Rajdev Archana, Groh William J
Krannert Institute of Cardiology, Indiana University School of Medicine, Indianapolis, IN 46202, USA.
William Jennings Bryan Dorn Veterans Affairs Medical Center, University of South Carolina, 6439 Garners Ferry Road, Columbia, SC 29209-1639, USA.
Card Electrophysiol Clin. 2015 Jun;7(2):303-8. doi: 10.1016/j.ccep.2015.03.011. Epub 2015 Mar 29.
In patients with muscular dystrophies, cardiac involvement leading to cardiomyopathy and arrhythmias occurs with variable prevalence, mirroring the phenotypic variability seen among and within the various hereditary myopathies. Knowledge of the incidence of arrhythmias and predictors of sudden death in the various hereditary myopathies can help guide screening and appropriate management of these patients, thereby improving survival. The noncardiac manifestations can lead to delayed recognition of symptoms, affect the decision to implant a prophylactic device, and once a decision is made to proceed with device implant, increase peri-procedural respiratory and anesthesia-related complications.
在肌肉萎缩症患者中,导致心肌病和心律失常的心脏受累发生率各不相同,这反映了各种遗传性肌病之间以及内部存在的表型变异性。了解各种遗传性肌病中心律失常的发生率和猝死的预测因素有助于指导对这些患者的筛查和适当管理,从而提高生存率。非心脏表现可能导致症状识别延迟,影响植入预防性装置的决策,并且一旦决定进行装置植入,会增加围手术期呼吸和麻醉相关并发症的发生。