Female patient with proximal myotonic myopathy and ventricular tachycardia.

A 68-year-old woman with known proximal myotonic myopathy was transferred to our hospital for further diagnostic and therapeutic evaluation after successful termination of an episode of sustained ventricular tachycardia. In 2001, the myopathy was diagnosed after symptomatic weakness of the hip flexors. A cardiomyopathy with slight reduction of systolic left ventricular function was found in 2002. Coronary angiography excluded relevant coronary artery disease. The electrophysiologic examination could provoke atrial flutter, but neither a ventricular tachycardia nor a delay in the AV conduction was found. ECG and Holter ECG did not reveal any abnormalities. A reduction of the left ventricular systolic function (EF 45%) with normal size of cardiac chambers was demonstrated by echocardiography. It is known that in the patient group with myotonic dystrophies cardiac involvement manifests itself as cardiomyopathy, conduction disturbance or arrhythmia. However, only a small percentage of all patients with myotonic myopathy actually suffer from cardiac involvement. Among the different types of cardiac involvement, conduction disturbances requiring pacemaker implantation are most frequent. Only some patients develop ventricular tachycardias, and even cases of sudden cardiac death have been described. As a result of the case reports in the literature and the findings in our patient an ICD system was implanted on March 4, 2004.