Introduction
Dystrophinopathies in the form of Duchenne and Becker’s muscular
dystrophies affect 1:5000 newborn males worldwide1. As
patients age, myocyte degeneration and myocyte fibrosis occur, resulting
in progressive immobility and respiratory failure. While neuromuscular
disease is severe in Duchenne Muscular Dystropy (DMD) and milder in
Becker’s Muscular Dystrophy (BMD), dystrophin deficient cardiomyopathy
tends to be more unpredictable in onset. Myocardial fibrosis and
arrhythmic manifestations are common and do not necessarily correlate to
the degree of skeletal muscle involvement2. In fact,
in cases of more mild muscular disease, cardiac dysfunction may precede
symptomatic skeletal muscle involvement3. Periodic
cardiac evaluation including imaging and routine surveillance of cardiac
arrhythmias is recommended, and advanced imaging frequently detects
myocardial fibrosis under the age of ten years old4.