Division of Cardiology, Department of Medicine II, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria.
Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria.
J Nucl Cardiol. 2023 Oct;30(5):1810-1821. doi: 10.1007/s12350-023-03205-7. Epub 2023 Feb 28.
Myocardial glycosphingolipid accumulation in patients with Fabry disease (FD) causes biochemical and structural changes. This study aimed to investigate sympathetic innervation in FD using hybrid cardiac positron emission tomography (PET)/magnetic resonance imaging (MRI).
Patients with different stages of Fabry disease were prospectively enrolled to undergo routine CMR at 1.5T, followed by 3T hybrid cardiac PET/MRI with [C]meta-hydroxyephedrine ([11C]mHED). Fourteen patients with either no evidence of cardiac involvement (n = 5), evidence of left ventricular hypertrophy (LVH) (n = 3), or evidence of LVH and fibrosis via late gadolinium enhancement (LGE) (n = 6) were analyzed. Compared to patients without LVH, patients with LVH or LVH and LGE had lower median T1 relaxation times (ms) at 1.5 T (1007 vs. 889 vs. 941 ms, p = 0.003) and 3T (1290 vs. 1172 vs. 1184 p = .014). Myocardial denervation ([11C]mHED retention < 7%·min) was prevalent only in patients with fibrosis, where a total of 16 denervated segments was found in two patients. The respective area of denervation exceeded the area of LGE in both patients (24% vs. 36% and 4% vs. 32%). However, sympathetic innervation defects ([11C]mHED retention ≤ 9%·min) occurred in all study groups. Furthermore, a reduced sympathetic innervation correlated with an increased left ventricular mass (p = .034, rs = - 0.57) and a reduced global longitudinal strain (GLS) (p = 0.023, rs = - 0.6).
Hybrid cardiac PET/MR with [11C]mHED revealed sympathetic innervation defects, accompanied by impaired GLS, in early stages of Fabry disease. However, denervation is only present in patients with advanced stages of FD showing fibrosis on CMR.
法布里病（FD）患者心肌糖脂积累导致生化和结构改变。本研究旨在使用心脏正电子发射断层扫描（PET）/磁共振成像（MRI）混合技术研究 FD 的交感神经支配。
前瞻性纳入不同阶段 FD 患者，在 1.5T 进行常规 CMR，随后在 3T 行心脏 PET/MRI 混合检查，采用[C]间羟麻黄碱([11C]mHED)。分析了 14 名无心脏受累（n=5）、左心室肥厚（LVH）（n=3）或 LVH 合并延迟钆增强（LGE）纤维化（n=6）的患者。与无 LVH 患者相比，LVH 或 LVH 合并 LGE 患者的 1.5T（1007 对 889 对 941ms，p=0.003）和 3T（1290 对 1172 对 1184p=0.014）的 T1 弛豫时间中位数较低。心肌去神经支配（[11C]mHED 保留<7%·min）仅见于纤维化患者，其中 2 例各有 16 个去神经支配节段。在这两名患者中，去神经支配的区域均超过 LGE 区域（24%对 36%和 4%对 32%）。然而，所有研究组均存在交感神经支配缺陷（[11C]mHED 保留≤9%·min）。此外，交感神经支配减少与左心室质量增加（p=0.034，rs=-0.57）和整体纵向应变（GLS）降低（p=0.023，rs=-0.6）相关。
心脏[11C]mHED PET/MRI 混合技术显示，早期 FD 患者存在交感神经支配缺陷，同时伴有 GLS 降低。然而，只有在 CMR 显示纤维化的 FD 晚期患者中才出现去神经支配。
