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Right Ventricular Function and T1-Mapping in Boys With Duchenne Muscular Dystrophy
Seraina A Dual1,2,3, Nyasha G Maforo4,5, Doff B McElhinney2
1Department of Radiology, Stanford University, Palo Alto, California, USA.
Journal of Magnetic Resonance Imaging : JMRI
|May 26, 2021
Summary
Right ventricular T1 (RV-T1) is elevated in boys with Duchenne muscular dystrophy (DMD), indicating myocardial remodeling. This finding is crucial for understanding cardiac involvement in DMD patients.
Area of Science:
- Cardiology
- Biomedical Imaging
- Genetics
Background:
- Clinical management of Duchenne muscular dystrophy (DMD) requires understanding cardiac involvement.
- Right ventricular (RV) structural and functional remodeling in DMD is understudied.
Purpose of the Study:
- Evaluate methods for measuring RV pre- and postcontrast T1 (RV-T1).
- Characterize myocardial remodeling in the RV of boys with DMD.
Main Methods:
- Prospective study of 27 boys with DMD and 17 healthy controls.
- Utilized 3.0T MRI with specific sequences (bSSFP, mc-PSIR, mLLRR).
- Assessed biventricular mass, volumes, ejection fraction, tricuspid annular excursion, LGE, and T1 maps.
Main Results:
- A 1-pixel thick RV circumferential ROI was most reliable for RV-T1 assessment (ICC > 0.91).
- Precontrast RV-T1 was significantly higher in boys with DMD compared to controls.
- Boys with DMD showed preserved RVEF but reduced RVMi, RVEDVi, and TAE compared to controls.
Conclusions:
- Precontrast RV-T1 is elevated in boys with DMD, correlating negatively with RVEF.
- This elevation suggests RV myocardial remodeling in DMD patients.
Keywords:
Duchenne muscular dystrophyT1-mappingcardiomyopathycardiovascular magnetic resonancelate gadolinium enhancementmyocardial remodeling
