应用心血管磁共振钆延迟增强及 T1 mapping 技术检测及评估艾森曼格综合征患者心肌纤维化
Detection and evaluation of myocardial fibrosis in Eisenmenger syndrome using cardiovascular magnetic resonance late gadolinium enhancement and T1 mapping.
机构信息
Cardiology Division, Department of Cardiology, West China Hospital, Sichuan University, Guoxue Xiang No. 37, Chengdu, Sichuan Province, 610041, People's Republic of China.
Department of Cardiology, The Second People's Hospital of Yibin, Yibin, Sichuan, 610041, People's Republic of China.
出版信息
J Cardiovasc Magn Reson. 2022 Nov 21;24(1):60. doi: 10.1186/s12968-022-00880-2.
BACKGROUND
Myocardial fibrosis is a common pathophysiological process involved in many cardiovascular diseases. However, limited prior studies suggested no association between focal myocardial fibrosis detected by cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) and disease severity in Eisenmenger syndrome (ES). This study aimed to explore potential associations between myocardial fibrosis evaluated by the CMR LGE and T1 mapping and risk stratification profiles including exercise tolerance, serum biomarkers, hemodynamics, and right ventricular (RV) function in these patients.
METHODS
Forty-five adults with ES and 30 healthy subjects were included. All subjects underwent a contrast-enhanced 3T CMR. Focal replacement fibrosis was visualized on LGE images. The locations of LGE were recorded. After excluding LGE in ventricular insertion point (VIP), ES patients were divided into myocardial LGE-positive (LGE) and LGE-negative (LGE) subgroups. Regions of interest in the septal myocardium were manually contoured in the T1 mapping images to determine the diffuse myocardial fibrosis. The relationships between myocardial fibrosis and 6-min walk test (6MWT), N-terminal pro-brain natriuretic peptide (NT-pro BNP), hematocrit, mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance index (PVRI), RV/left ventricular end-systolic volume (RV/LV ESV), RV ejection fraction (RVEF), and risk stratification were analyzed.
RESULTS
Myocardial LGE (excluding VIP) was common in ES (16/45, 35.6%), and often located in the septum (12/45, 26.7%). The clinical characteristics, hemodynamics, CMR morphology and function, and extracellular volume fraction (ECV) were similar in the LGE and LGE groups (all P > 0.05). ECV was significantly higher in ES patients (28.6 ± 5.9% vs. 25.6 ± 2.2%, P < 0.05) and those with LGE ES (28.3 ± 5.9% vs. 25.6 ± 2.2%, P < 0.05) than healthy controls. We found significant correlations between ECV and log NT-pro BNP, hematocrit, mPAP, PVRI, RV/LV ESV, and RVEF (all P < 0.05), and correlations trends between ECV and 6MWT (P = 0.06) in ES patients. An ECV threshold of 29.0% performed well in differentiating patients with high-risk ES from those with intermediate or low risk (area under curve 0.857, P < 0.001).
CONCLUSIONS
Myocardial fibrosis is a common feature of ES. ECV may serve as an important imaging marker for ES disease severity.
背景
心肌纤维化是多种心血管疾病共同的病理生理过程。然而，先前的有限研究表明，心血管磁共振（CMR）晚期钆增强（LGE）检测到的局灶性心肌纤维化与艾森曼格综合征（ES）的疾病严重程度之间没有关联。本研究旨在探讨 CMR LGE 和 T1 映射评估的心肌纤维化与运动耐量、血清生物标志物、血液动力学和右心室（RV）功能等风险分层特征之间的潜在关联。
方法
纳入 45 例 ES 患者和 30 例健康对照者。所有受试者均行对比增强 3T CMR 检查。LGE 图像上显示局灶性替代纤维化。记录 LGE 的位置。排除心室插入点（VIP）的 LGE 后，ES 患者被分为心肌 LGE 阳性（LGE）和 LGE 阴性（LGE）亚组。T1 映射图像中手动勾画间隔心肌的感兴趣区以确定弥漫性心肌纤维化。分析心肌纤维化与 6 分钟步行试验（6MWT）、N 末端脑利钠肽前体（NT-pro BNP）、红细胞压积、平均肺动脉压（mPAP）、肺血管阻力指数（PVRI）、RV/左心室收缩末期容积（RV/LV ESV）、RV 射血分数（RVEF）和风险分层之间的关系。
结果
ES 中局灶性心肌 LGE（不包括 VIP）较常见（16/45，35.6%），常位于间隔（12/45，26.7%）。LGE 和 LGE 组的临床特征、血液动力学、CMR 形态和功能以及细胞外容积分数（ECV）相似（均 P>0.05）。ES 患者（28.6±5.9% vs. 25.6±2.2%，P<0.05）和 LGE ES 患者（28.3±5.9% vs. 25.6±2.2%，P<0.05）的 ECV 明显高于健康对照组。我们发现 ECV 与 log NT-pro BNP、红细胞压积、mPAP、PVRI、RV/LV ESV 和 RVEF 之间存在显著相关性（均 P<0.05），并发现 ECV 与 ES 患者 6MWT 之间存在相关性趋势（P=0.06）。ECV 阈值为 29.0%时，可很好地区分高危 ES 患者与中危或低危 ES 患者（曲线下面积 0.857，P<0.001）。
结论
心肌纤维化是 ES 的一个常见特征。ECV 可能是 ES 疾病严重程度的重要影像学标志物。
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