Dipartimento di Scienze Cardiovascolari, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy; Cardiology Department, University Hospital "Santa Maria della Misericordia ", Azienda Sanitaria Universitaria Integrata Friuli Centrale, Udine, Italy.
Istituto di Cardiologia, Università Cattolica del Sacro Cuore, Rome, Italy.
Int J Cardiol. 2024 Dec 1;416:132489. doi: 10.1016/j.ijcard.2024.132489. Epub 2024 Aug 24.
A wide variety of non-invasive and invasive techniques for SCD risk stratification in non ischemic cardiomyopathy (NICM) have been proposed, including left ventricular (LV) ejection fraction, QRS duration, late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) and invasive electrophysiologic study with or without three-dimensional electroanatomic mapping (3D-EAM), to identify and characterize the arrhythmogenic substrate. There is still no clear consensus on the risk stratification in this clinical setting. The aim of our study is to characterize the 3D-EAM substrate in patients with the same clinical presentation of unexplained complex VAs and NICM using CMR, three-dimensional electranatomic mapping (3D-EAM) in association with endomyocardial biopsy (EMB) and genetic screening, as a more precise and early diagnostic assessment may provide important subsequent prognostic impact. The study was designed as a prospective multi-center observational evaluation and the patient follow-up was scheduled at 6 months interval. We enrolled 125 patients distinct into four different group by complete diagnostic work-up: myocarditis, non-dilated left ventricular cardiomyopathy, arrhythmogenic cardiomyopathy and control group. The four groups were compared in terms of clinical, imaging and 3D-EAM data. At multivariate analysis sustained VT/VF on admission [HR: 3.64 (1.79-7.4), p < 0.001], total bipolar scar area of left and right ventricle detected by 3D-EAM [HR: 2.24 (1.13-4.49), p = 0.02], histological diagnosis of myocarditis by 3D-EAM guided endomyocardial biopsy (EBM) [HR: 2.79 (1.04-7.44), p = 0.01] were independent predictors of complex VAs or death at follow-up. 3D-EAM guided EMB represent not only a valid diagnostic tool to identify the arrhythmogenic substrate in patients with NICM and ventricular arrhythmic phenotype but also an important predictor of complex Vas at long term follow-up.
已经提出了多种用于非缺血性心肌病（NICM）中 SCD 风险分层的非侵入性和侵入性技术，包括左心室（LV）射血分数、QRS 持续时间、心脏磁共振（CMR）上的晚期钆增强（LGE）和有或没有三维电解剖图（3D-EAM）的侵入性电生理研究，以识别和描述心律失常基质。在这种临床情况下，风险分层仍然没有明确的共识。我们的研究目的是使用 CMR 、三维电解剖图（3D-EAM）结合心内膜心肌活检（EMB）和基因筛查，对具有相同不明原因复杂 VA 和 NICM 临床表现的患者的 3D-EAM 基质进行特征描述，因为更精确和早期的诊断评估可能会提供重要的后续预后影响。该研究设计为前瞻性多中心观察性评估，患者随访间隔为 6 个月。我们通过完整的诊断工作将 125 名患者分为四个不同的组：心肌炎、非扩张性左心室心肌病、致心律失常性心肌病和对照组。比较了这四组患者的临床、影像学和 3D-EAM 数据。多变量分析显示，入院时持续性 VT/VF [HR：3.64（1.79-7.4），p<0.001]、3D-EAM 检测到的左、右心室总双极瘢痕面积 [HR：2.24（1.13-4.49），p=0.02]、3D-EAM 引导下心内膜心肌活检（EBM）的心肌炎组织学诊断 [HR：2.79（1.04-7.44），p=0.01]是随访时复杂 VA 或死亡的独立预测因子。3D-EAM 引导的 EMB 不仅是识别 NICM 和室性心律失常表型患者心律失常基质的有效诊断工具，也是长期随访时复杂 Vas 的重要预测因子。
