Quantification of myocardial fibrosis in muscular dystrophy by cardiovascular magnetic resonance imaging and biomarkers

2021 Fiscal Year Final Research Report

• Project/Area Number: 19K17189
• Research Category: Grant-in-Aid for Early-Career Scientists
• Allocation Type: Multi-year Fund
• Review Section: Basic Section 52040:Radiological sciences-related
• Research Institution: Jichi Medical University (2020-2021); Hokkaido University (2019)
• Principal Investigator: Aikawa Tadao 自治医科大学, 医学部, 客員研究員 (20795059)
• Project Period (FY): 2020-03-01 – 2022-03-31
• Keywords: 筋ジストロフィー / 心筋障害 / 核磁気共鳴画像 / MRI

Outline of Final Research Achievements

T1-mapping and extracellular volume fraction (ECV) quantification was prospectively performed using cardiovascular MRI in 6 male patients with muscular dystrophy and 5 female putative carriers of Duchenne or Becker muscular dystrophy. All patients were male with a median age of 16.0 years, while the median age of 5 putative carriers was 46.0 years. The patient group included patients with Duchenne muscular dystrophy (n=2), Becker muscular dystrophy (n=3), and myotonic dystrophy (n=1). All putative carriers were mothers of confirmed male patients with Duchenne or Becker muscular dystrophy without any symptoms and known comorbidities. Four of the patients and 1 of the putative carriers had a reduced left ventricular ejection fraction (<55%). Four patients and 2 putative carriers showed visually detected late gadolinium enhancement (LGE). Five patients and all putative carriers had an elevated ECV (>29.5% for men and >35.2% for women).

Free Research Field

循環器画像診断

Academic Significance and Societal Importance of the Research Achievements

本研究より、びまん性心筋障害を反映したECV上昇は筋ジストロフィーの患者および推定保因者の両方にみられることが明らかになった。筋ジストロフィーでは、遅延造影MRIで異常増強像がなくともびまん性心筋障害によりECVが上昇している症例もあり、ECVは遅延造影MRIよりも正確に心筋障害を反映した指標である可能性がある。今後は造影剤を用いないMRI撮像法による心筋障害の検出能についても明らかにしていく予定である。