Neuropediatrics 2004; 35 - V14
DOI: 10.1055/s-2004-819358

Clinical features of cardiomyopathy in muscular dystrophy Duchenne and Becker-Kiener

J Schessl 1, C Kirchmann 2, D Kececioglu 2, S Dittrich 2, R Korinthenberg 1
  • 1University Children's Hospital, University of Freiburg, Department of Neuropaediatrics and Muscular Disorders, University of Freiburg
  • 2Department of Paediatric Cardiology, University of Freiburg, Germany

Objective: To assess clinical features of cardiomyopathy in DMD and BMD.

Methods: Analysis of patient records and current echocardiography, ECG and Holter ECG.

Results: Prevalence of reduced left ventricular fraction shortening (FS <25%) was 24% in all patients (50 DMD, 12.1±4.7 years; 20 BMD, 17.1±8.5 years). Median age for onset of cardiomyopathy was 16.8±1.0 years in DMD and 30.4±3.4 years in BMD, p<0.05. Holter ECG in DMD demonstrated sinustachycardia in 26%, deprivation of circadian rhythm in 31% and reduced heart rate variability in 51%. Pathologic FS and ECG records were related to patients age and walking abilities. A dfference between patients treated with and without Prednisone was not seen.

Conclusion: We emphasise the ENMC recommendations to perform echocardiography annually in DMD and BMD >10 years. As the rate of disturbances of the cardiac autonomic nerval system is high, we further recommend Holter ECG on every cardiac examination. Further investigation should be conducted to determine if beta-blockers in combination with ACE-inhibitors can reduce cardiac autonomic nerval imbalance and thus improve patient outcome in DMD.

Keywords: Duchenne, Becker-Kiener, cardiomyopathy, cardiac autonomic nerval dysfunction