Cardiac involvement in children with neuro-muscular disorders

Abstract

Many inherited neuromuscular disorders include cardiac involvement as a typical clinical feature. Among the most common of them is the group of muscular dystrophies. Dilated cardiomyopathy, ventricular arrhythmias, atrial fibrillations, atrioventricular and intraventricular conduction abnormalities, and sudden cardiac death are well known pathological findings in Duchenne muscular dystrophies, myotonic dystrophy type I and 2, Emery-Dreifuss muscular dystrophies and different types of limb-girdle muscular dystrophies and other disorders. Detection of cardiac pathology in patients with different muscular dystrophies is possible with ECG, echocardiography and cardiovascular magnetic resonance imaging, which are recommended for screening and early cardioprotective treatment.

Keywords

• neuromuscular disorders
• duchenne muscular dystrophy
• myotonic dystrophy type 1, 2
• limb-girdle muscular dystrophy
• emery–dreifuss muscular dystrophy
• dilated cardiomyopathy
• ventricular arrhythmias
• atrial fibrillations
• atrioventricular conduction abnormalities
• intraventricular conduction abnormalities
• sudden cardiac death