MRI-Based Circumferential Strain in Boys with Early Duchenne Muscular Dystrophy Cardiomyopathy

Area of Science:

• Cardiology
• Biomarkers
• Medical Imaging

Background:

• Duchenne muscular dystrophy (DMD) cardiomyopathy is a leading cause of death.
• Late gadolinium enhancement (LGE) and reduced left ventricular ejection fraction (LVEF) are late indicators of DMD cardiac involvement.
• Early detection of cardiac dysfunction in DMD is crucial.

Purpose of the Study:

• To quantify regional end-systolic circumferential strain (E_cc) using cine Displacement Encoding with Stimulated Echoes (DENSE) in DMD boys without LGE.
• To compare E_cc with LVEF in differentiating DMD boys from healthy controls.
• To establish E_cc as an early biomarker for DMD-associated cardiomyopathy.

Main Methods:

• Prospective study involving 10 LGE-negative DMD boys and 12 healthy controls.
• Cardiovascular magnetic resonance (CMR) at 3T using navigator-gated cine DENSE.
• Estimation of global and regional E_cc and comparison with LVEF using statistical analysis and logistic regression.

Main Results:

• Boys with DMD showed significantly impaired septal E_cc compared to healthy controls (p=0.019).
• Both groups exhibited reduced septal E_cc and elevated lateral E_cc.
• Septal E_cc was more effective than LVEF in differentiating DMD boys from controls.

Conclusions:

• Reduced septal E_cc may indicate early cardiac involvement in DMD boys before LVEF reduction or LGE.
• Cine DENSE-derived E_cc is a promising biomarker for early detection of DMD cardiomyopathy.
• This finding aids in timely intervention and management of cardiac complications in DMD.