Cardiac involvement in muscular dystrophies leads to cardiomyopathy and rhythm disturbances, requiring specific monitoring and management strategies.
This review provides an overview of cardiac involvement in muscular dystrophies and offers practical recommendations for monitoring and management of associated cardiomyopathy and arrhythmias.
Cardiac disease is a common clinical manifestation present in a variety of neuromuscular disorders, most notably the muscular dystrophies. Heart disease may produce the presenting or predominant symptoms in these disorders but more often not does not result in clinical features at the time of initial presentation. Cardiac involvement in the muscular dystrophies results from pathologic changes in the myocardium and the cardiac conduction system, leading to cardiomyopathy and/or rhythm disturbances including supraventricular arrhythmias, life-threatening ventricular arrhythmias, and sudden cardiac death. This Review covers the spectrum of cardiac dysfunction in these inherited muscle disorders and proposes practical recommendations for monitoring and management. Muscle Nerve 57: 707-715, 2018.
Silvestri et al. (Sat,) conducted a review in Muscular dystrophies. Cardiac involvement in muscular dystrophies leads to cardiomyopathy and rhythm disturbances, requiring specific monitoring and management strategies.