TY - JOUR
T1 - Imaging predictors of adverse prognosis in Fabry disease cardiomyopathy
T2 - A systematic review and meta-analysis
AU - Stankowski, Kamil
AU - Figliozzi, Stefano
AU - Rojanathagoon, Thanakorn
AU - Bampatsias, Dimitrios
AU - Klettas, Dimitrios
AU - Monti, Lorenzo
AU - Bragato, Renato
AU - Masci, Pier-Giorgio
AU - Francone, Marco
AU - Condorelli, Gianluigi
AU - Imbriaco, Massimo
AU - Pieroni, Maurizio
AU - Camporeale, Antonia
AU - Georgiopoulos, Georgios
N1 - Publisher Copyright:
© 2025 The Author(s). European Journal of Clinical Investigation published by John Wiley & Sons Ltd on behalf of Stichting European Society for Clinical Investigation Journal Foundation.
PY - 2025/5
Y1 - 2025/5
N2 - BACKGROUND: Cardiac involvement represents the main cause of death in patients with Fabry disease (FD). Echocardiography and cardiovascular magnetic resonance (CMR) have an established diagnostic role, but their prognostic value remains unresolved. This systematic review and meta-analysis sought to assess the prognostic implications of imaging parameters in FD.METHODS: PubMed, ClinicalTrials.gov, Embase, Cochrane Library and Web of Science databases were searched for studies from inception through 1 May 2024. Studies including FD patients undergoing baseline imaging assessment and clinical follow-up were selected. Pre-defined study outcomes were a cardiovascular endpoint and a composite clinical endpoint. The study protocol was registered in PROSPERO (ID CRD42022342394).RESULTS: Fourteen studies, including 1713 FD patients (44.7% males), were selected. At pooled analysis, late gadolinium enhancement (hazard ratio [HR]: 4.45; 95% CI: 2.82-7.02), left atrium volume indexed (HR: 1.02 per mL/m
2; 95% CI: 1.01-1.03), E/e' (HR: 1.14 per unit increase; 95% CI: 1.08-1.21), left ventricular (LV) mass indexed (HR: 1.01 per mg/m
2; 95% CI: 1.00-1.02), maximum LV wall thickness (HR: 1.19 per mm, 95% CI: 1.04-1.36) and LV-global longitudinal strain (HR: 1.20 per unit increase; 95% CI: 1.16-1.25) were significantly associated with the cardiovascular endpoint, whereas T1-mapping and LV-ejection fraction were not. T1-mapping was associated with the composite endpoint (HR: 0.99 per msec increase; 95% CI: 0.98-1.00). Meta-regression analysis did not show any significant interaction between each of the potential effect modifiers.
CONCLUSIONS: Several imaging parameters were significant predictors of adverse clinical outcomes in patients with FD. Late gadolinium enhancement showed the strongest association with adverse prognosis.
AB - BACKGROUND: Cardiac involvement represents the main cause of death in patients with Fabry disease (FD). Echocardiography and cardiovascular magnetic resonance (CMR) have an established diagnostic role, but their prognostic value remains unresolved. This systematic review and meta-analysis sought to assess the prognostic implications of imaging parameters in FD.METHODS: PubMed, ClinicalTrials.gov, Embase, Cochrane Library and Web of Science databases were searched for studies from inception through 1 May 2024. Studies including FD patients undergoing baseline imaging assessment and clinical follow-up were selected. Pre-defined study outcomes were a cardiovascular endpoint and a composite clinical endpoint. The study protocol was registered in PROSPERO (ID CRD42022342394).RESULTS: Fourteen studies, including 1713 FD patients (44.7% males), were selected. At pooled analysis, late gadolinium enhancement (hazard ratio [HR]: 4.45; 95% CI: 2.82-7.02), left atrium volume indexed (HR: 1.02 per mL/m
2; 95% CI: 1.01-1.03), E/e' (HR: 1.14 per unit increase; 95% CI: 1.08-1.21), left ventricular (LV) mass indexed (HR: 1.01 per mg/m
2; 95% CI: 1.00-1.02), maximum LV wall thickness (HR: 1.19 per mm, 95% CI: 1.04-1.36) and LV-global longitudinal strain (HR: 1.20 per unit increase; 95% CI: 1.16-1.25) were significantly associated with the cardiovascular endpoint, whereas T1-mapping and LV-ejection fraction were not. T1-mapping was associated with the composite endpoint (HR: 0.99 per msec increase; 95% CI: 0.98-1.00). Meta-regression analysis did not show any significant interaction between each of the potential effect modifiers.
CONCLUSIONS: Several imaging parameters were significant predictors of adverse clinical outcomes in patients with FD. Late gadolinium enhancement showed the strongest association with adverse prognosis.
UR - http://www.scopus.com/inward/record.url?scp=85216451914&partnerID=8YFLogxK
U2 - 10.1111/eci.14388
DO - 10.1111/eci.14388
M3 - Review article
C2 - 39840688
SN - 0014-2972
VL - 55
JO - European Journal of Clinical Investigation
JF - European Journal of Clinical Investigation
IS - 5
M1 - e14388
ER -