1080 EFFECTS OF ENZYME REPLACEMENT THERAPY ON CMR PARAMETERS IN PATIENTS WITH FABRY DISEASE: A SYSTEMATIC REVIEW AND META-ANALYSIS


 
 
 Cardiovascular death is the leading cause of mortality in patients affected by Fabry Disease (FD). Cardiac magnetic resonance (CMR) is the gold-standard imaging modality to assess FD heart involvement and enzyme replacement therapy (ERT) has the potential to slow or revert FD organ involvement and dysfunction. However, the effect of ERT on cardiac disease progression remains undefined. Thus, we performed a systematic review and meta-analysis to assess the impact of ERT on CMR parameters in FD patients.
 
 
 
 Data search was conducted from inception through June 1, 2022, using the following Medical Subject Heading terms: “Fabry Disease” AND (“Cardiac MRI” OR “Cardiac Magnetic Resonance” OR “Cardiovascular Magnetic Resonance” OR “CMR”) AND (“ERT” OR “Enzyme Replacement Therapy”. Studies were included if contemplated FD patients undergoing baseline and follow-up CMR after ERT. Pre-defined study outcomes were the change of i) left ventricular wall mass (LVM; grams); ii) LVM indexed (grams/mq); iii) maximum left ventricular wall thickness (MLVWT, mm), iv) Late gadolinium enhancement (LGE; % of LVM) and v) native-T1 mapping (msec) between baseline and follow-up CMR. The study protocol was registered in the PROSPERO database (CRD42022336223).
 
 
 
 Ten studies including 517 FD patients were included. Between baseline and follow-up CMR, there was a significant decrease in LVM (pooled hazard ratio: -19.5 grams [95% CIs, -34.3; -4.63]), LVM indexed (-2.84 grams/mq [95% CIs, -5.24; -0.44]), MLVWT (-0.96 mm [95% CIs -1.9; -0.02]), and an increase in LGE (0.8% [95% CIs, 0.47; 1.14). T1-mapping did not significantly change (pooled hazard ratio: 6.12 msec [95% CIs -2.39; 14.62]). There was publication bias in LVM (Egger test: p-value = 0.047) but not in LVM index, MLVWT, T1-mapping and LGE.
 
 
 
 In a pooled-analysis including 427 FD patients treated with ERT undergoing baseline and follow-up CMR, there was a significant decrease in LVM, LVM indexed, and LVMWT. In contrast, there was an increase in LGE extent whereas native-T1 mapping values did not significantly change.
