Art and science of risk stratification of sudden cardiac death in hypertrophic cardiomyopathy: Current state, unknowns, and future directions

The progress in the management of hypertrophic cardiomyopathy (HCM) over the last several decades has resulted in great improvements in quality of life and overall survival for HCM patients. Yet, sudden cardiac death (SCD) due to ventricular tachyarrhythmias is among the common causes of HCM-related mortality. SCD risk stratification is a central and often challenging domain in the care of the HCM patient. Distinguishing the individuals most likely to benefit from a primary prevention implantable-cardioverter defibrillator (ICD) from those truly at a low risk of SCD in whom an ICD is not necessary is a nuanced process. Clinicians need to carefully balance the potential benefit and risks of ICDs, particularly in young patients. Because of intense investigations in diverse HCM cohorts globally, two main approaches to SCD risk stratification in HCM have emerged, one based on major SCD risk factors and one based on a mathematically derived risk score. In this overview, we discuss the current state, latest advances and remaining unknowns about established and novel markers of risk of SCD in HCM. We also review how the risk factor- and risk score-based assessments can and should be used in conjunction to enhance rather than contradict each other in facilitating informed ICD decision-making in contemporary clinical practice.