Parisi Vanda, Graziosi Maddalena, Lopes Luis R, De Luca Antonio, Pasquale Ferdinando, Tini Giacomo, Targetti Mattia, Cueto Maria R, Moura Ana R, Ditaranto Raffaello, Torlasco Camilla, Taglieri Nevio, Nardi Elena, Lovato Luigi, Augusto João B, Galiè Nazzareno, Crotti Lia, Gasperetti Alessio, Biffi Mauro, Autore Camillo, Merlo Marco, Olivotto Iacopo, Sinagra Gianfranco, Elliott Perry M, Biagini Elena
Cardiology Unit, Cardiac Thoracic and Vascular Department, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.
Department of Medical and Surgical Sciences (DIMEC), University of Bologna, Bologna, Italy.
Eur J Prev Cardiol. 2024 Nov 1. doi: 10.1093/eurjpc/zwae353.
Left ventricular (LV) ring-like scar on cardiac magnetic resonance (CMR) has been linked to malignant arrhythmias in patients with non-ischemic 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.
One-hundred-fifteen patients (median age 39 [IQR 28-52], 42% females) were identified at 6 referral centres. Inclusion criteria were ring-like LV scar (≥ 3 contiguous segments with subepicardial/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 (HR:1.030, 95% 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 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).
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 subgroup.
心脏磁共振成像(CMR)显示的左心室(LV)环状瘢痕与非缺血性心肌病患者的恶性心律失常有关。本研究旨在对该表型进行全面评估,并确定危及生命的心律失常事件(LAEs)的危险因素,LAEs是心脏性猝死(SCD)、心脏性猝死未遂和持续性室性心动过速的综合指标。
在6个转诊中心共纳入115例患者(中位年龄39岁[四分位间距28 - 52岁],42%为女性)。纳入标准为LV环状瘢痕(同一层面≥3个连续节段伴有心外膜下/中层延迟钆增强(LGE))以及以下情况之一:致病/可能致病的基因变异、心肌病家族史或致心律失常性心肌病诊断。在研究随访期间,无LAEs的生存率为60%(3.8次事件/100例患者/年);中位随访4.6年(四分位间距1.7 - 8.4年)时为84%。多变量分析显示,前Q波(HR:1.030,95%CI:1.014 - 1.046,p < 0.001)、QRS波宽度(HR:4.642,95%CI:1.296 - 16.628,p = 0.018)和左心室舒张末期容积指数(LVEDVi)(HR:1.011,95%CI:1.001 - 1.021,每增加1 mL/m²,p = 0.040)与LAEs独立相关;具有良好的鉴别能力(Harrell氏C指数 = 0.796)。确定了三个风险类别:正常心电图、心电图异常且无LAEs预测变量、心电图异常且有≥1个LAEs预测变量,生存率分别从100%降至65%和49%(对数秩检验 = 0.015)。
在本研究中,LV环状瘢痕表型与存在前Q波、QRS波延长和LVEDVi增加时的高恶性心律失常发生率相关。正常心电图确定了一个低风险亚组。