Cardiac Magnetic Resonance Depiction of Different Morphological Appearances of Becker Cardiomyopathy in Siblings

Abstract

Becker muscular dystrophy (BMD) is an X-linked recessive disorder involving mutation of the dystrophin gene. Cardiac involvement in BMD is frequent and represents the foremost cause of mortality. Two male siblings with severe left ventricular (LV) dysfunction and presence of deletion in the dystrophin gene underwent cardiac magnetic resonance (CMR) imaging, which revealed typical but varying imaging findings. The CMR revealed dilated left ventricle with severe global hypokinesis with preserved right ventricular (RV) function. Few patchy areas of septal edema were seen with typical epicardial enhancement along the LV lateral wall and the RV side of septum in one sibling. Both the siblings revealed an elevated myocardial native T1 values. CMR has the potential to detect cardiac involvement early by identifying and quantifying fibrosis, before wall motion abnormalities set in and determine prognosis in patients with muscular dystrophy and BMD carriers.