Cardiovascular Health Supervision for Individuals Affected by Duchenne or Becker Muscular Dystrophy

Abstract

Duchenne muscular dystrophy is the most common and severe form of the childhood muscular dystrophies. The disease is typically diagnosed between 3 and 7 years of age and follows a predictable clinical course marked by progressive skeletal muscle weakness with loss of ambulation by 12 years of age. Death occurs in early adulthood secondary to respiratory or cardiac failure. Becker muscular dystrophy is less common and has a milder clinical course but also results in respiratory and cardiac failure. The natural history of the cardiomyopathy in these diseases has not been well established. As a result, patients traditionally present for cardiac evaluation only after clinical symptoms become evident. The purpose of this policy statement is to provide recommendations for optimal cardiovascular evaluation to health care specialists caring for individuals in whom the diagnosis of Duchenne or Becker muscular dystrophy has been confirmed.