Department of Anesthesiology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
Department of Anesthesiology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
Rev Esp Anestesiol Reanim (Engl Ed). 2023 Nov;70(9):540-544. doi: 10.1016/j.redare.2023.09.008. Epub 2023 Sep 6.
Emery-Dreifuss muscular dystrophy is associated with cardiac abnormalities and rarely heart transplantation may be the treatment of choice. In this case, a male patient with Emery- Dreifuss muscular dystrophy developed NYHA class IV heart failure at 33 years of age and was submitted to heart transplantation. Anesthesia was adapted to prevent the development of malignant hyperthermia and rhabdomyolysis. The surgery was a success and the patient's progress was extremely positive with symptomatic improvement. In these patients, is critical to adjust not only his positioning but also the therapy administered in order to reduce iatrogeny and promote a faster recovery.
肌营养不良症伴心肌病变与心脏异常有关，且心脏移植可能是首选治疗方法。本例患者为男性，33 岁，诊断为肌营养不良症伴心肌病变，心功能 NYHA 分级达 IV 级，行心脏移植术。麻醉管理旨在预防恶性高热和横纹肌溶解的发生。手术成功，患者术后恢复迅速，症状改善明显。此类患者，不仅要调整其体位，还要调整治疗方案，以减少医源性损伤，促进更快康复，这一点至关重要。
