Efficacy of Implantable Cardioverter–Defibrillators for the Prevention of Sudden Death in Patients with Hypertrophic Cardiomyopathy

Maron, Barry J.; Shen, Win Kuang; Link, Mark S.; Epstein, Andrew E.; Almquist, Adrian K.; Daubert, James P.; Bardy, Gust H.; Favale, Stefano; Rea, Robert F.; Boriani, Giuseppe; Estes, N.A. Mark; Spirito, Paolo; Casey, Susan A.; Stanton, Marshall S.; Betocchi, Sandro

doi:10.1056/nejm200002103420601

Cited by 915 publications

(647 citation statements)

References 40 publications

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“…Seven of these individuals had inappropriate shocks attributable to lead failure. In an early HCM ICD series, 12 of 128 (9.4%) individuals followed for an average of 3.1 years had a lead fracture 2. Nine of these had an inappropriate shock.…”

Section: Discussionmentioning

confidence: 99%

Subcutaneous Implantable Cardioverter Defibrillator in Patients With Hypertrophic Cardiomyopathy: An Initial Experience

Weinstock

Bader

Maron

et al. 2016

JAHA

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BackgroundThe subcutaneous implantable cardioverter defibrillator (S‐ICD) has been developed to avert risks associated with transvenous defibrillator leads. The technology is attractive for younger patients, such as those with hypertrophic cardiomyopathy (HCM). However, there are limited data on S‐ICD use in HCM.Methods and Results HCM patients identified at risk for sudden death were considered for S‐ICD implantation. Patients were screened for potential oversensing by surface electrocardiography (ECG). At implant, defibrillation threshold (DFT) testing was performed at 65, 50, and 35 joules (J). Twenty‐seven patients were considered for S‐ICD implantation, and after screening, 23 (85%) remained eligible. The presence of a bundle branch block was associated with screening failure, whereas elevated body mass index (BMI) showed a trend toward association. One patient passed screening at rest, but failed with an ECG obtained after exercise. At implant, the S‐ICD terminated ventricular fibrillation (VF) with a 65J shock in all 15 implanted patients and a 50J shock was successful in 12 of 15. A 35J shock terminated VF in 10 of 12 patients. DFT failure at 50 J was associated with a higher BMI. There were no appropriate shocks after a median follow‐up of 17.5 (3–35) months, and 1 patient received an inappropriate shock attributable to a temporary reduction in QRS amplitude while bending forward, resulting in oversensing, despite successful screening.ConclusionsIn a high‐risk HCM cohort without a pacing indication referred for consideration of an ICD, the majority were eligible for S‐ICD. The S‐ICD is effective at recognizing and terminating VF at implant with a wide safety margin.

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Section: Discussionmentioning

confidence: 99%

Subcutaneous Implantable Cardioverter Defibrillator in Patients With Hypertrophic Cardiomyopathy: An Initial Experience

Weinstock

Bader

Maron

et al. 2016

JAHA

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“…Decisions regarding ICD implantation and programming of these devices were made by the managing cardiologist and/or electrophysiologist. Stored intracardiac electrograms were analyzed to classify arrhythmias responsible for precipitating defibrillator discharges, according to following definitions 5. VF or VFL was defined as an irregular or regular tachycardia with a mean cycle length (CL) of ≤240 ms. VT was defined as a regular tachycardia arising from the ventricle with a CL >240 and <600 ms. First appropriate ICD intervention was defined as an ICD therapy for VT/VF.…”

Section: Methodsmentioning

confidence: 99%

Implantable Cardioverter‐Defibrillator Therapy in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Predictors of Appropriate Therapy, Outcomes, and Complications

