Detection of sympathetic denervation defects in Fabry disease by hybrid [¹¹C]meta-hydroxyephedrine positron emission tomography and cardiac magnetic resonance.

ABSTRACT:

Background

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).

Methods and results

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 [¹¹C]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 L

SUBMITTER: Gatterer C 

PROVIDER: S-EPMC10558396 | biostudies-literature | 2023 Oct

REPOSITORIES: biostudies-literature