Usefulness of Cardiac Magnetic Resonance Imaging in Patients with Neuromuscular Dystrophies (Duchenne/Becker Muscular Dystrophy)

Abstract

Background: Cardiac magnetic resonance imaging (CMR) is commonly used in patients with Duchenne (DMD) and Becker (DMB) Neuromuscular Dystrophies. Late gadolinium enhancement (LGE) identifies areas of middle, subepicardial, or subendocardial wall fibrosis, and volumetric left ventricular ejection fraction (LVEF) is considered the gold standard in the diagnosis and prognosis of these dystrophies. Myocardial fibrosis occurs in patients with neuromuscular dystrophies. Objectives: The purposes of our study were to determine the presence of cardiac fibrosis using CMR, to determine neuromuscular and cardiac involvement, and to evaluate the cardiovascular outcomes of these patients. Material y métodos: A descriptive cross-sectional study of 16 consecutive patients was conducted from March 2021 to July 2022 in the Cardiac Imaging Service of Diagnóstico Médico and CEMET- Tucumán. Results: A total of 16 patients were evaluated, 100% of them with confirmed diagnosis of DMD/DMB by laboratory, enzymes and genetic tests. Mean age was 19 years. All patients had severe stage of the Vignos Scale and were under neurological treatment. All patients were also treated with beta-blockers or angiotensin-converting enzyme inhibitors. CMR revealed severe LVEF impairment <35% in 4 patients, segmental or global left ventricular (LV) wall motion disorders in 8 patients, and variable distribution pattern (diffuse, mesocardial, subendocardial and subepicardial patterns) of LGE in 12 patients. Non-compacted myocardium was observed in 6, and mild pericardial effusions in 2 patients. Conclusion: CMR should be included as a screening method in patients with neuromuscular dystrophies. Its contribution to clinical, echocardiographic and therapeutic staging is of utmost importance