Parisi, VandaGraziosi, MaddalenaLopes, Luis R.Luca, Antonio DePasquale, FerdinandoTini, GiacomoTargetti, MattiaCueto, Maria R.Moura, Ana R.Ditaranto, RaffaelloTorlasco, CamillaTaglieri, NevioNardi, ElenaLovato, LuigiAugusto, João B.Galiè, NazzarenoCrotti, LiaGasperetti, AlessioBiffi, MauroAutore, CamilloMerlo, MarcoOlivotto, IacopoSinagra, GianfrancoElliott, Perry M.Biagini, Elena2024-11-262024-11-262024-11-142047-4873http://hdl.handle.net/10400.14/47296Aims Left ventricular (LV) ring-like scar on cardiac magnetic resonance (CMR) has been linked to malignant arrhythmias in patients with non-ischaemic cardiomyopathy. This study aimed to perform a comprehensive evaluation of this phenotype and to identify risk factors for life-threatening arrhythmic events (LAEs), a composite of sudden cardiac death (SCD), aborted SCD, and sustained ventricular tachycardia. Methods and results One hundred and fifteen patients [median age 39 (interquartile range, IQR, 28–52), 42% females] were identified at 6 referral centres. Inclusion criteria were ring-like LV scar [≥3 contiguous segments with sub-epicardial/midwall late gadolinium enhancement (LGE) in the same slice] and one among: pathogenic/likely pathogenic genetic variant, family history for cardiomyopathy, or arrhythmogenic cardiomyopathy diagnosis. During the study follow-up, survival free from LAEs was 60% (3.8 events/100 patients/year); at a median follow-up of 4.6 years (IQR 1.7–8.4) it was 84%. On multivariable analysis, anterior Q waves [hazard ratio (HR): 1.030, 95% confidence intervals (CI): 1.014–1.046, P < 0.001], QRS width (HR: 4.642, 95% CI: 1.296–16.628, P = 0.018), and LV end-diastolic volume index (LVEDVi; HR: 1.011, 95% CI: 1.001–1.021, per mL/m2 increase, P = 0.040) were independently associated with LAEs; with good discrimination power (Harrell’s C-index = 0.796). Three risk categories were identified: normal electrocardiogram (ECG), abnormal ECG and no LAEs predictive variables, abnormal ECG and ≥1 LAEs predictive variables, with a decreasing survival from 100 to 65% and 49%, respectively (Log-rank test = 0.015). Conclusion In this study, the LV ring-like scar phenotype was associated with a high rate of malignant arrhythmias in presence of anterior Q waves, QRS prolongation, and increased LVEDVi. A normal ECG identified a lower risk sub-group.engArrhythmiasCardiac magnetic resonanceCardiomyopathyElectrocardiogramPrognosisRing-like scarRisk stratificationjournal article10.1093/eurjpc/zwae35339486037001354075800001