Ventricular Tachycardia in a Patient with Neuromuscular Disease

A 41-year-old woman with type 1 myotonic dystrophy and a family history of sudden cardiac death presented with frequent palpitations and near syncope. During an electrophysiology study, she was found to have a prolonged HV interval and easily inducible wide complex tachycardia, consistent with bundle branch reentrant ventricular tachycardia for which she underwent successful catheter ablation of the right bundle branch. The importance of clinical surveillance for conduction abnormalities and arrhythmias in patients with myotonic dystrophy is discussed.