Leo Isabella, Dellegrottaglie Santo, Scatteia Alessandra, Torella Daniele, Abete Raffaele, Aquaro Giovanni Donato, Baggiano Andrea, Barison Andrea, Bogaert Jan, Calo' Leonardo, Camastra Giovanni, Carigi Samuela, Carrabba Nazario, Casavecchia Grazia, Censi Stefano, Cicala Gloria, De Cecco Carlo N, De Lazzari Manuel, Di Giovine Gabriella, Dobrovie Monica, Focardi Marta, Fusini Laura, Gaibazzi Nicola, Gismondi Annalaura, Gravina Matteo, Guglielmo Marco, Lanzillo Chiara, Lombardi Massimo, Lorenzoni Valentina, Lozano-Torres Jordi, Margonato Davide, Martini Chiara, Marzo Francesca, Masci Pier-Giorgio, Masi Ambra, Moro Claudio, Muscogiuri Giuseppe, Mushtaq Saima, Nese Alberto, Palumbo Alessandro, Pavon Anna Giulia, Pedrotti Patrizia, Perazzolo Marra Martina, Pradella Silvia, Presicci Cristina, Rabbat Mark G, Raineri Claudia, Rodriguez-Palomares Jose' F, Sbarbati Stefano, Schoepf U Joseph, Squeri Angelo, Sverzellati Nicola, Symons Rolf, Tat Emily, Timpani Mauro, Todiere Giancarlo, Valentini Adele, Varga-Szemes Akos, Volpe Alessandra, Guaricci Andrea Igoren, Schwitter Juerg, Pontone Gianluca
Advanced Cardiovascular Imaging Unit, Clinica Villa dei Fiori, Acerra (Naples), Italy.
Department of Experimental and Clinical Medicine, Magna Graecia University, Catanzaro, Italy.
Eur Heart J Cardiovasc Imaging. 2025 Feb 3. doi: 10.1093/ehjci/jeaf043.
Accurate risk stratification for patients with non-dilated left ventricular cardiomyopathy (NDLVC) remains challenging due to lack of dedicated clinical trials. This post-hoc analysis aims to delineate the arrhythmic risk and assess the incremental value of cardiac magnetic resonance (CMR) imaging in the DERIVATE (CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DebrillAtor ThErapy) study cohort meeting the NDLVC diagnostic criteria.
Patients with NDLVC from DERIVATE registry were identified in the absence of left ventricular (LV) dilatation and in the presence of non-ischaemic LV scarring ("fibrotic NDLVC") or isolated LV systolic dysfunction (LV ejection fraction <50%) without fibrosis ("hypokinetic NDLVC"). The primary endpoint was all-cause mortality. Major adverse arrhythmic cardiac events (MAACE) were the secondary endpoint and included sudden cardiac death (SCD) and aborted SCD.
One hundred ninety-seven NDLVC patients were identified from the cohort of the DERIVATE study (Mean age: 59±14 years; Male: 135). Over a median follow-up of 2.7 years, 15 (8%) patients died, and 8 (4%) experienced MAACE. Patients with "hypokinetic" NDLVC had significantly lower rates of MAACE than non-ischaemic dilated cardiomyopathy (NIDCM) (p=0.001), while patients with "fibrotic" NDLVC had same rate of both primary (p=0.48) and secondary endpoints (p= 0.616) compared to NIDMC patients. Multivariable analysis identified LGE with midwall distribution as an independent predictor of MAACE in NDLVC patients (Hazard Ratio 6.7, 95% Confidence Interval: 1.33-33.67; p=0.021).
NDLVC patients exhibit a heterogeneous risk profile for arrhythmic events. The presence of midwall LGE, similarly to NIDCM, is a significant predictor of MAACE, highlighting the importance of CMR imaging for risk stratification.
由于缺乏专门的临床试验，对非扩张型左心室心肌病（NDLVC）患者进行准确的风险分层仍然具有挑战性。这项事后分析旨在在DERIVATE（用于一级预防植入式心脏复律除颤器治疗的心脏磁共振）研究队列中确定符合NDLVC诊断标准的患者的心律失常风险，并评估心脏磁共振（CMR）成像的增量价值。
从DERIVATE注册研究中识别出无左心室（LV）扩张且存在非缺血性LV瘢痕（“纤维化NDLVC”）或无纤维化的孤立LV收缩功能障碍（LV射血分数<50%）（“运动减弱NDLVC”）的NDLVC患者。主要终点是全因死亡率。主要不良心律失常心脏事件（MAACE）是次要终点，包括心源性猝死（SCD）和未遂SCD。
从DERIVATE研究队列中识别出197例NDLVC患者（平均年龄：59±14岁；男性：135例）。在中位随访2.7年期间，15例（8%）患者死亡，8例（4%）发生MAACE。“运动减弱”型NDLVC患者的MAACE发生率显著低于非缺血性扩张型心肌病（NIDCM）（p=0.001），而“纤维化”型NDLVC患者的主要终点（p=0.48）和次要终点发生率（p=0.616）与NIDCM患者相同。多变量分析确定，中层心肌分布的延迟强化（LGE）是NDLVC患者MAACE的独立预测因素（风险比6.7，95%置信区间：1.33-33.67；p=0.021）。
NDLVC患者心律失常事件的风险特征存在异质性。中层心肌LGE的存在与NIDCM一样，是MAACE的重要预测因素，突出了CMR成像在风险分层中的重要性。
