Key result
High-risk DERIVATE 2.0 score linked to ~8-fold higher arrhythmic event risk in non-ischemic cardiomyopathy.
Why the study?
Sudden cardiac death risk stratification in non-ischemic cardiomyopathy remains challenging. This study assessed the accuracy of the recently proposed Derivate Risk Score 2.0 for predicting major arrhythmic events in a Portuguese NICM cohort.
Does the Derivate Risk Score 2.0 accurately predict Major Arrhythmic Cardiac Events (sudden cardiac death, sustained ventricular tachycardia, or appropriate ICD therapy) in a Portuguese cohort of patients with non-ischemic cardiomyopathy?
Cohort (n=385)
No
Does the Derivate Risk Score 2.0 accurately predict Major Arrhythmic Cardiac Events (sudden cardiac death, sustained ventricular tachycardia, or appropriate ICD therapy) in a Portuguese cohort of patients with non-ischemic cardiomyopathy?
The CMR-based DERIVATE Risk Score 2.0 demonstrated robust discrimination and calibration for predicting major arrhythmic events in a Portuguese cohort with non-ischemic cardiomyopathy, effectively identifying high-risk patients who experienced the majority of events.
May aid arrhythmic risk stratification in non-ischemic cardiomyopathy; extends DRS 2.0 validation but requires prospective confirmation.
Introduction and Objectives Sudden cardiac death (SCD) risk stratification in non-ischemic cardiomyopathy (NICM) remains challenging. The recently proposed Derivate Risk Score 2.0 (DRS 2.0) seeks to enhance risk prediction using three variables: sex, cardiac magnetic resonance–derived left ventricular ejection fraction (CMR-LVEF), and the presence of midwall late gadolinium enhancement (LGE). The aim of this study was to assess the accuracy of the DRS 2.0 for predicting Major Arrhythmic Events (MAACE) in a Portuguese NICM cohort. Methods Patients with the clinical diagnosis of NICM who underwent CMR for clinical purposes were included in this single-center retrospective study. Patients with obstructive coronary artery disease, infiltrative, inflammatory or hypertrophic cardiomyopathy, or significant valvular or congenital heart disease were excluded. The DRS 2.0 score was calculated for each patient based on male sex, CMR-derived LVEF and on the presence and pattern of midwall LGE as reported. The primary endpoint was Major Arrhythmic Cardiac Events (MAACE), defined as SCD, sustained ventricular tachycardia (VT), or appropriate ICD therapy (shock or ATP). Follow-up was performed using clinical records and device interrogations. Results A total of 385 patients were enrolled (age 62.5 years (±14.9); 63% male, median CMR-LVEF 37% (27-43), midwall LGE present 51.4%). 102 patients had an ICD (26.5%), 5 had an ICD previous to CMR and the remaining 97 patients received one during follow up. The median DRS2.0 score was 6.0 (IQR 4.0 – 9.0). Risk distribution according to the DRS 2.0 was 20% low risk (n=76), 53% intermediate risk (n=203), and 28% high risk (n=106). During a mean follow up of 3.0 ±1.4 years, 26 MAACE (6.8%) occurred, including 7 SCD, 9 sustained VT, and 10 appropriate ICD therapies. The annualized MAACE rates were 0.4%/year in low risk, 1.0%/year in intermediate-risk, and 6.0%/year in the high- risk patients. Patients classified as high risk by DRS 2.0 represented 28% of the cohort yet accounted for 73% of all MAACE (HR 8.00, 95%CI 3.36-19.04, p<0.001) The DRS 2.0 showed good discrimination (C statistic 0.76, 95%CI: 0.66-0.85, p<0.001). Calibration was excellent, as indicated by a non-significant Hosmer–Lemeshow test (χ² = 0.802, p = 0.371). Conclusions DRS 2.0 showed robust discrimination and calibration in our Portuguese NICM cohort, effectively identifying a small subgroup that accounted for most arrhythmic events. This score appears applicable in our population and may assist in refining SCD risk stratification and management.Figure 1 - DRS 2.0
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Sousa et al. (2026) conducted a cohort in Non-ischemic cardiomyopathy (NICM) (n=385). DERIVATE Risk Score 2.0 (DRS 2.0) high-risk classification vs. Low and intermediate risk classification by DRS 2.0 was evaluated on Major Arrhythmic Cardiac Events (MAACE): sudden cardiac death (SCD), sustained ventricular tachycardia (VT), or appropriate ICD therapy (shock or ATP) (HR 8.00, 95% CI 3.36-19.04, p=<0.001). High-risk classification by the DERIVATE Risk Score 2.0 strongly predicted major arrhythmic events in non-ischemic cardiomyopathy (HR 8.00; 95% CI 3.36-19.04; p<0.001; C-statistic 0.76).
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