P atients with hypertrophic cardiomyopathy (HCM) are at increased risk for sudden cardiac death (SCD), mostly caused by ventricular arrhythmias. SCD may occur as the initial presentation of HCM, often in asymptomatic or mildly symptomatic patients. 1 In fact, HCM is the most frequent cause of SCD in young people, including trained athletes. 2,3 Implantable cardioverter defibrillator (ICD) therapy may effectively terminate potentially life-threatening ventricular arrhythmias, thereby preventing SCD and prolonging life. Still, ICD therapy is not without risk, because inappropriate interventions and device-related complications may occur. Clinical Perspective on p 559Previous observational studies have reported on the use of ICD therapy for primary and secondary preventions of SCD in HCM. A complete overview of outcome and complications after ICD therapy in patients with HCM at risk for SCD is currently not available. The goal of this analysis was to pool the individual studies in an effort to examine the precise rate of cardiac and noncardiac mortality, appropriate and inappropriate interventions, and complications. This knowledge may aid clinical decision making and counseling in patients with HCM at increased risk for SCD considered for ICD therapy. Methods Study DesignThis systematic review and meta-analysis included all available original studies reporting clinical outcome and complications in patients with HCM who underwent ICD implantation. Studies that did not provide data on outcome or complications and review manuscripts were excluded. Studies focussing on SCD in patients with HCM without ICD were excluded. Literature SearchThe online MEDLINE database was searched for literature in Background-Previous observational studies demonstrated that patients with hypertrophic cardiomyopathy at risk for sudden cardiac death (SCD) may benefit from implantable cardioverter defibrillator (ICD) therapy. A complete overview of outcome and complications after ICD therapy is currently not available. This study pools data from published studies on outcome and complications after ICD therapy in patients with hypertrophic cardiomyopathy. Methods and Results-A PubMed database search returned 27 studies on 16 cohorts reporting outcome and complications after ICD therapy in patients with hypertrophic cardiomyopathy. In case of >1 publications on a particular cohort, the publication with the largest number of patients was included in the meta-analysis. ICD interventions, complications, and mortality rates were extracted, pooled, and analyzed. There were 2190 patients (mean age, 42 years; 38% women), most of whom (83%) received an ICD for primary prevention of SCD. Risk factors for SCD were left ventricular wall thickness ≥30 mm (20%), family history of SCD (43%), nonsustained ventricular tachycardia (46%), syncope (41%), and abnormal blood pressure response (25%). During the 3.7-year follow-up, the annualized cardiac mortality rate was 0.6%, the noncardiac mortality rate was 0.4%, and the appropriate ICD intervention rate was 3...

Long-term mortality and (aborted) SCD rates after ASA and myectomy are similarly low. Patients who undergo ASA have more than twice the risk of permanent pacemaker implantation and a 5 times higher risk of the need for additional septal reduction therapy compared with those who undergo myectomy.

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