Cardiovascular Surveillance of Duchenne and Becker Muscular Dystrophy and Female Carriers

Virginia      Spehrs-Ciaffi; Pierre-Yves      Jeannet; Nicole      Sekarski

Abstract

Duchenne muscular dystrophy is the most common form of the childhood muscular dystrophies. It follows a predictable clinical course marked by progressive skeletal muscle weakness, lost of ambulation before teen- age and death 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. Female carriers of both entities are also affected in a variable degree. The natural history of the cardiac involvement in these diseases has not been well established and without screening it progresses asymptomatically until the cardiac reserve has been eroded, when symptoms and signs of cardiac failure emerge. The purpose of this work is to provide recommendations for adequate cardiovascular care in this particular population based on the updated literature as referenced.

Keywords: Neuromuscular diseases, Duchenne muscular dystrophy, Becker muscular dystrophy, cardiovascular care

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