Cardiac MRI in Duchenne and Becker Muscular Dystrophy

Area of Science:

• Cardiology
• Neurology
• Medical Imaging

Background:

• Cardiovascular magnetic resonance imaging (CMRI) is crucial for detecting cardiac involvement in Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD).
• CMRI is underutilized in routine clinical practice within the Indian healthcare system.
• Early detection of cardiac issues in DMD/BMD is vital for patient management.

Purpose of the Study:

• To determine the prevalence of CMRI abnormalities in Indian patients with DMD and BMD.
• To compare CMRI findings with phenotypic and genotypic characteristics.
• To assess the utility of CMRI in routine clinical practice for these conditions.

Main Methods:

• A prospective, observational study included 46 genetically diagnosed DMD and BMD patients.
• CMRI was performed between March 2020 and March 2022.
• Abnormal CMRI defined by late gadolinium enhancement (myocardial fibrosis), wall motion abnormalities, or reduced ejection fraction (LVEF <55%).

Main Results:

• Cardiac abnormalities were observed in 50% of patients (23/46).
• Late gadolinium enhancement (LGE) was more prevalent in DMD (42.1%), while reduced LVEF was more common in BMD (62.5%).
• Age and disease duration correlated with LGE in DMD; no genotype-CMRI correlation was found.

Conclusions:

• Significant differences exist in myocardial fibrosis and LV dysfunction between DMD and BMD patients.
• CMRI parameters showed variations based on the specific muscular dystrophy type.
• The lack of genotype-CMRI correlation in this cohort warrants further investigation.