylactically implanted with devices have survived with good quality of life. The data reported here are also consistent with our prior experience (and that of other investigators), albeit with a slightly lower ICD event rate, probably attributable to greater numbers of primary prevention devices implanted most recently as this therapy continues to penetrate into HCM practice.…”

Section: Discussionmentioning

confidence: 99%

“…1 The implantable cardioverter-defibrillator (ICD) is now established as a fundamental therapy for prevention of sudden death in this patient population, independent of age, and based on a series of multicenter studies and meta-analyses over the past >15 years. [1][2][3][4][5][6][7][8][9][10][11] Nevertheless, a remaining question is whether ICD interventions that terminate ventricular tachycardia/ ventricular fibrillation (VT/VF) trigger deterioration in clinical course or impairment in quality of life (including psychological). Therefore, to address whether longterm clinical course and mortality risk subsequent to termination of life-threatening arrhythmias is distinctive in HCM, we have assembled a large multicenter international cohort of high-risk HCM patients with ICDs.…”

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confidence: 99%

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

Maron

Casey

Olivotto

et al. 2018

Circ: Arrhythmia and Electrophysiology

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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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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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

Maron

Casey

Olivotto

et al. 2018

Circ: Arrhythmia and Electrophysiology

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“…This is comparable to high-risk patients with an ischaemic cardiomyopathy. 30 There is no doubt that the ICD is efficacious in preventing SCD. The primary difficulty for the clinician arises when trying to select patients for whom the risks are outweighed by the benefits of ICD implantation.…”

Section: Primary Prevention Of Sudden Cardiac Deathmentioning

confidence: 99%

“…The difficulty in using our existing knowledge of SCD in HCM is that our current established risk markers have a low positive predictive value (10-20 %). 24,30,52 To improve the predictive value, the guidelines combine certain risk markers with risk modifiers. It should be noted that simply adding risk factors does not always result in a cumulative increase in the risk of SCD.…”

Section: Accf/aha Recommendations For Implantable Cardioverter-defibrmentioning

confidence: 99%

ICD Therapy for Primary Prevention in Hypertrophic Cardiomyopathy

Trivedi

Knight

2016

Arrhythmia &Amp; Electrophysiology Review

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HCM is a heterogeneous disease that is classically transmitted in an autosomal dominant fashion. HCM often arises from mutations in genes responsible for the formation of cardiac sarcomeres.Sarcomeric mutations, initially discovered in the 1980s, are found in up to 70 % of patients with a family history of HCM.11 The most common mutations are in the myosin heavy chain (MYH7 gene) and myosin binding protein C (MYBPC3 gene). 12 There is marked heterogeneity in penetrance, however, and a poor understanding of how the different genotypes manifest as HCM phenotypes. Currently, there is minimal evidence to support the use of genetic testing to identify patients who are at high risk of SCD. Small studies have found that patients with a more malignant inheritance of their mutations (i.e. homozygous mutations) as well as the presence of a MYH7 gene mutation have AbstractHypertrophic cardiomyopathy (HCM) is a common and heterogeneous disorder that increases an individual's risk of sudden cardiac death (SCD). This review article discusses the relevant factors that are involved in the challenge of preventing SCD in patients with HCM.The epidemiology of SCD in patients is reviewed as well as the structural and genetic basis behind ventricular arrhythmias in HCM. The primary prevention of SCD with implantable cardioverter-defibrillator (ICD) therapy is the cornerstone of modern treatment for individuals at high risk of SCD. The focus here is on the current and emerging predictors of SCD as well as risk stratification recommendations from both North American and European guidelines. Issues related to ICD implantation, such as programming, complications and inappropriate therapies, are discussed. The emerging role of the fully subcutaneous ICD and the data regarding its implantation are reviewed. KeywordsHypertrophic cardiomyopathy, sudden cardiac death, implantable cardioverter defibrillator, subcutaneous implantable cardioverter-defibrillator Disclosure: Dr Trivedi has no conflicts of interest to declare. Dr Knight has been a consultant to manufacturers of implantable defibrillators and participated in clinical trials sponsored

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

et al. 2019

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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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Contemporary strategies for risk stratification and prevention of sudden death with the implantable defibrillator in hypertrophic cardiomyopathy

Cited by 69 publications

References 88 publications

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

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

ICD Therapy for Primary Prevention in Hypertrophic Cardiomyopathy

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

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