Validation of the HCM Risk-SCD model in patients with hypertrophic cardiomyopathy and future perspectives
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Published on: August 8, 2022
Levent Pay1, Tuğba Çetin2, Şeyda Dereli2
1Department of Cardiology, Ardahan State Hospital, Sugoze, Turkey.
Insights
The hypertrophic cardiomyopathy (HCM) Risk-Sudden Cardiac Death (SCD) model identifies high-risk patients but may miss some individuals. A new, more sensitive algorithm is needed for better risk stratification in HCM patients.
Area of Science:
- Cardiology
- Clinical Risk Stratification
- Sudden Cardiac Death
Background:
- Hypertrophic cardiomyopathy (HCM) poses a risk of sudden cardiac death (SCD).
- The HCM Risk-SCD model aids in assessing SCD risk but has limitations in identifying all at-risk individuals.
Purpose of the Study:
- To evaluate the real-world performance of the HCM Risk-SCD model.
- To assess the model's accuracy in predicting SCD in a large cohort of consecutive HCM patients.
Main Methods:
- Retrospective analysis of 389 consecutive HCM patients followed from 2004-2021.
- Comparison of HCM Risk-SCD scores between surviving and non-surviving patients.
- Receiver Operating Characteristic (ROC) curve analysis to determine model sensitivity and specificity.
Main Results:
- The mean HCM Risk-SCD score was significantly lower in survivors (1.8%) compared to non-survivors (3.0%).
- The model showed varying sensitivity and specificity across different risk thresholds (e.g., 61% sensitivity/61% specificity at ≥2.0%, 17% sensitivity/99% specificity at ≥6.0%).
- A notable proportion of non-survivors had scores above 6% (19.1%), indicating limitations in risk prediction.
Conclusions:
- The HCM Risk-SCD model is valuable for identifying high-risk HCM patients for SCD.
- The model's sensitivity is insufficient for accurately identifying all patients at risk of SCD.
- Development of a novel risk algorithm with enhanced sensitivity is warranted for improved patient management.
Background:
The hypertrophic cardiomyopathy (HCM) risk- sudden cardiac death (SCD) model provides a convenient tool for determining the risk of SCD in patients with HCM even though some patients with low-risk scores still remain at risk of SCD. Hence, the aim of our study was to assess the performance of HCM Risk-SCD in a large series of consecutive patients with HCM who had been followed up in a tertiary center.
Methods:
The study population consisted of 389 consecutive HCM patients who had been followed up between 2004 and 2021. Demographic and clinical characteristics, estimated 5-year risk using the HCM Risk-SCD model, were compiled, and survival data were collected during follow-up. Patients were divided into 2 groups according to their long-term survival, and HCM risk-SCD scores of these two groups were compared.
Results:
The long-term mortality was observed in 47 patients out of 389 patients in the during a mean follow-up of 55.5 ± 12.7 months. The mean HCM Risk-SCD score of surviving patients was significantly lower than that of non-survivors (1.8% vs. 3.0%, p < .001). The HCM Risk-SCD score was above 6% in nine (2.6%) survivors and nine (19.1%) non-survivors (p < .001). The ROC curve based on the HCM Risk-SCD score had 61% sensitivity and 61% specificity for risk threshold of for 2.0%, 38% sensitivity and 99% specificity a threshold of ≥4%, 17% sensitivity, and 99% specificity for a threshold of ≥6%.
Conclusion:
A new risk algorithm with higher sensitivity is needed, although the HCM risk-SCD model is still quite useful in identifying patients at a high risk for SCD.

