杜氏肌营养不良(DMD)伴严重心肌病

Ji Hyoung Park, K. Lee, W. Wang, H. Lim
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引用次数: 0

摘要

杜氏肌营养不良症(DMD)是一种进行性肌病。呼吸疗法的发展提高了DMD患者的预期寿命。这一变化增加了伴有晚期心肌病的DMD患者麻醉给药的机会。我们报告一个严重的心肌病病例在一个14岁的男孩与DMD,肾上腺功能不全,和严重的智力低下,谁经历了心脏骤停成功复苏。患者行喂养胃造口手术以缓解反复吸入性肺炎,期间心脏指数和心率下降。心肌病已成为DMD患者的新挑战;左心室的正常功能对维持终末器官灌注具有重要意义。
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Duchenne Muscular Dystrophy (DMD) with Severe Cardiomyopathy
Duchenne muscular dystrophy (DMD) is a progressive myopathy. The development of respiratory therapy has increased the life expectancy of DMD patients. This change has increased the chances of anesthesia administration in DMD patients with advanced cardiomyopathy. We report a severe cardiomyopathy case in a 14-year-old boy with DMD, adrenal insufficiency, and severe mental retardation, who experienced a sudden cardiac arrest with successful resuscitation. The patient underwent feeding gastrostomy surgery to relieve recurrent aspiration pneumonia, during which cardiac index and heart rate decreased. Cardiomyopathy has emerged as a new challenge in DMD patients; it is important to maintain end organ perfusion by proper function of the left ventricle.
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