2023 American Academy of Neurology Abstract Website

Objective:

To explore whether myocardial hypoxia exists in DMD patients and whether an association exists between regional myocardial oxygenation and fibrosis, using oxygenation-sensitive cardiovascular magnetic resonance and native T1 mapping.

Background:

Myocardial hypoxia is common in many cardiomyopathies and has been proven to be closely related to the occurrence and development of myocardial fibrosis. However, limited studies are available on the relationship between myocardial oxygenation and fibrosis in DMD patients using oxygenation-sensitive magnetic resonance, and whether myocardium hypoxia exists in DMD patients and its underlying relationship with fibrosis in DMD remains unclear.

Design/Methods:

In this prospective study, 91 DMD patients and 30 volunteers underwent CMR. The correlation between oxygenation SI and native T1 value was assessed by Pearson or Spearman correlation coefficients and the association between oxygenation SI and LGE was analyzed by logistic regression. The survival curves were generated by the Kaplan–Meier analysis and compared by the log-rank test.

Results:

In LGE- patients , the oxygenation SI of all segments except the septal segments was significantly decreased than those of the controls. Inferolateral SI and anterolateral SI were weakly correlated with native T1 values. As for LGE+ patients, a further and significant reduction in oxygenation SI was observed relative to the controls. SI was moderately correlated with native T1 values in both negative and positive segments. Multivariable regression modeling indicated inferolateral SI independently associated with LGE. At an average follow-up of 18.63 months, 31 patients experienced progress of LGE. Patients with reduced inferolateral SI were more likely to have an exacerbation of fibrosis.

Conclusions:

Analysis of OS-CMR and native T1 mapping revealed that myocardial hypoxia is common and related to the onset of myocardial fibrosis in DMD patients. And myocardial oxygenation by OS-CMR might be used as the quantitative imaging biomarker for the assessment of myocardial damage and prediction of fibrosis progression in DMD patients.