Orgeron

James

Riele

et al. 2017

JAHA

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BackgroundArrhythmogenic right ventricular dysplasia/cardiomyopathy is characterized by ventricular arrhythmias and sudden cardiac death. Once the diagnosis is established, risk stratification to determine whether implantable cardioverter‐defibrillator (ICD) placement is warranted is critical.Methods and ResultsThe cohort included 312 patients (163 men, age at presentation 33.6±13.9 years) with definite arrhythmogenic right ventricular dysplasia/cardiomyopathy who received an ICD. Over 8.8±7.33 years, 186 participants (60%) had appropriate ICD therapy and 58 (19%) had an intervention for ventricular fibrillation/flutter. Ventricular tachycardia at presentation (hazard ratio [HR]: 1.86; 95% confidence interval [CI], 1.38–2.49; P<0.001), inducibility on electrophysiology study (HR: 3.14; 95% CI, 1.95–5.05; P<0.001), male sex (HR: 1.62; 95% CI, 1.20–2.19; P=0.001), inverted T waves in ≥3 precordial leads (HR: 1.66; 95% CI, 1.09–2.52; P=0.018), and premature ventricular contraction count ≥1000/24 hours (HR: 2.30; 95% CI, 1.32–4.00; P=0.003) were predictors of any appropriate ICD therapy. Inducibility at electrophysiology study (HR: 2.28; 95% CI, 1.10–4.70; P=0.025) remained as the only predictor after multivariable analysis. The predictors for ventricular fibrillation/flutter were premature ventricular contraction ≥1000/24 hours (HR: 4.39; 95% CI, 1.32–14.61; P=0.016), syncope (HR: 1.85; 95% CI, 1.10–3.11; P=0.021), aged ≤30 years at presentation (HR: 1.76; 95% CI, 1.04–3.00; P<0.036), and male sex (HR: 1.73; 95% CI, 1.01–2.97; P=0.046). Younger age at presentation (HR: 3.14; 95% CI, 1.32–7.48; P=0.010) and high premature ventricular contraction burden (HR: 4.43; 95% CI, 1.35–14.57; P<0.014) remained as independent predictors of ventricular fibrillation/flutter. Complications occurred in 66 participants (21%), and 64 (21%) had inappropriate ICD interventions. Overall mortality was low at 2%, and 4% underwent heart transplantation.ConclusionThese findings represent an important step in identifying predictors of ICD therapy for potentially fatal ventricular fibrillation/flutter and should be considered when developing a risk stratification model for arrhythmogenic right ventricular dysplasia/cardiomyopathy.

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“…Clinical signs and symptoms of disease include shortness of breath, angina, palpitations, syncope, and, most importantly, sudden death. Cardiac arrhythmias cause both syncope and sudden death in HCM (3).…”

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confidence: 99%

Somatic events modify hypertrophic cardiomyopathy pathology and link hypertrophy to arrhythmia

Wolf

Moskowitz

Arno

et al. 2005

Proc. Natl. Acad. Sci. U.S.A.

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Sarcomere protein gene mutations cause hypertrophic cardiomyopathy (HCM), a disease with distinctive histopathology and increased susceptibility to cardiac arrhythmias and risk for sudden death. Myocyte disarray (disorganized cell-cell contact) and cardiac fibrosis, the prototypic but protean features of HCM histopathology, are presumed triggers for ventricular arrhythmias that precipitate sudden death events. To assess relationships between arrhythmias and HCM pathology without confounding human variables, such as genetic heterogeneity of disease-causing mutations, background genotypes, and lifestyles, we studied cardiac electrophysiology, hypertrophy, and histopathology in mice engineered to carry an HCM mutation. Both genetically outbred and inbred HCM mice had variable susceptibility to arrhythmias, differences in ventricular hypertrophy, and variable amounts and distribution of histopathology. Among inbred HCM mice, neither the extent nor location of myocyte disarray or cardiac fibrosis correlated with ex vivo signal conduction properties or in vivo electrophysiologically stimulated arrhythmias. In contrast, the amount of ventricular hypertrophy was significantly associated with increased arrhythmia susceptibility. These data demonstrate that distinct somatic events contribute to variable HCM pathology and that cardiac hypertrophy, more than fibrosis or disarray, correlates with arrhythmic risk. We suggest that a shared pathway triggered by sarcomere gene mutations links cardiac hypertrophy and arrhythmias in HCM.fibrosis ͉ somatic modifiers ͉ disarray ͉ electrophysiology ͉ left ventrical wall thickness

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Efficacy of Implantable Cardioverter–Defibrillators for the Prevention of Sudden Death in Patients with Hypertrophic Cardiomyopathy

Cited by 915 publications

References 40 publications

Subcutaneous Implantable Cardioverter Defibrillator in Patients With Hypertrophic Cardiomyopathy: An Initial Experience

Subcutaneous Implantable Cardioverter Defibrillator in Patients With Hypertrophic Cardiomyopathy: An Initial Experience

Implantable Cardioverter‐Defibrillator Therapy in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Predictors of Appropriate Therapy, Outcomes, and Complications

Somatic events modify hypertrophic cardiomyopathy pathology and link hypertrophy to arrhythmia

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