The value of cardiac MRI versus echocardiography in the pre-operative assessment of patients with Duchenne muscular dystrophy

Brunklaus, Andreas; Parish, Emma; Muntoni, Francesco; Scuplak, Stephen; Tucker, Stewart; Fenton, Matthew; Hughes, Marina; Manzur, Adnan

doi:10.1016/j.ejpn.2015.03.008

Cited by 49 publications

(40 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…However, this study did not assess the other echocardiographic methods of LV functional assessment. [8] In our study, we found a higher correlation between 2-dimensional FS and CMR LVEF than that of M-mode FS.…”

Section: Discussionsupporting

confidence: 48%

“…[6] Recent evaluations in patients with DMD have demonstrated moderate reproducibility of echocardiographic measures of LV function and a correlation coefficient of only 0.67 between fractional shortening (FS) by echocardiography and left ventricular ejection fraction (LVEF) by cardiac magnetic resonance imaging (CMR). [7, 8] Echocardiography and CMR have been compared in other disease processes and, in patients with myocardial infarction, echocardiography underestimates LV volumes and LVEF and is less sensitive in the detection of focal wall motion abnormalities. [9] CMR provides superior delineation of the border between the blood pool and endocardium and image quality is not affected by external factors such as body habitus.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Evaluation of Echocardiographic Measures of Left Ventricular Function in Patients with Duchenne Muscular Dystrophy: Assessment of Reproducibility and Comparison to Cardiac Magnetic Resonance Imaging

Soslow

Slaughter

et al. 2016

Journal of the American Society of Echocardiography

View full text Add to dashboard Cite

Background Patients with Duchenne muscular dystrophy (DMD) require frequent imaging to assess left ventricular (LV) function. Poor imaging windows can limit the diagnostic utility of echocardiography. Cardiac MRI (CMR) is the gold standard for assessment of LV function but has not been universally adopted in DMD patients. The study objectives were: 1) evaluate reproducibility of echocardiographic measures of LV function; 2) evaluate which echocardiographic methods correlate best with CMR LVEF; 3) evaluate whether CMR provides additional value compared with echocardiography. Methods 28 DMD participants prospectively underwent echocardiography and CMR. Two blinded readers measured: fractional shortening (FS) from M-mode and 2-dimensional images and LV ejection fraction (LVEF) using 4-chamber, biplane Simpson, 5/6 area-length, and 3-dimensional methods. Speckle tracking echocardiography was used to analyze circumferential strain. Readers subjectively rated function and segmental wall motion. Agreement was assessed using intraclass correlation coefficient, Bland-Altman plots, Spearman correlation, and weighted Kappa. Results 2-dimensional FS and 5/6 area-length LVEF had the best combination of reproducibility and correlation with CMR LVEF, though both misclassified approximately 20% as either normal or abnormal function. Other measures of LV function were less reproducible with worse correlation to CMR LVEF. 37% of segments not visible on echocardiography were felt to have wall motion abnormalities by CMR. Conclusions 2-dimensional FS and 5/6 area-length LVEF represent the most accurate and reproducible echocardiographic measures of LV function in patients with DMD. CMR should be considered when neither of these techniques are measurable or when it is necessary to detect more subtle cardiovascular changes.

show abstract

Section: Discussionsupporting

confidence: 48%

Section: Introductionmentioning

confidence: 99%

Evaluation of Echocardiographic Measures of Left Ventricular Function in Patients with Duchenne Muscular Dystrophy: Assessment of Reproducibility and Comparison to Cardiac Magnetic Resonance Imaging

Soslow

Slaughter

et al. 2016

Journal of the American Society of Echocardiography

View full text Add to dashboard Cite

show abstract

“…144,[149][150][151][152] An important limitation in the application of standard transthoracic echocardiography in the care of patients with DMD and BMD is the diminishment of imaging (acoustic) windows because of progression of scoliosis or obesity with advancing age. 153 Thus, the use of other imaging modalities, such as cardiac magnetic resonance imaging (CMR), might better fully characterize the severity of cardiac involvement in older patients. 153 The role of CMR in the assessment of patients with DMD and BMD is evolving.…”

Section: Cardiac Evaluation In Dmd and Bmdmentioning

confidence: 99%

“…153 Thus, the use of other imaging modalities, such as cardiac magnetic resonance imaging (CMR), might better fully characterize the severity of cardiac involvement in older patients. 153 The role of CMR in the assessment of patients with DMD and BMD is evolving. CMR is capable of providing objective 3-dimensional anatomic analysis, including assessment of ventricular volumes, EFs, and hypokinesia.…”

Section: Cardiac Evaluation In Dmd and Bmdmentioning

confidence: 99%

Management of Cardiac Involvement Associated With Neuromuscular Diseases: A Scientific Statement From the American Heart Association

