Predictive electrocardiographic score for evaluating prognosis in patients with Duchenne's muscular dystrophy

Electrocardiographic changes were evaluated in 20 patients with a diagnosis of Duchenne's muscular dystrophy (DMD) for a period of 4 to 9 years preceding their death. Certain common electrocardiographic findings were noted in patients during the terminal stage of their disease, and may have prognostic significance. These included: an R wave in lead V1 of less than 0.6 mV, in lead V5 of less than 1.1 mV, and in lead V6 of less than 1.0 mV; abnormal T waves in leads II, III, a VF, V5 and V6; cardiac conduction disturbances; premature ventricular contractions; and sinus tachycardia. A predictive scoring system was proposed based on these findings. The predictive scores for patients who died primarily from cardiac failure were significantly higher than for patients who died primarily from respiratory failure. This score increased to over 10 points as the patient's clinical condition deteriorated. In conclusion, a predictive score is useful in managing patients with DMD, particularly when evaluating for possible cardiac failure.