Validation of the HCM Risk‐SCD model in patients with hypertrophic cardiomyopathy and future perspectives

• Published in: Pacing and clinical electrophysiology Vol. 46; no. 12; pp. 1519 - 1525
• Main Authors: Pay, Levent, Çetin, Tuğba, Dereli, Şeyda, Kadı, Hikmet, Yumurtaş, Ahmet Çağdaş, Çınar, Tufan, Hayıroğlu, Mert İlker
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01.12.2023
• Subjects: Cardiomyopathy; Cardiomyopathy, Hypertrophic - complications; Cardiomyopathy, Hypertrophic - diagnosis; Death, Sudden, Cardiac - epidemiology; HCM risk‐SCD; Humans; hypertrophic cardiomyopathy; ICD; Population studies; Risk Assessment; Risk Factors; sudden cardiac death; Survival; hypertrophic cardiomyopathy; ICD; sudden cardiac death; HCM risk-SCD
• ISSN: 0147-8389; 1540-8159
• DOI: 10.1111/pace.14876

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.