Feingold¹,

Mahle²,

Auerbach³

et al. 2017

Circulation

220

195

View full text Add to dashboard Cite

For many neuromuscular diseases (NMDs), cardiac disease represents a major cause of morbidity and mortality. The management of cardiac disease in NMDs is made challenging by the broad clinical heterogeneity that exists among many NMDs and by limited knowledge about disease-specific cardiovascular pathogenesis and course-modifying interventions. The overlay of compromise in peripheral muscle function and other organ systems, such as the lungs, also makes the simple application of endorsed adult or pediatric heart failure guidelines to the NMD population problematic. In this statement, we provide background on several NMDs in which there is cardiac involvement, highlighting unique features of NMD-associated myocardial disease that require clinicians to tailor their approach to prevention and treatment of heart failure. Undoubtedly, further investigations are required to best inform future guidelines on NMD-specific cardiovascular health risks, treatments, and outcomes. N euromuscular diseases (NMDs) encompass a broad spectrum of diagnoses with overlapping but distinct phenotypes. Common to many NMDs is cardiac involvement. Although the past 3 decades have seen marked advances in our understanding of many NMDs, significant gaps in knowledge remain on how best to approach cardiac care in these patients. For example, survival in Duchenne muscular dystrophy (DMD) has been extended through the use of glucocorticoid use and respiratory support, yet cardiac complications remain a significant cause of morbidity and mortality.1-3 To achieve further gains in care, we will need to improve our understanding of the pathophysiologies driving cardiac involvement in NMDs and advance treatments aimed at preventing the progression of heart failure (HF) and sudden death in NMDs. The recently published findings of an expert working group on cardiac involvement in DMD, which outlined key gaps in knowledge and made specific recommendations aimed at improving diagnosis and management, are a step toward this goal. 4 In this statement, we include a comprehensive overview of the major categories of NMDs with cardiac involvement. For each, a brief background of the gene defect(s), common clinical manifestations (particularly cardiac findings), and current therapies is summarized. Gaps in knowledge are highlighted, and where possible, clinical treatment suggestions are made by the expert writing group appointed by the American Heart Association to review the available literature. Selection of the writing group was performed in accordance with the American Heart Association's conflict-of-interest management policy. Participants volunteered to write sections relevant to their expertise and experience. CLINICAL STATEMENTS AND GUIDELINESconducted a general search of the literature, restricted to human subjects research published between 1980 and 2016. Drafts of each section were written and sent to the chair of the writing group for incorporation into a single document, which was then edited. The edited document was discussed electron...

show abstract

“…This is not the case in human trials, where minimally invasive monitoring methods are necessary. Currently such noninvasive methods include the evaluation of patients’ muscles’ physical capacity1415, MRI based functional assessments of cardiac and skeletal muscles161718, and quantification of circulating biomarkers. The most commonly used circulating biomarker for MD is serum muscle creatine kinase (mCK), which leaks into the blood stream upon muscle damage.…”

mentioning

confidence: 99%

Circulating miRNAs are generic and versatile therapeutic monitoring biomarkers in muscular dystrophies

Israeli¹,

Poupiot²,

Amor³

et al. 2016

Sci Rep

View full text Add to dashboard Cite

The development of medical approaches requires preclinical and clinical trials for assessment of therapeutic efficacy. Such evaluation entails the use of biomarkers, which provide information on the response to the therapeutic intervention. One newly-proposed class of biomarkers is the microRNA (miRNA) molecules. In muscular dystrophies (MD), the dysregulation of miRNAs was initially observed in muscle biopsy and later extended to plasma samples, suggesting that they may be of interest as biomarkers. First, we demonstrated that dystromiRs dysregulation occurs in MD with either preserved or disrupted expression of the dystrophin-associated glycoprotein complex, supporting the utilization of dystromiRs as generic biomarkers in MD. Then, we aimed at evaluation of the capacity of miRNAs as monitoring biomarkers for experimental therapeutic approach in MD. To this end, we took advantage of our previously characterized gene therapy approach in a mouse model for α-sarcoglycanopathy. We identified a dose-response correlation between the expression of miRNAs on both muscle tissue and blood serum and the therapeutic benefit as evaluated by a set of new and classically-used evaluation methods. This study supports the utility of profiling circulating miRNAs for the evaluation of therapeutic outcome in medical approaches for MD.

show abstract

The value of cardiac MRI versus echocardiography in the pre-operative assessment of patients with Duchenne muscular dystrophy

Cited by 49 publications

References 29 publications

Evaluation of Echocardiographic Measures of Left Ventricular Function in Patients with Duchenne Muscular Dystrophy: Assessment of Reproducibility and Comparison to Cardiac Magnetic Resonance Imaging

Evaluation of Echocardiographic Measures of Left Ventricular Function in Patients with Duchenne Muscular Dystrophy: Assessment of Reproducibility and Comparison to Cardiac Magnetic Resonance Imaging

Management of Cardiac Involvement Associated With Neuromuscular Diseases: A Scientific Statement From the American Heart Association

Circulating miRNAs are generic and versatile therapeutic monitoring biomarkers in muscular dystrophies

Contact Info

Product

Resources

About