Abstract
Sudden cardiac death (SCD) is the most devastating complication in hypertrophic cardiomyopathy (HCM). The implantable cardioverter–defibrillator (ICD) has proven to be effective in SCD prevention in several clinical scenarios. In HCM population, it has demonstrated to successfully abort life-threatening ventricular arrhythmias despite the extreme morphology characteristic of HCM, often with massive degrees of left ventricular hypertrophy and/or LV outflow tract obstruction. Studies showed a high rate of appropriate intervention in secondary prevention and in primary prevention of patients considered at high risk. This appropriate intervention rate is even more significant considering the young and otherwise healthy patients that compose HCM population. Since SCD incidence in HCM is relatively low, optimal identification of patients at high risk is crucial. Classical strategy of risk stratification based on clinical risk factors has several limitations and has proven to overestimate risk. A new risk prediction model that provides individual 5-year estimated risk appears to be superior to traditional models based on bivariate risk factors. Perioperative complications seem to be similar to those related to the implant of other cardiac devices, while long-term complications have been traditionally in the spotlight. HCM patients are considered more vulnerable to ICD-related complications and inappropriate ICD therapy because of their young age at implant and increased prevalence of atrial fibrillation, but long-term follow-up data on ICD-related complications in general practice is limited. The subcutaneous implantable cardioverter defibrillator seems to be a safe and effective alternative in HCM, although long-term data are scarce.
Highlights
Hypertrophic cardiomyopathy is a genetically determined heart muscle disease, most often caused by mutations in one of the sarcomere proteins genes, that confers a very diverse natural history[1,2,3]
The average age of patients at the time of implantation was 40 years and more than 25 percent were under the age of 31 years (Figure 1). 34 percent of the defibrillators were implanted for secondary prevention, while the remaining 66 percent defibrillators were implanted prophylactically for primary prevention, in considered high-risk patients, consideration that was based on the generally accepted risk factors for Sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) patients
The implantable cardioverter–defibrillator (ICD) has proved to be a safe and effective therapy preventing SCD in HCM patients, which constitutes probably the major disease related concern. It reliably aborts lifethreatening ventricular arrhythmias in those HCM patients judged to be at high risk for SCD, without needing to perform defibrillation threshold (DFT) testing routinely
Summary
Hypertrophic cardiomyopathy is a genetically determined heart muscle disease, most often caused by mutations in one of the sarcomere proteins genes, that confers a very diverse natural history[1,2,3]. While annual appropriate intervention rates are lower in patients with HCM than in those with CAD, they are truly significant given the context of a much younger population usually free of limiting heart failure symptoms and other comorbidities This long high risk period is especially important considering that the interval from ICD implant to first appropriate device intervention is variable, and often considerable in length, even as long as 10 years. Appropriate interventions occurred in 14 percent of patients judged at increased risk for SCD by risk stratification, with cumulative 5-year probability of discharge of 12% These rates predict the important impact of the therapy over the many years that these young and usually free from heart failure patients will be at risk. These issues need to be carefully considered when evaluating therapeutic options in children with HCM
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