Echocardiographic and electrocardiographic findings of cardiomyopathy in Duchenne and Becker-Kiener muscular dystrophies

Abstract

We assessed the cardiac findings in Duchenne muscular dystrophy (DMD) and Becker-Kiener muscular dystrophy (BMD) patients in the large outpatient group of our single-center institution. The analysis included the revision of patient records (retrospectively) and current echocardiography, electrocardiogram (ECG), and Holter ECG data (prospectively). Reduced left ventricular fraction shortening (FS) < 25% was found in 24% of all patients (50 DMD, 12.1 +/- 4.7 years: 20 BMD, 17.1 +/- 8.5 years). Median age of onset of FS < 25% was 16.8 +/- 1.0 in DMD and 30.4 +/- 3.4 in BMD; (p < 0.05). Holter ECG in DMD demonstrated sinus tachycardia in 26% deprivation of circadian rhythm in 31%, and reduced heart rate variability in 51%. For these reasons, we recommend carrying out echocardiography annually in DMD and BMD > 10 years. Because the rate of disturbances in the cardiac autonomic nervous system is high in DMD, we also recommend Holter ECG annually. Further investigation should be conducted to determine if treatment with ss-blockers and ACE inhibitors in combination reduces cardiac autonomic nervous imbalance, thus improving patient outcome in DMD.