Department of Emergency Medicine, University of Virginia, Charlottesville, VA, United States of America; Department of Pediatrics, University of Virginia, Charlottesville, VA, United States of America.
Department of Pediatrics, University of Virginia, Charlottesville, VA, United States of America.
Am J Emerg Med. 2022 Oct;60:101-105. doi: 10.1016/j.ajem.2022.07.056. Epub 2022 Aug 2.
Patients with Duchenne muscular dystrophy are living longer and are increasingly seen in Emergency Departments. Though the most common cause of death remains progressive respiratory failure, increased life expectancies have unmasked the significance of progressive myocardial dysfunction, now associated with nearly 40% of mortalities in the DMD population. Cardiac complications such as arrhythmias and cardiomyopathy are becoming ever more widely recognized. Emergency physicians may encounter DMD patients with untreated, undiagnosed or worsening of known heart disease. This review will initially familiarize the emergency physician with the pathophysiology and lifetime trajectory of care for these patients before describing specific emergency department evaluation and treatment.
杜氏肌营养不良症患者的寿命越来越长，在急诊科也越来越常见。尽管最常见的死亡原因仍然是进行性呼吸衰竭，但预期寿命的延长揭示了进行性心肌功能障碍的重要性，现在与 DMD 人群中近 40%的死亡率有关。心律失常和心肌病等心脏并发症越来越被广泛认识。急诊医生可能会遇到未经治疗、未诊断或已知心脏病恶化的 DMD 患者。本综述将在描述特定的急诊科评估和治疗之前，使急诊医生熟悉这些患者的病理生理学和终生治疗轨迹。
