Role of serial cardiac 18F-FDG PET-MRI in Anderson-Fabry disease: a pilot study.

Abstract

AIM:

We investigated the value of serial cardiac 18F-FDG PET-MRI in Anderson-Fabry disease (AFD) and the potential relationship of imaging results with FASTEX score.

METHODS AND RESULTS:

Thirteen AFD patients underwent cardiac 18F-FDG PET-MRI at baseline and follow-up. Coefficient of variation (COV) of FDG uptake and FASTEX score were assessed. At baseline, 9 patients were enzyme replacement therapy (ERT) naïve and 4 patients were under treatment. Two patients presented a FASTEX score of 0 indicating stable disease and did not show any imaging abnormality at baseline and follow-up PET-MRI. Eleven patients had a FASTEX score > 20% indicating disease worsening. Four of these patients without late gadolinium enhancement (LGE) and with normal COV at baseline and follow-up had a FASTEX score of 35%. Three patients without LGE and with abnormal COV at baseline and follow-up had a FASTEX score ranging from 30 to 70%. Three patients with LGE and abnormal COV at baseline and follow-up had a FASTEX score between 35 and 75%. Finally, one patient with LGE and normal COV had a FASTEX score of 100%. Of the 12 patients on ERT at follow-up, FASTEX score was significantly higher in those 4 showing irreversible cardiac injury at baseline compared to 8 with negative LGE (66 ± 24 vs. 32 ± 21, p = 0.03).

CONCLUSION:

18F-FDG PET-MRI may be effective to monitor cardiac involvement in AFD.

• Authors: Carmela Nappi, Andrea Ponsiglione, Antonio Pisani, Eleonora Riccio, Teodolinda Di Risi, Maurizio Pieroni, Michele Klain, Roberta Assante, Wanda Acampa, Emanuele Nicolai, Letizia Spinelli, Alberto Cuocolo, Massimo Imbriaco
• Journal: Insights into imaging (Insights Imaging) Vol. 12 Issue 1 Pg. 124 (Sep 06 2021) ISSN: 1869-4101 [Print] Germany
• PMID: 34487259 (Publication Type: Journal Article)
• Copyright: © 2021. The Author(s).