Yoon Jin A, Lee Heirim, Lee In Sook, Song You Seon, Lee Byeong-Ju, Kim Soo-Yeon, Shin Yong Beom
Department of Rehabilitation Medicine, Pusan National University Hospital, Pusan National University School of Medicine and Biomedical Research Institute, Busan, Korea.
Department of Pediatrics, Pusan National University Hospital, Pusan National University School of Medicine and Biomedical Research Institute, Busan, Korea.
Ann Rehabil Med. 2024 Dec;48(6):405-412. doi: 10.5535/arm.240006. Epub 2024 Dec 16.
To compare the progression of muscle fibrosis of various site and its relation between cardiac deterioration in Duchenne muscular dystrophy (DMD). In this study aimed to examine the associations between echocardiogram-based cardiac function indices and fibrosis of the abdominal and lower extremity muscles in patients with DMD to facilitate early detection of cardiac dysfunction and identify its predictors.
Twenty-one patients with DMD patients were enrolled in the study. The association between cardiac dysfunction and fibrosis of the abdominal and lower extremity muscles was determined by analyzing the echocardiography and elastography. Non-parametric Spearman rank correlation coefficients were used to examine the pairwise relationships between cardiac function and muscle elasticity.
All patients were male and non-ambulant. Their mean age was 18.45±4.28 years. The strain ratios of the abdominal muscle and quadriceps muscles were significantly higher than those of the medial gastrocnemius. The strain ratio of the rectus abdominis muscle has a significant negative correlation with left ventricular ejection fraction. Cardiac function and valvular insufficiency were not significantly correlated with muscle strain ratio. According to the result of our study, the only skeletal muscle which showed significant correlation with cardiac dysfunction was degree abdominal muscle fibrosis.
The degree of fibrosis of respiratory muscles was also significantly associated with cardiac dysfunction; therefore, it can be used as a predictor of cardiac dysfunction in patients with DMD in clinical practice.
比较杜氏肌营养不良症（DMD）患者不同部位肌肉纤维化的进展情况及其与心脏功能恶化之间的关系。本研究旨在探讨基于超声心动图的心脏功能指标与DMD患者腹部及下肢肌肉纤维化之间的关联，以促进心脏功能障碍的早期检测并确定其预测因素。
21例DMD患者纳入本研究。通过分析超声心动图和弹性成像来确定心脏功能障碍与腹部及下肢肌肉纤维化之间的关联。采用非参数Spearman等级相关系数来检验心脏功能与肌肉弹性之间的成对关系。
所有患者均为男性且无法行走。他们的平均年龄为18.45±4.28岁。腹肌和股四头肌的应变率显著高于腓肠肌内侧头。腹直肌的应变率与左心室射血分数呈显著负相关。心脏功能和瓣膜关闭不全与肌肉应变率无显著相关性。根据我们的研究结果，唯一与心脏功能障碍显示出显著相关性的骨骼肌是腹部肌肉纤维化程度。
呼吸肌的纤维化程度也与心脏功能障碍显著相关；因此，在临床实践中它可作为DMD患者心脏功能障碍的预测指标。
