Implantable cardioverter-defibrillators in hypertrophic cardiomyopathy: Patient outcomes, rate of appropriate and inappropriate interventions, and complications

Implantable cardioverter defibrillator therapy in hypertrophic cardiomyopathy: an updated systematic review and meta-analysis of outcomes and complications

“…Similarly, inappropriate shocks occurred at a rate of 4.9% (95% CI: 3.9-6.0) per year (I 2 =75%) ( Figure 3). Among six studies (7,8,10,25,29,33), heart transplant was required in 1.6% of patients per year. The annual incidence of lead malfunction and lead displacement was 1.4% (95% CI: 0.8-2.5) and 1.3% (95% CI: 0.8-2.0) respectively.…”

“…The incidence of sudden death in HCM is low, around 0.7-1% per annum, and often young patients are affected (2). The use of implantable cardioverter-defibrillator (ICD) protects against SCD secondary to ventricular tachycardia (VT), ventricular fibrillation (VF), or bradycardia, with excellent results (3)(4)(5)(6)(7)(8). A major concern with ICD therapy is the delivery of inappropriate shocks, namely in response to supraventricular tachycardia and atrial fibrillation.…”

Hypertrophic Cardiomyopathy in Children, Adolescents, and Young Adults Associated With Low Cardiovascular Mortality With Contemporary Management Strategies

“…51 Also, we would expect that this rate of excess ICDs in HCM patients, and SD rates, as well, will decrease further with emerging advances in risk stratification, including use of contrast-enhanced cardiovascular magnetic resonance. 36 Nevertheless, the sizeable proportion of young patients with device complications cited here and elsewhere [1][2][3][4]20,21,[24][25][26][27] contributes to the ICD decision-making dilemma, 52 in which the preservation of life is weighed against the not insignificant potential for inappropriate shocks and lead-related problems over long periods of time. Indeed, the device complication rate of 26% reported here exceeds by 1.7-fold our appropriate ICD intervention rate of 15%.…”

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However, the past 2 decades have witnessed the emergence of an expanded and effective risk stratification model, resulting in more reliable identification of those patients at increased SD risk, who may become candidates for the life-saving benefits of implantable cardioverter-defibrillators (ICDs), 1,9,11,13,[19][20][21][22][23][24][25][26][27] and advancements in heart transplantation, modern resuscitation techniques, and surgical management of outflow tract obstruction, as well. [9][10][11][12][13]23,23,[28][29][30][31] Whether these contemporary management options have resulted in reduced mortality rates for children and young adults with HCM is currently unresolved, and is the subject of the present clinical outcome investigation.

Methods

Patient Selection

Databases from 2 major HCM tertiary referral centers, Minneapolis Heart Institute Foundation and Tufts Medical Center, were accessed and 474 consecutive patients <30 years of age were identified presenting between 1992 and 2013.

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Validation of the 2014 European Society of Cardiology Guidelines Risk Prediction Model for the Primary Prevention of Sudden Cardiac Death in Hypertrophic Cardiomyopathy

“…Genotype was not predictive of appropriate ICD interventions, 23 but patients with double or triple mutations are at increased risk of end-stage progression and ventricular arrhythmias. 24 Current DNA sequencing is expensive and time consuming; especially if analysis has to be continued after the first mutation has been found.…”