Current perspectives of sudden cardiac death management in hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disorder characterized by left ventricular hypertrophy. Sudden cardiac death (SCD) is a rare but the most catastrophic complication in patients with HCM. Implantable cardioverter-defibrillators (ICDs) are widely recognized as effective preventive measures for SCD. Individualized risk stratification and early intervention in HCM can significantly improve patient prognosis. In this study, we review the latest findings regarding pathogenesis, risk stratification, and prevention of SCD in HCM patients, highlighting the clinic practice of cardiovascular magnetic resonance imaging for SCD management.

The Prognostic Value of Left Ventricular Entropy From T1 Mapping in Patients With Hypertrophic Cardiomyopathy.

Background: The prognostic value of left ventricular (LV) entropy in hypertrophic cardiomyopathy (HCM) is unclear. Objectives: This study aimed to assess the prognostic value of LV entropy from T1 mapping in HCM. Methods: A total of 748 participants with HCM, who underwent cardiovascular magnetic resonance (CMR), were consecutively enrolled. LV entropy was quantified by native T1 mapping. A competing risk analysis and a Cox proportional hazards regression analysis were performed to identify potential associations of LV entropy with sudden cardiac death (SCD) and cardiovascular death (CVD), respectively. Results: A total of 40 patients with HCM experienced SCD, and 65 experienced CVD during a median follow-up of 43 months. Participants with increased LV entropy (≥4.06) were more likely to experience SCD and CVD (all P < 0.05) in the entire study cohort or the subgroup with low late gadolinium enhancement (LGE) extent (<15%). After adjustment for the European Society of Cardiology predictors and the presence of high LGE extent (≥15%), LV mean entropy was an independent predictor for SCD (HR: 1.03; all P < 0.05) by the multivariable competing risk analysis and CVD (HR: 1.06; 95% CI: 1.03-1.09; P < 0.001) by multivariable Cox regression analysis. Conclusions: LV mean entropy derived from native T1 mapping, reflecting myocardial tissue heterogeneity, was an independent predictor of SCD and CVD in participants with HCM. (Cardiac Magnetic Resonance Imaging Clinical Application Registration Study; ChiCTR1900024094).

Validation of the Risk Stratification for Sudden Cardiac Death in Chinese Patients With Hypertrophic Cardiomyopathy.

Accurate identification of hypertrophic cardiomyopathy (HCM) patients at high risk of sudden cardiac death (SCD) event is challenging. The objective of this study was to validate the three SCD risk stratifications recommended by the 2014 European Society of Cardiology (ESC) guideline, the 2020 American Heart Association /American College of Cardiology (AHA/ACC) guideline, and the 2022 ESC guideline in Chinese patients with HCM. Our study population are made up of a cohort of 856 HCM patients without prior SCD events. The endpoint was defined as SCD or equivalent events (successful resuscitation after cardiac arrest or appropriate ICD shock for ventricular tachycardia or ventricular fibrillation). During a median follow-up of 43 months, SCD endpoints occurred in 44 (5.1%) patients. A total of 34 (77.3%) patients suffering from SCD events were classified correctly into high-risk groups by the 2020 AHA/ACC guideline, 27(61.4%) by the 2022 ESC guideline, and 13 (29.6%) by the 2014 ESC guideline. The C-statistic of the 2020 AHA/ACC guideline was 0.68 (95% CI, 0.60-0.76), which performed better than the 2022 ESC guideline (0.65: 95% CI, 0.56-0.73), and the 2014 ESC guideline (0.58: 95% CI, 0.48-0.67). The 2020 AHA/ACC guideline displayed better discrimination for SCD risk stratification in Chinese HCM patients than the other two guidelines, with a higher sensitivity but lower specificity.