Duchenne muscular dystrophy (DMD) is a myopathy with a natural history of progressive cardiomyopathy and vasogenic edema, fatty infiltration, and myocardial fibrosis. We recently identified a group of patients with DMD who presented with acute onset of chest pain (ACP), troponin leak (TL), and new late gadolinium enhancement (LGE), similar in presentation to acute viral myocarditis (AM). It is unclear if these patients have suffered an episode of AM or if this is a different disease process. Given the presumed acute myocardial necrosis in both processes, we hypothesize that native T1 and T2 values would be elevated in AM and DMD patients with ACP compared to asymptomatic DMD patients. Read More
Meeting name: SPR 2019 Annual Meeting & Postgraduate Course , 2019
Authors: Lee Simon, Krishnamurthy Rajesh, Krishnamurthy Ramkumar, Johnston Thomas, Hor Kan
Keywords: Parametric Mapping, Cardiomyopathy
Cardiomyopathy is the leading cause of mortality for Becker Muscular Dystrophy (BMD).Risk stratification is challenging due to the wide variation of cardiomyopathy onset, progression, and severity; with cardiomyopathy developing independent of skeletal myopathy progression. Current guidelines recommend “complete cardiac evaluation” at ten years of age, but do not specify the type of imaging modality. We have been performing cardiac magnetic resonance imaging (CMR) routinely by 10 years of age. We sought to determine the progression of BMD cardiomyopathy during the adolescent years among patients who have evidence of fibrosis by late gadolinium enhancement(LGE). Read More
Meeting name: SPR 2019 Annual Meeting & Postgraduate Course , 2019
Authors: Johnston Thomas, Lee Simon, Krishnamurthy Ramkumar, Krishnamurthy Rajesh, Hor Kan
Keywords: Cardiomyopathy, LGE