Related Experiment Video
Updated: May 22, 2025
Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
Published on: August 8, 2022
Validation of Guideline Recommendation on Sudden Cardiac Death Prevention in Hypertrophic Cardiomyopathy
Masashi Amano1, Hiroaki Kitaoka2, Yusuke Yoshikawa3
1Department of Heart Failure and Transplantation, National Cerebral and Cardiovascular Center, Suita, Japan.
Insights
Sudden cardiac death (SCD) risk is highest in end-stage hypertrophic cardiomyopathy (HCM) patients. Current guidelines offer some risk stratification, but the 2024 ACC/AHA guidelines appear to better differentiate risk between certain patient classes.
Area of Science:
- Cardiology
- Genetics
- Public Health
Background:
- Hypertrophic cardiomyopathy (HCM) poses a risk of sudden cardiac death (SCD).
- The HCM Risk-SCD calculator and current guidelines inform decisions for implantable cardioverter-defibrillator (ICD) placement.
- Accurate risk stratification is crucial for preventing SCD in HCM patients.
Purpose of the Study:
- To evaluate clinical profiles and SCD occurrence across different HCM phenotypes.
- To validate the performance of current SCD risk prediction guidelines using a large Japanese registry.
- To compare the effectiveness of the 2024 ACC/AHA and 2023 ESC guidelines in stratifying SCD risk.
Main Methods:
- A retrospective, multicenter, observational, longitudinal cohort study.
- Inclusion of 3,611 consecutive patients diagnosed with HCM.
- Primary endpoint: composite of SCD or an equivalent event, with a 5-year follow-up.
Main Results:
- End-stage HCM (ejection fraction <50%) showed a high 5-year SCD incidence (18.5%).
- Midventricular obstruction and nonobstructive HCM had lower 5-year SCD rates (6.9% and 4.7%).
- Both 2024 ACC/AHA and 2023 ESC guidelines stratified SCD risk, but the 2024 ACC/AHA guidelines showed better differentiation between recommendation Classes 2a and 2b.
Conclusions:
- Patients with end-stage HCM exhibit a significantly worse prognosis regarding SCD.
- Current guidelines effectively stratify SCD risk in HCM patients.
- The 2024 ACC/AHA guidelines demonstrate improved risk stratification capabilities between specific risk classes compared to the 2023 ESC guidelines.
Background:
To prevent sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM), the HCM Risk-SCD calculator and guideline recommendations are used to aid decision making for implantable cardioverter-defibrillator placement.
Objectives:
The aim of this study was to assess the clinical profiles and occurrence of SCD by phenotypes of HCM and validate the performance of the current guidelines from a large-scale Japanese multicenter registry.
Methods:
This was a retrospective, multicenter, observational, longitudinal cohort study that enrolled 3,611 consecutive patients with HCM. The primary endpoint was a composite of SCD or an equivalent event.
Results:
The 5-year cumulative incidence of SCD events was markedly high in patients with end-stage HCM, defined by ejection fraction <50% (18.5%), followed by midventricular obstruction and nonobstructive HCM (6.9% and 4.7%). The 5-year cumulative incidence rates of SCD events for each recommendation class by the 2 guidelines were as follows: with the 2024 ACC (American College of Cardiology)/AHA (American Heart Association) guidelines, 23.8%, 7.2%, 5.7%, and 2.3% for Classes 1, 2a, 2b, and 3, respectively, and with the 2023 ESC (European Society of Cardiology) guidelines, 23.8%, 2.9%, 9.3%, and 2.6%, respectively. The 5-year risk was not well stratified between Classes 2a and 2b with the 2024 ACC/AHA guidelines (P = 0.101), and the event rate was even reversed with the 2023 ESC guidelines (P = 0.545).
Conclusions:
Among HCM phenotypes, the prognosis of patients with end-stage HCM was markedly worse. The 2024 ACC/AHA and 2023 ESC guidelines well stratified SCD risk in patients with HCM; the 2024 ACC/AHA guidelines seemed to better stratify SCD risk between Classes 2a and 2b compared with the 2023 ESC guidelines.
Related Concept Videos
01:17Pathophysiology of Heart Failure
01:10Pre-Procedural Guidelines for Assessing Blood Pressure