当前位置: X-MOL 学术Circ. Cardiovasc. Imaging › 论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
Quantitative Myocardial Perfusion in Fabry Disease.
Circulation: Cardiovascular Imaging ( IF 7.5 ) Pub Date : 2019-07-04 , DOI: 10.1161/circimaging.119.008872
Kristopher D Knott 1, 2 , Joao B Augusto 1, 2 , Sabrina Nordin 1, 2 , Rebecca Kozor 3 , Claudia Camaioni 2 , Hui Xue 4 , Rebecca K Hughes 1, 2 , Charlotte Manisty 1, 2 , Louise A E Brown 5 , Peter Kellman 4 , Uma Ramaswami 6 , Derralyn Hughes 6 , Sven Plein 5 , James C Moon 1, 2
Affiliation  

BACKGROUND Fabry disease (FD) is an X-linked lysosomal storage disease resulting in tissue accumulation of sphingolipids. Key myocardial processes that lead to adverse outcomes in FD include storage, hypertrophy, inflammation, and fibrosis. These are quantifiable by multiparametric cardiovascular magnetic resonance. Recent developments in cardiovascular magnetic resonance perfusion mapping allow rapid in-line perfusion quantification permitting broader clinical application, including the assessment of microvascular dysfunction. We hypothesized that microvascular dysfunction in FD would be associated with storage, fibrosis, and edema. METHODS A prospective, observational study of 44 FD patients (49 years, 43% male, 24 [55%] with left ventricular hypertrophy [LVH]) and 27 healthy controls with multiparametric cardiovascular magnetic resonance including vasodilator stress perfusion mapping. Myocardial blood flow (MBF) was measured and its associations with other processes investigated. RESULTS Compared with LVH- FD, LVH+ FD had higher left ventricular ejection fraction (73% versus 68%), more late gadolinium enhancement (85% versus 15%), and a lower stress MBF (1.76 versus 2.36 mL/g per minute). The reduction in stress MBF was more pronounced in the subendocardium than subepicardium. LVH- FD had lower stress MBF than controls (2.36 versus 3.00 mL/g per minute; P=0.002). Across all FD, late gadolinium enhancement and low native T1 were independently associated with reduced stress MBF. On a per-segment basis, stress MBF was independently associated with wall thickness, T2, extracellular volume fraction, and late gadolinium enhancement. CONCLUSIONS FD patients have reduced perfusion, particularly in the subendocardium with greater reductions with LVH, storage, edema, and scar. Perfusion is reduced even without LVH suggesting it is an early disease marker.

中文翻译:

法布里病中的定量心肌灌注。

背景法布里病(FD)是一种导致鞘脂组织积聚的X连锁溶酶体贮积病。导致 FD 不良结果的关键心肌过程包括储存、肥大、炎症和纤维化。这些可以通过多参数心血管磁共振来量化。心血管磁共振灌注图的最新发展允许快速在线灌注量化,从而允许更广泛的临床应用,包括评估微血管功能障碍。我们假设 FD 中的微血管功能障碍与储存、纤维化和水肿有关。方法 对 44 名 FD 患者(49 岁,43% 男性,24 名 [55%] 左心室肥大 [LVH])和 27 名健康对照者进行多参数心血管磁共振,包括血管扩张剂应力灌注图。测量心肌血流量 (MBF) 并调查其与其他过程的关联。结果 与 LVH-FD 相比,LVH+ FD 具有更高的左心室射血分数(73% 对 68%),更晚的钆增强(85% 对 15%)和更低的应力 MBF(1.76 对 2.36 mL/g/分钟) . 心内膜下应力 MBF 的降低比心外膜下更明显。LVH-FD 的应激 MBF 低于对照组(2.36 对 3.00 mL/g/分钟;P=0.002)。在所有 FD 中,晚期钆增强和低原生 T1 与应力 MBF 降低独立相关。在每个细分市场的基础上,应力 MBF 与壁厚、T2、细胞外体积分数和晚期钆增强独立相关。结论 FD 患者的灌注减少,尤其是心内膜下层,LVH、贮积、水肿和瘢痕减少幅度更大。即使没有 LVH,灌注也会减少,这表明它是早期疾病标志物。
更新日期:2019-07-05
down
wechat
bug