Sudden cardiac death in nonischemic cardiomyopathy: Refining risk assessment

Sudden cardiac death (SCD) risk assessment among patients with nonischemic cardiomyopathy (NICM) has been has been less straightforward than for patients with ischemic cardiomyopathy. The common surrogate that has been associated with highest SCD risk for all cardiomyopathies, and which has been universally used to guide implantation of primary‐prevention implantable cardioverter‐defibrillators (ICDs), is left ventricular ejection fraction (LVEF) ≤35%. However, this practice has been called into question, especially in light of recent trials suggesting that ICD treatment may not be of additional survival benefit among those with NICM treated with optimal medical therapy. This Clinical Review attempts to offer refinements to the current practice of SCD risk assessment among patients with NICM, with specific focus on importance of NICM etiology and efforts to identify myocardial scarring and arrhythmogenic substrate, both of which may provide greater information about SCD risk than the LVEF alone. These concepts are illustrated further as they apply to hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, and cardiac sarcoidosis, all of which are increasingly recognized NICM substrates associated with SCD and for which refinements for assessing risk are lacking in conventional guidelines.