2019
DOI: 10.3233/jnd-190415
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The Added Value of Cardiac Magnetic Resonance in Muscular Dystrophies

Abstract: Muscular dystrophies (MD) represent a heterogeneous group of rare genetic diseases that often lead to significant weakness due to progressive muscle degeneration. In many forms of MD, cardiac manifestations including heart failure, atrial and ventricular arrhythmias and conduction abnormalities can occur and may be a predominant feature of the disease. Cardiac magnetic resonance (CMR) can assess cardiac anatomy, global and regional ventricular function, volumes and mass as well as presence of myocardial inflam… Show more

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Cited by 16 publications
(3 citation statements)
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“…20) Most importantly, CMR can detect myocardial fi-brosis through late gadolinium enhancement (LGE), which has been shown to precede LV dysfunction and clinically overt cardiac involvement in other muscular dystrophies. 21) Despite its promising role in cardiac evaluation, CMR imaging is still not commonly employed for cardiac screening in dysferlinopathies. In two CMR studies by Choi, et al 12) and Rosales, et al, 13) myocardial fibrosis was present in four out of five MM and three out of nine patients with LGMD2B, respectively.…”
Section: Discussionmentioning
confidence: 99%
“…20) Most importantly, CMR can detect myocardial fi-brosis through late gadolinium enhancement (LGE), which has been shown to precede LV dysfunction and clinically overt cardiac involvement in other muscular dystrophies. 21) Despite its promising role in cardiac evaluation, CMR imaging is still not commonly employed for cardiac screening in dysferlinopathies. In two CMR studies by Choi, et al 12) and Rosales, et al, 13) myocardial fibrosis was present in four out of five MM and three out of nine patients with LGMD2B, respectively.…”
Section: Discussionmentioning
confidence: 99%
“…Given that, for example in DMD the symptomatic therapy against loss of ambulation, kyphoscoliosis, and respiratory dysfunction has considerably improved over the last decades, cardiac involvement became increasingly important as it is nowadays a leading cause of death in patients with DMD (3). As cardiac magnetic resonance (CMR) imaging is the non-invasive reference standard for left ventricular (LV) function assessment and allows myocardial tissue characterization (4), recently several studies have investigated cardiac involvement in patients with DMD and BMD by CMR (5,6). However, in rarer muscular dystrophies, such as in LGMD, less CMR data about cardiac involvement exist.…”
Section: Introductionmentioning
confidence: 99%
“…5 The early identification of cardiac involvement in patients with Duchenne muscular dystrophy (DMD) is important as it allows for a timely in troduction of cardioprotective therapy to slow down the progression of heart failure and to reduce symptoms. 6 Based on scarce data from limited cohort studies, it is the fibrosis that is the most frequent and relevant abnormality evolving throughout patients' lifetime.…”
mentioning
confidence: 99%