ECG ABNORMALITIES IN PEDIATRIC-PATIENTS WITH MYOTONIC-DYSTROPHY

Electrocardiographic (EKG) abnormalities are frequent in patients with myotonic dystrophy; cardiac complications may lead to significant mor bidity and mortality. The charts of 17 pediatric patients with myotoni c dystrophy were reviewed to ascertain the frequency of EKG abnormalit ies and cardiovascular symptoms. Fifteen of 17 patients had abnormal E KGs with sinus bradycardia being the most common abnormality. Only 1 o f 17 patients had cardiovascular symptoms. Four patients had moderate to severe weakness and 3 of them had a conduction disturbance (i.e., f irst-degree AV block or intraventricular conduction delay). Two of the remaining 13 patients with mild weakness had conduction disturbances. No pediatric patients had progressive EKG abnormalities during follow -up. Baseline EKG study of pediatric patients with myotonic dystrophy is recommended because abnormalities are frequent and usually asymptom atic. Frequent follow-up EKGs are probably unnecessary unless the pati ent is symptomatic or has heart block.