Updated risk assessments for sudden cardiac death in hypertrophic cardiomyopathy patients with implantable cardioverter-defibrillator

Abstract

Hypertrophic cardiomyopathy (HCM) is a genetic disease associated with a risk of malignant ventricular tachyarrhythmias and sudden cardiac death (SCD). Assessment of the SCD risk is crucial for its clinical management, and there has been considerable interest in developing risk stratification strategies. An implantable cardioverter-defibrillator (ICD) is a life-saving treatment for patients with HCM who are at a high-risk of ventricular tachyarrhythmias and SCD. However, a substantial number of ICD recipients experience adverse effects arising from inappropriate device therapy and implant-related complications. This has led to numerous investigations of the risk of SCD and the indications for ICD implantation. American guidelines were recently updated to include new risk markers, including left ventricular systolic dysfunction, apical aneurysm, and extensive late gadolinium enhancement, while European guidelines recommend individualized estimated 5-year SCD risk assessment models. Studies evaluating other risk factors for SCD in patients with HCM have also been published. Drawing on recent guidelines and publications on clinical risk factors, we focus this review on updated risk assessments for SCD with ICD therapy in patients with HCM.

Optimization of guideline-directed medical treatment for heart failure patients with reduced ejection fraction

“…Hypertrophic cardiomyopathy (HCM), characterized by cardiac hypertrophy resulting from sarcomere mutations, may manifest with overt dysfunction in 5–10% of cases [ 58 ]. In patients with HCM and preserved EF, the primary therapeutic objectives include alleviating LV outflow tract obstruction, maintaining optimal volume status, and preventing sudden cardiac death [ 59 ]. In cases of burnout-phase HCM, the initiation of HF medications such as ARNIs, ACE inhibitors, ARBs, beta-blockers, and MRAs may be considered [ 60 ].…”