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oai:recercat.cat:11351/127422025-10-24T10:30:59Zcom_2072_378070com_2072_378040col_2072_378092

2025-10-24T10:30:59Z urn:hdl:11351/12742 Asymmetric Myocardial Involvement as an Early Indicator of Cardiac Dysfunction in Pediatric Dystrophinopathies: A Study on Cardiac Magnetic Resonance (CMR) Parametric Mappings Esmel Vilomara, Roger RIAZA MARTIN, LUCIA Costa-Comellas, Laura Sabaté Rotés, Anna Gran, Ferran Imatgeria per al diagnòstic en pediatria Insuficiència cardíaca - Imatgeria per ressonància magnètica Cor - Malalties - Imatgeria per ressonància magnètica Distròfia muscular de Duchenne ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Diagnosis::Diagnostic Techniques and Procedures::Diagnostic Imaging::Tomography::Magnetic Resonance Imaging::Magnetic Resonance Imaging, Cine DISEASES::Nervous System Diseases::Neuromuscular Diseases::Muscular Diseases::Muscular Disorders, Atrophic::Muscular Dystrophies::Muscular Dystrophy, Duchenne DISEASES::Cardiovascular Diseases::Heart Diseases::Cardiomyopathies Other subheadings::Other subheadings::Other subheadings::/diagnostic imaging TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::diagnóstico::técnicas y procedimientos diagnósticos::diagnóstico por imagen::tomografía::imagen por resonancia magnética::imagen de cinerresonancia magnética ENFERMEDADES::enfermedades del sistema nervioso::enfermedades neuromusculares::enfermedades musculares::trastornos musculares atróficos::distrofias musculares::distrofia muscular de Duchenne ENFERMEDADES::enfermedades cardiovasculares::enfermedades cardíacas::miocardiopatías Otros calificadores::Otros calificadores::Otros calificadores::/diagnóstico por imagen Cardiac magnetic resonance; Cardiomyopathy; Dystrophinopathies Resonancia magnética cardíaca; Miocardiopatía; Distrofinopatías Ressonància magnètica cardíaca; Miocardiopatia; Distrofinopaties Dystrophinopathies, such as Duchenne and Becker muscular dystrophy, frequently lead to cardiomyopathy, being its primary cause of mortality. Detecting cardiac dysfunction early is crucial, but current imaging methods lack insight into microstructural remodeling. This study aims to assess the potential of cardiac magnetic resonance (CMR) parametric mappings for early detection of myocardial involvement in dystrophinopathies and explores whether distinct involvement patterns may indicate impending dysfunction. In this prospective study, 23 dystrophinopathy patients underwent CMR with tissue mappings. To establish a basis for comparison, a control group of 173 subjects was analyzed. CMR protocols included SSFP, T2-weighted and T1-weighted sequences pre and post gadolinium, and tissue mappings for native T1 (nT1), extracellular volume (ECV), and T2 relaxation times. The difference between the left ventricular posterior wall and the interventricular septum was calculated to reveal asymmetric myocardial involvement. Significant differences in LV ejection fraction (LVEF), myocardial mass, and late gadolinium enhancement confirmed abnormalities in patients. Tissue mappings: nT1 (p < 0.001) and ECV (p = 0.002), but not T2, displayed substantial variations, suggesting sensitivity to myocardial involvement. Asymmetric myocardial involvement in nT1 (p = 0.01) and ECV (p = 0.012) between septal and LV posterior wall regions was significant. While higher mapping values didn’t correlate with dysfunction, asymmetric involvement in nT1 (ρ=-0.472, p = 0.023) and ECV (ρ=-0.460, p = 0.049) exhibited a significant negative correlation with LVEF. CMR mappings show promise in early myocardial damage detection in dystrophinopathies. Although mapping values may not directly correspond to dysfunction, the negative correlation between asymmetric involvement in nT1 and ECV with LVEF suggests their potential as early biomarkers. Larger, longitudinal studies are needed for a comprehensive understanding and improved risk stratification in dystrophinopathies. 2025-10-24T10:30:59Z 2025-10-24T10:30:59Z 2025-03-11T11:03:24Z 2025-03-11T11:03:24Z 2024 2025-03 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion http://hdl.handle.net/11351/12742 Pediatric Cardiology;46 https://doi.org/10.1007/s00246-024-03488-8 Attribution 4.0 International http://creativecommons.org/licenses/by/4.0/ info:eu-repo/semantics/openAccess Springer Scientia