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Performance of the DERIVATE Risk Score 2.0 for predicting major arrhythmic events in non-ischemic cardiomyopathy: A portuguese validation study
Session:
Prémio Jovem Investigador
Speaker:
francisco sousa
Congress:
CPC 2026
Topic:
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Theme:
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Subtheme:
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Session Type:
Sessão de Prémios
FP Number:
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Authors:
Francisco Sousa; Catarina Santos- Jorge; Pedro Freitas; Pedro M. Lopes; Sara Guerreiro; João Abecasis; Francisco Gama; Cláudia Silva; Rita Dos Reis Santos; Sérgio Maltez; Bruno Rocha; António Miguel Ferreira
Abstract
<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong>Introduction: </strong></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">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). </span></span><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">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.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong>Methods: </strong></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">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. </span></span><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">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. </span></span><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">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. </span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong>Results</strong></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">A total of 385 patients were enrolled (age 62.5 years (±14.9); 63% male, mean CMR-LVEF 34.5% ± 10.7, 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. </span></span><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">The median DRS2.0 score was 2.0 (IQR 2.0). 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). </span></span><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">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 6.7%/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). </span></span><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">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).</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong>Conclusion: </strong></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">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.</span></span></p>
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