Upper arm and cardiac magnetic resonance imaging in Duchenne muscular dystrophy

Gaur, Lasya; Hanna, Alexander; Bandettini, W. Patricia; Fischbeck, Kenneth H.; Arai, Andrew E.; Mankodi, Ami

doi:10.1002/acn3.367

Cited by 15 publications

(23 citation statements)

References 29 publications

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“…However, in another study, the majority of DMD subjects had normal or decreased T2 . Yet another study using quantitative maps of T1 and T2 relaxation times and muscle fat fraction measurements of the upper arm skeletal muscles and heart in ambulatory boys with DMD and age‐matched healthy volunteers, the cardiac‐optimized sequences detected fatty infiltration and oedema in the upper arm skeletal muscles but not the myocardium in these DMDs with normal ejection fraction …”

Section: Cmr Indices For Dmd Evaluationmentioning

confidence: 89%

“…Furthermore, patients with DMD may be more prone to myocarditis than control subjects, because the abnormal dystrophin behaves as a vehicle for different viruses . However, during the early stages, it may remain undetected by echocardiography, which is unable to perform tissue abnormalities, occurring without associated changes in LVEF; furthermore, the diagnosis of myocarditis can be missed, because an increase in cardiac troponin was detected in only 20% of cases …”

Section: Cmr Indices For Dmd Evaluationmentioning

confidence: 99%

“…53 Yet another study using quantitative maps of T1 and T2 relaxation times and muscle fat fraction measurements of the upper arm skeletal muscles and heart in ambulatory boys with DMD and age-matched healthy volunteers, the cardiac-optimized sequences detected fatty infiltration and oedema in the upper arm skeletal muscles but not the myocardium in these DMDs with normal ejection fraction. 54 Finally, the evaluation of extracellular cardiac volume (ECV) in patients with DMD is a useful tool to assess the total extent of myocardial fibrosis, as well as to depict subtle, diffuse fibrosis in areas of normal-appearing myocardium on LGE images. Thus, myocardial ECV is a potential additional quantitative tool for accurate detection of cardiac involvement and risk stratification in patients with DMD.…”

Section: T1 Mappingmentioning

confidence: 99%

See 2 more Smart Citations

Oedema‐fibrosis in Duchenne Muscular Dystrophy: Role of cardiovascular magnetic resonance imaging

Mavrogeni

Papavasiliou

Giannakopoulou

et al. 2017

Eur J Clin Investigation

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Duchenne muscular dystrophy (DMD) is an X-linked muscle disorder characterized by progressive, irreversible loss of cardiac and skeletal muscular function.Muscular enlargement in DMD is attributed to oedema, due to the increased cytoplasmic Na+ concentration. The aim of this review was to present the current experience and emphasize the role of cardiovascular magnetic resonance (CMR) in the diagnosis of this condition. DMD patients' survival depends on ventilatory assistance, as respiratory muscle dysfunction was the most common cause of death in the past. Currently, due to improved ventilatory assistance, cardiomyopathy has become the main cause of death, even though clinically overt heart failure may be absent. CMR is the technique of choice to assess the pathophysiologic phenomena taking place in DMD, such as myocardial oedema and subepicardial fibrosis. The classic index to assess oedema is the T2-weighted short-tau inversion recovery (T2w-STIR), as it suppresses the signal from flowing blood and resident fat and enhances sensitivity to tissue fluid. Furthermore, CMR is the most reliable technique to detect and quantify fibrosis in DMD. Recently, the new indices T2, T1 mapping (native and postcontrast) and the extracellular volume (ECV) allow a more accurate approach of myocardial oedema and fibrosis. To conclude, the assessment of cardiac oedema and subepicardial fibrosis in the inferolateral wall of the left heart ventricle are the most important early finding in DMD with preserved ventricular function, and CMR, using both the classic and the new indices, is the best technique to detect and monitor these lesions.

