Cardiac MRI and Echocardiography for Early Diagnosis of Cardiomyopathy Among Boys With Duchenne Muscular Dystrophy: A Cross-Sectional Study

• DOI: 10.3389/fped.2022.818608
• Publication Date: 2022-03-14T07:26:52Z
• Authors: Nidhi Prakash; Renu Suthar; Bhupendra Kumar S...; Uma Debi; Rohit Manoj Kumar; Naveen Sankhyan
• Keywords: Duchenne muscular dystrophy; cardiac MRI (CMRI); Cardiomyopathy; Molecular Mechanisms of Muscle Regeneration and Atrophy; Cardiology; Electrocardiogram Interpretation; Heart failure; Pediatrics; RJ1-570; 03 medical and health sciences; 0302 clinical medicine; Biochemistry, Genetics and Molecular Biology; Health Sciences; Pathology; echocardiography; Cardiac Imaging; ejection fraction; Molecular Biology; Internal medicine; Cross-sectional study; Dilated Cardiomyopathy; Diagnosis and Management of Arrhythmogenic Right Ventricular Cardiomyopathy; Diagnosis and Management of Hypertrophic Cardiomyopathy; Life Sciences; Muscular dystrophy; 3. Good health; Duchenne muscular dystrophy (DMD); Medicine; Cardiology and Cardiovascular Medicine; Cardiomyopathies; cardiomyopathy; Cardiac Screening

BackgroundCardiomyopathy is an important cause of morbidity and mortality in boys with Duchenne muscular dystrophy (DMD). Early diagnosis is a prerequisite for timely institution of cardioprotective therapies.ObjectiveWe compared cardiac MRI (CMRI) with transthoracic echocardiography (TTE) including tissue Doppler imaging (TDI) and speckle tracking echocardiography (STE) for diagnosis of cardiomyopathy in early ambulatory boys with DMD.MethodologyThis cross-sectional study was conducted between June 2018 and December 2020. Consecutive boys between 7 and 15 years of age with DMD were enrolled. Percentage ejection fraction (EF), fractional shortening, wall motion abnormalities, early diastolic mitral annulus velocity (Ea), medial mitral annulus ratio (E/Ea), and global strain were measured with STE. CMRI-derived EF, segmental hypokinesia, and late gadolinium enhancement (LGE) were studied and compared.ResultsA total of 38 ambulatory boys with DMD were enrolled. The mean age was 8.8 ± 1.6 years, and none had clinical features suggestive of cardiac dysfunction. In the TTE, EF was ≤55% in 5 (15%), FS was ≤28% in 3 (9%), and one each had left ventricular wall thinning and wall hypokinesia. In TDI, none had diastolic dysfunction, and STE showed reduced global strain of < 18% in 3 (9%) boys. CMRI-derived EF was ≤55% in 20 (53%) boys and CMRI showed the presence of left ventricular wall hypokinesia in 9 (24%) and LGE in 4 (11%) boys.ConclusionCardiomyopathy remains clinically asymptomatic among early ambulatory boys with DMD. A significantly higher percentage of boys revealed early features of DMD-related cardiomyopathy in CMRI in comparison with echocardiography.