The prevalence and natural progress of myocardial fibrosis in Duchenne muscular dystrophy patients. (25th November 2020)

• Record Type: Journal Article
• Authors: Xu, R; Xu, H; Liu, H; Guo, Y
• Is Part Of: European heart journal. Volume 41:(2020)Supplement 2
• Publication Date: 2020-11-25
• Subjects: Cardiac Magnetic Resonance: Myocardium; Cardiology -- Periodicals; Heart -- Diseases -- Periodicals; 616.12005
• DOI: 10.1093/ehjci/ehaa946.0214
• Languages: English
• ISSNs: 0195-668X

Abstract

Abstract: Purpose: The cardiac disease is now as the leading cause of morbidity and mortality in DMD patients. The early detection of cardiac involvement is challenging and the frequency and presence of the cardiac involvement in the first decade is uncertain. The aim of the current study was to characterize the frequency, pattern and extent of LGE in the first decade in DMD patients, and identify a set of prognosis factors that portend the presence and extent of LGE in those patients. Methods: There are 93 DMD patients (mean age: 8.31±2.30 years, rang: 3–13 years) with a biopsy showing absent dystrophin and/or DNA analysis demonstrating a characteristic dystrophin mutation enrolled. All patients underwent a complete cine imaging and LGE imaging. The left ventricular function and tissue characteristic were analyzed and statistic. Results: There were 42% patients presented LGE-positive. Six years old was the youngest age onset myocardial fibrosis, and after 7 years old, the frequency of LGE positive rising fast. Moreover, all LGE positive patients showed complex patterns with non-ischaemic pattern, including septum, papillary muscle and right ventricular involvement. The free wall, inferior, apical and septum were the most common position. The papillary and right ventricular were rarely common. And all patients presented right ventricular involvement associated with septum involvement. Moreover, compared with younger patients (<7 years), the presence and extent of LGE in older patients (≥7 years) were significant higher. And the logistic analysis showed that the age is a strong predictor of presence of LGE (OR: 1.61, 95% CI: 1.26–2.05, P<0.000). Conclusion: The myocardial fibrosis could onset in DMD patients in the first decade with a rapid rising and presented with complex patterns. The early CMR examination is necessary for the assessment of cardiac involvement and should consider to complete in younger age in DMD patients. Funding Acknowledgement: Type of funding source: Foundation. Main funding source(s): The National Natural Science Foundation