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Section: Cmr Indices For Dmd Evaluationmentioning

confidence: 89%

Section: Cmr Indices For Dmd Evaluationmentioning

confidence: 99%

Section: T1 Mappingmentioning

confidence: 99%

See 1 more Smart Citation

Oedema‐fibrosis in Duchenne Muscular Dystrophy: Role of cardiovascular magnetic resonance imaging

Mavrogeni

Papavasiliou

Giannakopoulou

et al. 2017

Eur J Clin Investigation

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“…The reduction in the chest wall motion may also be related to alterations in the elasticity of the lungs associated with diminished lung volumes 32. Note that our patients did not have parenchymal lung disease, thoracic spine kyphoscoliosis, or congestive heart failure,15 which are known to cause reduction in residual volume.…”

Section: Discussionmentioning

confidence: 88%

Respiratory magnetic resonance imaging biomarkers in Duchenne muscular dystrophy

Mankodi

Kovacs

Norato

et al. 2017

Ann Clin Transl Neurol

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ObjectiveTo examine the diaphragm and chest wall dynamics with cine breathing magnetic resonance imaging (MRI) in ambulatory boys with Duchenne muscular dystrophy (DMD) without respiratory symptoms and controls.MethodsIn 11 DMD boys and 15 controls, cine MRI of maximal breathing was recorded for 10 sec. The lung segmentations were done by an automated pipeline based on a Holistically‐Nested Network model (HNN method). Lung areas, diaphragm, and chest wall motion were measured throughout the breathing cycle.ResultsThe HNN method reliably identified the contours of the lung and the diaphragm in every frame of each dataset (~180 frames) within seconds. The lung areas at maximal inspiration and expiration were reduced in DMD patients relative to controls (P = 0.02 and <0.01, respectively). The change in the lung area between inspiration and expiration correlated with percent predicted forced vital capacity (FVC) in patients (r s = 0.75, P = 0.03) and was not significantly different between groups. The diaphragm position, length, contractility, and motion were not significantly different between groups. Chest wall motion was reduced in patients compared to controls (P < 0.01).InterpretationCine breathing MRI allows independent and reliable assessment of the diaphragm and chest wall dynamics during the breathing cycle in DMD patients and controls. The MRI data indicate that ambulatory DMD patients breathe at lower lung volumes than controls when their FVC is in the normal range. The diaphragm moves normally, whereas chest wall motion is reduced in these boys with DMD.

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“…In another MRI study, fatty infiltration and oedema were detected in the upper arm skeletal muscles but not in the myocardium in DMD patients. The authors suggested that imaging the heart and skeletal muscle using the same magnetic resonance methods during a single scan may be useful to assess relative disease status and therapeutic response in clinical trials.…”

Section: Mri For Heart Muscles and Brainmentioning

confidence: 99%

Brain and heart magnetic resonance imaging/spectroscopy in duchenne muscular dystrophy

Mavrogeni

Pons

Nikas

et al. 2017

Eur J Clin Investigation

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Duchenne muscular dystrophy (DMD) is an X-linked muscle disorder characterized by progressive and irreversible loss of muscular function. As muscular disease progresses, the repair mechanisms cannot compensate for cellular damage, leading inevitably to necrosis and progressive replacement by fibrous and fatty tissue. Cardiomyopathy and respiratory failure are the main causes of death in DMD. In addition to the well-described muscle and heart disease, cognitive dysfunction affects around 30% of DMD boys. Myocardial fibrosis, assessed by late gadolinium enhancement (LGE), using cardiovascular magnetic resonance imaging (CMR), is an early marker of heart involvement in both DMD patients and female carriers. In parallel, brain MRI identifies smaller total brain volume, smaller grey matter volume, lower white matter fractional anisotropy and higher white matter radial diffusivity in DMD patients. The in vivo brain evaluation of mdx mice, a surrogate animal model of DMD, showed an increased inorganic phosphate (P(i))/phosphocreatine (PCr) and pH. In this paper, we propose a holistic approach using techniques of magnetic resonance imaging, spectroscopy and diffusion tensor imaging as a tool to create a "heart and brain imaging map" in DMD patients that could potentially facilitate the patients' risk stratification and also future research studies in the field. K E Y W O R D Sbrain magnetic resonance imaging, brain magnetic resonance spectroscopy, cardiovascular magnetic resonance imaging, diffusion tensor imaging, duchenne muscular dystrophy

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Upper arm and cardiac magnetic resonance imaging in Duchenne muscular dystrophy

Cited by 15 publications

References 29 publications

Oedema‐fibrosis in Duchenne Muscular Dystrophy: Role of cardiovascular magnetic resonance imaging

Oedema‐fibrosis in Duchenne Muscular Dystrophy: Role of cardiovascular magnetic resonance imaging

Respiratory magnetic resonance imaging biomarkers in Duchenne muscular dystrophy

Brain and heart magnetic resonance imaging/spectroscopy in duchenne muscular dystrophy

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