Heart Institute Cincinnati Children's Hospital Medical Center Cincinnati OH.
Department of Pediatrics University of Cincinnati College of Medicine Cincinnati OH.
J Am Heart Assoc. 2021 Oct 5;10(19):e021037. doi: 10.1161/JAHA.121.021037. Epub 2021 Sep 25.
Background Patients with Duchenne muscular dystrophy (DMD) develop cardiomyopathy because of a dystrophin deficiency causing fibrofatty replacement of the myocardium. Corticosteroid use and mobility limitations place these patients at risk for increased adiposity. We sought to determine the association of adiposity with cardiovascular dysfunction in patients with DMD. Methods and Results This was a retrospective review of patients with DMD who underwent both cardiac magnetic resonance imaging and dual-energy x-ray absorptiometry within 1 year. The cardiac magnetic resonance imaging parameters included left ventricular ejection fraction and the presence of late gadolinium enhancement (LGE positive [LGE+]). The adiposity indices, measured by dual-energy x-ray absorptiometry, included percentage of body fat, whole body fat mass indexed to height, and body mass index. A total of 324 patients were identified. Fifty-two percent had LGE+, and 36% had cardiac dysfunction (left ventricular ejection fraction <55%). Patients with cardiac dysfunction had higher whole body fat mass indexed to height and body mass index on univariate analysis (mean difference between patients with and without cardiac dysfunction: +2.9 kg/m, =0.001; and +1.5 kg/m, =0.03, respectively). whole body fat mass indexed to height remained independently associated with cardiac dysfunction on multivariable analysis after adjusting for age, LGE+, and corticosteroid duration. High whole body fat mass indexed to height and percentage of body fat were associated with LGE+ on univariate analysis (mean difference between patients with and without LGE+: +2.0 kg/m, =0.02; and +2.4%, =0.02, respectively). Using multivariable analysis, including age and cardiac dysfunction, high percentage of body fat remained independently associated with LGE+. Conclusions This study demonstrates an independent association of adiposity with cardiac dysfunction and LGE+ in patients with DMD. Preventing adiposity may mitigate the later development of ventricular dysfunction in DMD.
由于肌营养不良蛋白缺乏导致心肌纤维脂肪替代，杜氏肌营养不良症（DMD）患者会发生心肌病。皮质类固醇的使用和运动能力受限使这些患者面临肥胖风险增加的风险。我们旨在确定 DMD 患者的肥胖与心血管功能障碍的关系。
这是一项回顾性研究，纳入了在 1 年内同时接受心脏磁共振成像和双能 X 射线吸收法检查的 DMD 患者。心脏磁共振成像参数包括左心室射血分数和晚期钆增强（LGE 阳性[LGE+]）。通过双能 X 射线吸收法测量的肥胖指数包括体脂百分比、全身脂肪质量与身高的比值和体重指数。共纳入 324 例患者。52%的患者有 LGE+，36%的患者有心脏功能障碍（左心室射血分数<55%）。单因素分析显示，有心脏功能障碍的患者全身脂肪质量与身高的比值和体重指数更高（患者有和无心脏功能障碍之间的平均差异：+2.9kg/m，=0.001；+1.5kg/m，=0.03）。在校正年龄、LGE+和皮质类固醇持续时间后，全身脂肪质量与身高的比值在多变量分析中仍然与心脏功能障碍独立相关。全身脂肪质量与身高的比值和体脂百分比在单因素分析中与 LGE+相关（患者有和无 LGE+之间的平均差异：+2.0kg/m，=0.02；+2.4%，=0.02）。使用包括年龄和心脏功能障碍的多变量分析，高体脂百分比仍然与 LGE+独立相关。
本研究表明，肥胖与 DMD 患者的心脏功能障碍和 LGE+独立相关。预防肥胖可能会减轻 DMD 患者心室功能障碍的后期发展。
