By Wayne Forrest, staff writer

October 31, 2018 --

Tuesday, November 27 | 3:50 p.m.-4:00 p.m. | SSJ03-06 | Room E353A
Simultaneous cardiac FDG-PET/MRI could be a welcome option for the early detection of cardiac involvement in Anderson-Fabry disease and identifying different stages of disease progression, according to researchers from Italy.

Dr. Andrea Ponsiglione from University Federico II in Naples, will present findings from 17 women with Anderson-Fabry disease and normal left ventricular (LV) function. The patients underwent cardiac PET/MRI (Biograph mMR, Siemens Healthineers) after administration of FDG and gadolinium-diethylenetriamine penta-acetic acid (DPTA) for assessment of late gadolinium enhancement assessment, which is an indication of intramyocardial fibrosis. Five patients (29%) showed focal late gadolinium enhancement and were excluded from the final analysis.

Mean MRI T1 values were significantly lower in patients with Anderson-Fabry disease than in control subjects. With PET, seven patients (58%) showed abnormal coefficient of variation values, which suggest a pattern of inflammation. The other five women (42%) showed normal coefficient of variation values with homogeneous FDG uptake. Patients with abnormal coefficient of variation values also showed higher mean T1 values of lateral segments of the mid-LV wall, which also could indicate a correlation between progressive myocyte sphingolipid accumulation and inflammation.

The study shows the potential role of FDG-PET/MRI in the early detection of cardiac involvement in patients with Anderson-Fabry disease and also monitoring disease progression, according to Ponsiglione and colleagues.

Last Updated hh 10/29/2018 2:08:16 PM