Regional Circumferential Strain is a Biomarker for Disease Severity in Duchenne Muscular Dystrophy Heart Disease: A Cross-Sectional Study

Hor, Kan N.; Kissoon, Narayan R.; Mazur, Wojciech; Gupta, Rahul; Ittenbach, Richard F.; Al‐Khalidi, Hussein R.; Cripe, Linda H.; Raman, Subha V.; Puchalski, Michael D.; Gottliebson, William; Benson, D. Woodrow

doi:10.1007/s00246-014-0972-9

Cited by 30 publications

(48 citation statements)

References 34 publications

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“…In our study results, children with DMD demonstrated significantly lower circumferential strain than healthy controls, as mentioned in other studies. 7 10 Hor, et al 7 reported that circumferential strain at the LV free wall on cardiac MRI was significantly lower in patients with DMD than in controls, despite the LV EFs in children with DMD being within normal limits. Ryan, et al 10 published a study that found that all circumferential strain is significantly decreased in patients with DMD, particularly in the anteroseptal, inferior, and inferolateral myocardial segments.…”

Section: Discussionmentioning

confidence: 99%

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Early Marker of Myocardial Deformation in Children with Duchenne Muscular Dystrophy Assessed Using Echocardiographic Myocardial Strain Analysis

Eun

Jung

et al. 2016

Yonsei Med J

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PurposeAs cardiomyopathy is more prevalent and currently the leading cause of death in Duchenne muscular dystrophy (DMD), early detection of myocardial involvement is important. The purpose of this study was to analyze myocardial strain in DMD children, for the possibility of early detection of myocardial dysfunction.Materials and MethodsWe reviewed medical records of DMD patients who were >10 years of age (15.6±1.6 years, 12.5–18 years), from March 2013 to June 2014. Data of 24 DMD children who underwent echocardiography with three-layer specific myocardial strain were compared with 24 controls (age: 9.3±4.0 years, 5.5–17 years).ResultsEpicardial longitudinal strain was lower in DMD (DMD: -9.3±3.8%; control: -12.3±4.3%; p=0.012). Radial strain (DMD: 24.1±11.1%; control: 37.3±25.9%; p=0.027) and strain rate (SR) (DMD: 1.68±0.91; control: 2.42±0.84; p=0.006) on parasternal short axis view were lower in DMD. Circumferential strains in the endocardium (DMD: -17.5±4.7%; control: -24.2±5.3%; p<0.001), myocardium (DMD: -12.7±3.8%; control: -18.0±4.0%; p<0.001), and epicardium (DMD: -8.4±4.0%; control: -12.2±5.0%; p=0.006) were significantly decreased in DMD. Circumferential SRs were lower in the endocardial (DMD: -1.46±0.38; control: -1.78±0.27; p=0.002) and myocardial layers (DMD: 1.02±0.27; control: -1.28±0.22; p=0.001).ConclusionIn DMD patients, deteriorations in myocardial circumferential strain might be an indicator for predicting cardiomyopathy.

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Section: Discussionmentioning

confidence: 99%

“…It was demonstrated that patients with DMD who were <10 years of age showed lower LV myocardial circumferential strain on cardiac MRI than healthy children. 7 …”

Section: Introductionmentioning

confidence: 99%

Early Marker of Myocardial Deformation in Children with Duchenne Muscular Dystrophy Assessed Using Echocardiographic Myocardial Strain Analysis

Eun

Jung

et al. 2016

Yonsei Med J

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“…162 Abnormalities in segmental circumferential strain, detectable by echocardiography and CMR, may also be an early biomarker of myocardial dysfunction in DMD, preceding the development of myocardial fibrosis. 147,163 There is also emerging evidence that female DMD, and to a lesser extent BMD, carriers have subclinical cardiac involvement, characterized by reduced LVEF or subepicardial LV lateral free wall LGE on CMR. 130 Although CMR imaging does not suffer from the limitation of poor acoustic windows attributable to body habitus or lung disease, there are factors that may limit its utility for imaging patients with NMDs.…”

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

205

195

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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...

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“…Cardiac magnetic resonance has been used to measure strain. However, it may be challenging in patients with DMD, especially in young children who cannot cooperate during the examination [ 28 ]. In the past, echocardiographers measured myocardial strain using tissue Doppler imaging, which is limited by poor echocardiographic windows and the probe angle [ 27 ].…”

Section: Discussionmentioning

confidence: 99%

Usefulness of speckle-tracking echocardiography for early detection in children with Duchenne muscular dystrophy: a meta-analysis and trial sequential analysis

Song

Zhang

Wang

et al. 2020

Cardiovasc Ultrasound

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Background Duchenne muscular dystrophy (DMD) is the most common form of inherited muscle disease in children. The incidence of cardiomyopathy induced by DMD increases with age. Left ventricular ejection fraction usually fails to reflect the subclinical left ventricular dysfunction. Several studies have assessed this dysfunction using myocardial strain measured by speckle-tracking echocardiography (STE). However, the results were inconsistent and incomplete. Methods Several databases were searched from their inception to February 5, 2020. The summarized weighted mean difference (WMD) with 95% confidence intervals (CIs) were estimated for myocardial strain between DMD and healthy controls and a meta-analysis was conducted. Trial sequential analysis estimated whether the resulting evidence was sufficient. Results Eight studies with a total of 269 DMD children and 299 healthy participants were included. STE revealed that global longitudinal strain (GLS), global circumferential strain, average longitudinal strain (measured by two-dimensional STE at the apical four-chamber view), and average circumferential strain (measured by two-dimensional STE at the papillary muscle short-axis level) decreased (WMD = 4.17, 95% CI: 3.03–5.32; WMD = 3.98, 95% CI: 0.29–7.68; WMD = 4.18, 95% CI: 2.75–5.62; and WMD = 4.90, 95% CI: 2.38–7.43, respectively; all P < 0.05) compared with the controls and global radial strain was unchanged in the DMD group (WMD = − 4.33, 95% CI: − 9.53–0.87, P = 0.103). Trial sequential analysis indicated that available GLS samples were sufficient and confirmed that adequate evidence was accumulated. The credibility of other myocardial strains was questioned due to insufficiently involved studies. Conclusion GLS can be useful for early detection of left ventricle myocardial dysfunction in children with DMD.

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Regional Circumferential Strain is a Biomarker for Disease Severity in Duchenne Muscular Dystrophy Heart Disease: A Cross-Sectional Study

Cited by 30 publications

References 34 publications

Early Marker of Myocardial Deformation in Children with Duchenne Muscular Dystrophy Assessed Using Echocardiographic Myocardial Strain Analysis

Early Marker of Myocardial Deformation in Children with Duchenne Muscular Dystrophy Assessed Using Echocardiographic Myocardial Strain Analysis

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

Usefulness of speckle-tracking echocardiography for early detection in children with Duchenne muscular dystrophy: a meta-analysis and trial sequential analysis

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