Division of Cardiology, The Heart Center, Nationwide Children's Hospital, The Ohio State University, Columbus, Ohio, USA.
Pediatr Pulmonol. 2021 Apr;56(4):753-759. doi: 10.1002/ppul.25181. Epub 2020 Nov 27.
Duchenne muscular dystrophy (DMD) is the most common form of childhood muscular dystrophy resulting in progressive muscle wasting and weakness. With advancements in respiratory care and the use of glucocorticoids, cardiomyopathy has surpassed respiratory compromise as the leading cause of morbidity and mortality in this patient population. As muscular dystrophy remains a relative contraindication to heart transplantation, end-stage heart failure management represents a major therapeutic challenge. Long-term left ventricular assist device (LVAD) therapy has emerged as a promising management strategy to improve the survival and quality of life in DMD cardiomyopathy. Preoperative planning, optimal patient selection, aggressive postoperative rehabilitation, and continued discussion of goals of care are critical considerations for the appropriate use of LVAD in DMD patients with cardiomyopathy.
杜氏肌营养不良症（DMD）是导致进行性肌肉萎缩和无力的最常见的儿童肌肉营养不良症。随着呼吸治疗的进步和糖皮质激素的使用，心肌病已超过呼吸功能障碍，成为该患者群体发病率和死亡率的主要原因。由于肌肉营养不良症仍然是心脏移植的相对禁忌症，因此终末期心力衰竭的管理是一个主要的治疗挑战。长期左心室辅助装置（LVAD）治疗已成为改善 DMD 心肌病患者生存和生活质量的有前途的治疗策略。对于 DMD 心肌病患者使用 LVAD，术前规划、最佳患者选择、积极的术后康复以及持续讨论护理目标是至关重要的考虑因素。
