Zhuang Baiyan, Li Shuang, Zhang Hongkai, Sun Zhonghua, Wang Hui, Xu Lei
Department of Radiology, Beijing Anzhen Hospital, Beijing Institute of Heart, Lung, and Blood Vessel Diseases, Capital Medical University, Beijing, China.
Medical Radiation Science, Discipline of Medical Radiation Science, Curtin Medical School, Curtin University, Perth, Western Australia, Australia.
Open Heart. 2025 Jun 18;12(1):e003441. doi: 10.1136/openhrt-2025-003441.
Non-dilated left ventricular cardiomyopathy (NDLVC) is a novel cardiomyopathy characterised by normal LV size and non-ischaemic myocardial scarring or fatty tissue replacement. This study aimed to explore the clinical and cardiac magnetic resonance (CMR) characteristics of NDLVC compared with dilated cardiomyopathy (DCM) and event rates of patients with NDLVC and reduced LV ejection fraction (NDLVC-REF).
A retrospective cohort study of 396 patients, including 210 with NDLVC (135 classified as NDLVC-REF) and 186 with DCM, who underwent CMR imaging between 2015 and 2017, was conducted. Follow-up lasted until May 2024, with a composite endpoint of major adverse cardiovascular events (only NDLVC patients were followed).
NDLVC patients exhibited better cardiac function than those with DCM, with higher LVEF (40.1%±15.8% vs 23.3%±8.8%, p<0.001). Compared with DCM, the presence of late gadolinium enhancement (LGE) was lower in the NDLVC group (77.4% vs 64.8%, p<0.001). NDLVC-REF showed a comparable prevalence of LGE presence with DCM (70.4% vs 77.4%, p=0.06) but lower LGE mass (4.8 (0, 9.9) g vs 6.8 (4.0, 11.0) g, p=0.01). Over a median follow-up of 83 months, 62 patients with NDLVC (29.5%) reached the composite endpoint. Multivariable analyses (forward logistic regression) identified right ventricular ejection fraction (RVEF) (0.98 (0.96, 0.99), p=0.01, Harrell's C-index=0.65) as the significant predictor of adverse outcomes in NDLVC. The presence of epicardium-involved LGE, left atrial volume index, LVEF, global radial strain and global circumferential strain was also associated with adverse events in NDLVC. In NDLVC-REF, RVEF <40% independently predicted major adverse cardiovascular events (2.19 (1.14-4.20), p=0.01).
The clinical baseline and CMR parameters of NDLVC were different from those of DCM. RVEF was a powerful predictor of adverse events in NDLVC and NDLVC-REF.
非扩张型左室心肌病（NDLVC）是一种新型心肌病，其特征为左室大小正常、无缺血性心肌瘢痕形成或脂肪组织替代。本研究旨在探讨NDLVC与扩张型心肌病（DCM）相比的临床和心脏磁共振（CMR）特征，以及NDLVC伴左室射血分数降低（NDLVC-REF）患者的事件发生率。
对396例患者进行回顾性队列研究，其中包括210例NDLVC患者（135例分类为NDLVC-REF）和186例DCM患者，这些患者在2015年至2017年间接受了CMR成像检查。随访持续至2024年5月，主要不良心血管事件为复合终点（仅对NDLVC患者进行随访）。
NDLVC患者的心脏功能优于DCM患者，左室射血分数更高（40.1%±15.8%对23.3%±8.8%，p<0.001）。与DCM相比，NDLVC组钆延迟增强（LGE）的发生率更低（77.�%对64.8%，p<0.001）。NDLVC-REF的LGE发生率与DCM相当（70.4%对77.4%，p=0.06），但LGE质量更低（4.8（0，9.9）g对6.8（4.0，11.0）g，p=0.01）。在中位随访83个月期间，62例NDLVC患者（29.5%）达到复合终点。多变量分析（向前逻辑回归）确定右室射血分数（RVEF）（0.98（0.96，0.99），p=0.01，Harrell氏C指数=0.65）是NDLVC不良结局的显著预测因子。累及心外膜的LGE、左房容积指数、LVEF、整体径向应变和整体圆周应变的存在也与NDLVC的不良事件相关。在NDLVC-REF中，RVEF<40%独立预测主要不良心血管事件（2.19（1.14-4.20），p=0.01）。
NDLVC的临床基线和CMR参数与DCM不同。RVEF是NDLVC和NDLVC-REF不良事件的有力预测因子。
