Northern Ontario School of Medicine, Sadbury and Thunder Bay, Ontario, Canada.
Can J Cardiol. 2012 Jul-Aug;28(4):516.e1-3. doi: 10.1016/j.cjca.2012.01.012. Epub 2012 Apr 4.
Emery-Dreifuss muscular dystrophy (EDMD) is an inherited disorder affecting skeletal and cardiac muscles and characterized by muscular atrophy, contractures, and cardiomyopathy with conduction defects. It can be X-linked or autosomal. Not all patients with EDMD develop heart involvement, but heart disease associated with EDMD can be unpredictable and may be life threatening. In rare cases heart problems may be the first symptom of EDMD. Early recognition of heart involvement is of utmost importance as placement of a pacemaker and/or defibrillator may be lifesaving.
先天性肌营养不良症（EDMD）是一种遗传性疾病，影响骨骼和心肌，其特征是肌肉萎缩、挛缩和心肌病伴传导缺陷。它可以是 X 连锁或常染色体的。并非所有 EDMD 患者都有心脏受累，但与 EDMD 相关的心脏病可能是不可预测的，并且可能危及生命。在极少数情况下，心脏问题可能是 EDMD 的首发症状。早期识别心脏受累至关重要，因为植入起搏器和/或除颤器可能有救命作用。
