Loss of base-to-apex circumferential strain gradient assessed by cardiovascular magnetic resonance in Fabry disease: relationship to T1 mapping, late gadolinium enhancement and hypertrophy
Loss of base-to-apex circumferential strain gradient assessed by cardiovascular magnetic resonance in Fabry disease: relationship to T1 mapping, late gadolinium enhancement and hypertrophy
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DOI：
10.1186/s12968-019-0557-0
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发表时间：
2019-08-01
影响因子：
6.4
通讯作者：
Hanneman, Kate
中科院分区：
文献类型：
--
作者：
Mathur, Shobhit;Dreisbach, John G.;Hanneman, Kate
心脏受累是法布里病（Fabry disease，FD）的常见病，也是FD死亡的主要原因.我们探讨了FD患者心血管磁共振（CMR）心肌应变、T1标测、晚期钆增强（LGE）与左室肥厚（LVH）之间的关系。（45.0 ± 14.5岁，37%男性）和8名健康对照者（40.113.7岁，63%男性）接受了3 T CMR，包括电影平衡稳态自由进动（bSSFP），LGE和改进的Look-See反演恢复（MOLLI）T1映射。结果在FD患者中，有8例LVH（FD LVH+，21%），17例LGE（FD LGE+，45%），其中17例为左室肥厚（FD LGE+，45%）。19例FD患者（50%）既无LVH也无LGE（FD LVH-LGE-）。健康对照组均无LVH或LGE。FD患者和健康对照组在GLS方面无显著差异（-15.3 +/- 3.5% vs. -16.3 +/-1.5%，p=0.45），GCS（-19.4 +/- 3.0% vs. -19.5 +/-2.9%，p=0.84）或基底-心尖LS梯度（7.5 +/- 3.8% vs. 9.3 +/-3.5%，p=0.24）。FD患者的基底-心尖CS梯度（2.1 +/- 3.7% vs. 6.5 +/-2.2%，p=0.002）和自体T1（1170.2 +/- 37.5ms vs. 1239.0 +/- 18.0ms，p
BackgroundCardiac involvement is common and is the leading cause of mortality in Fabry disease (FD). We explored the association between cardiovascular magnetic resonance (CMR) myocardial strain, T1 mapping, late gadolinium enhancement (LGE) and left ventricular hypertrophy (LVH) in patients with FD.MethodsIn this prospective study, 38 FD patients (45.014.5years, 37% male) and 8 healthy controls (40.113.7years, 63% male) underwent 3T CMR including cine balanced steady-state free precession (bSSFP), LGE and modified Look-Locker Inversion recovery (MOLLI) T1 mapping. Global longitudinal (GLS) and circumferential (GCS) strain and base-to-apex longitudinal strain (LS) and circumferential strain (CS) gradients were derived from cine bSSFP images using feature tracking analysis.Results Among FD patients, 8 had LVH (FD LVH+, 21%) and 17 had LGE (FD LGE+, 45%). Nineteen FD patients (50%) had neither LVH nor LGE (FD LVH- LGE-). None of the healthy controls had LVH or LGE. FD patients and healthy controls did not differ significantly with respect to GLS (-15.3 +/- 3.5% vs. -16.3 +/- 1.5%, p=0.45), GCS (-19.4 +/- 3.0% vs. -19.5 +/- 2.9%, p=0.84) or base-to-apex LS gradient (7.5 +/- 3.8% vs. 9.3 +/- 3.5%, p=0.24). FD patients had significantly lower base-to-apex CS gradient (2.1 +/- 3.7% vs. 6.5 +/- 2.2%, p=0.002) and native T1 (1170.2 +/- 37.5ms vs. 1239.0 +/- 18.0ms, p