Department of Cardiology, West China Hospital, Sichuan University, Chengdu, 610041, Sichuan Province, China.
Department of Cardiovascular Surgery/Pediatric Heart Center, West China Hospital, Sichuan University, Chengdu, 610041, Sichuan Province, China.
J Cardiovasc Magn Reson. 2018 Sep 27;20(1):69. doi: 10.1186/s12968-018-0488-1.
Myocardial fibrosis is a common pathophysiological process that is related to ventricular remodeling in congenital heart disease. However, the presence, characteristics, and clinical significance of myocardial fibrosis in Ebstein's anomaly have not been fully investigated. This study aimed to evaluate myocardial fibrosis using cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) and T1 mapping techniques, and to explore the significance of myocardial fibrosis in adolescent and adult patients with Ebstein's anomaly.
Forty-four consecutive patients with unrepaired Ebstein's anomaly (34.0 ± 16.2 years; 18 males), and an equal number of age- and gender-matched controls, were included. A comprehensive CMR protocol consisted of cine, LGE, and T1 mapping by modified Look-Locker inversion recovery (MOLLI) sequences were performed. Ventricular functional parameters, native T1, extracellular volume (ECV), and LGE were analyzed. Associations between myocardial fibrosis and disease severity, ventricular function, and NYHA classification were analyzed.
LGE was found in 10 (22.7%) patients. Typical LGE in Ebstein's anomaly was located in the endocardium of the septum within the right ventricle (RV). The LV ECV of Ebstein's anomaly were significantly higher than those of the controls (30.0 ± 3.8% vs. 25.3 ± 2.3%, P < 0.001). An increased ECV was found to be independent of the existence of LGE. Positive LGE or higher ECV (≥30%) was associated with larger fRV volume, aRV volume, increased disease severity, and worse NYHA functional class. In addition, ECV was significantly correlated with the LV ejection fraction (P < 0.001).
Both focal and diffuse myocardial fibrosis were observed in adolescent and adult patients with Ebstein's anomaly. Increased diffuse fibrosis is associated with worse LV function, increased Ebstein's severity, and worse clinical status.
心肌纤维化是一种常见的病理生理过程，与先天性心脏病的心室重构有关。然而，Ebstein 异常中的心肌纤维化的存在、特征和临床意义尚未得到充分研究。本研究旨在使用心血管磁共振（CMR）晚期钆增强（LGE）和 T1 映射技术评估心肌纤维化，并探讨心肌纤维化在青少年和成年 Ebstein 异常患者中的意义。
纳入 44 例未经修复的 Ebstein 异常患者（34.0 ± 16.2 岁；18 名男性）和相同数量的年龄和性别匹配的对照组。进行了包括电影、LGE 和通过改良 Look-Locker 反转恢复（MOLLI）序列的 T1 映射的综合 CMR 方案。分析了心室功能参数、原生 T1、细胞外容积（ECV）和 LGE。分析了心肌纤维化与疾病严重程度、心室功能和 NYHA 分类之间的相关性。
10 例（22.7%）患者发现 LGE。Ebstein 异常中的典型 LGE 位于右心室（RV）的间隔心内膜内。Ebstein 异常的 LV ECV 明显高于对照组（30.0 ± 3.8%比 25.3 ± 2.3%，P < 0.001）。发现增加的 ECV 独立于 LGE 的存在。阳性 LGE 或更高的 ECV（≥30%）与更大的 fRV 容积、aRV 容积、增加的疾病严重程度和更差的 NYHA 功能分级相关。此外，ECV 与 LV 射血分数显著相关（P < 0.001）。
在青少年和成年 Ebstein 异常患者中观察到局灶性和弥漫性心肌纤维化。弥漫性纤维化增加与 LV 功能恶化、Ebstein 严重程度增加和临床状况恶化相关。
