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

Constantin Gatterer, Tim Wollenweber, Verena Pichler, Chrysoula Vraka, Gere Sunder-Plassmann, Max Lenz, Christian Hengstenberg, Marcus Hacker, Christian Loewe, Senta Graf, Dietrich Beitzke (Corresponding author)

Publications: Contribution to journalArticlePeer Reviewed

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 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). Conclusion: 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.

Original languageEnglish
Pages (from-to)1810–1821
Number of pages12
JournalJournal of Nuclear Cardiology
Volume30
Issue number5
Early online date28 Feb 2023
DOIs
Publication statusPublished - Oct 2023

Austrian Fields of Science 2012

  • 302054 Nuclear medicine

Keywords

  • cardiomyopathy
  • Fabry disease
  • hybrid imaging
  • sympathetic denervation
  • [C]mHED

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