Left ventricular systolic function and the pattern of late-gadolinium-enhancement independently and additively predict adverse cardiac events in muscular dystrophy patients

Florian, Anca; Ludwig, Anna; Engelen, Markus A.; Waltenberger, Johannes; Rösch, Sabine; Sechtem, Udo; Yılmaz, Ali

doi:10.1186/s12968-014-0081-1

Cited by 92 publications

(64 citation statements)

References 32 publications

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“…Further evidence has emerged showing that particularly an impaired LVEF and a "transmural" pattern of LGE appear to predict the occurrence of adverse cardiac events in DMD patients. 20 The question remains how to clinically interpret findings of low grade LGE when this is mostly associated with normal cardiac function, or the rare case with extensive LGE but relatively preserved LVEF? LGE is based on the delayed washin and wash-out of gadolinium in myocardial areas with increased extracellular space due to fibrosis.…”

Section: Discussionmentioning

confidence: 99%

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

Brunklaus¹,

Parish²,

Muntoni³

et al. 2015

European Journal of Paediatric Neurology

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Section: Discussionmentioning

confidence: 99%

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

Brunklaus¹,

Parish²,

Muntoni³

et al. 2015

European Journal of Paediatric Neurology

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“…More recently, clinical implications of LGE in DMD were demonstrated when Florian et al [24] showed that “transmural” LGE was associated with more adverse events in muscular dystrophy subjects and Tandon et al [25] found correlations between the presence of LGE, LVEF and genotype. Soslow et al also looked at post-contrast T1 ratios and found lower ratios in DMD subjects compared to controls, even in LGE negative subjects [26].…”

Section: Discussionmentioning

confidence: 99%

Native T1 values identify myocardial changes and stratify disease severity in patients with Duchenne muscular dystrophy

Olivieri

Kellman

McCarter

et al. 2016

Journal of Cardiovascular Magnetic Resonance

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BackgroundDuchenne muscular dystrophy (DMD) is an X-linked, inherited disorder causing dilated cardiomyopathy with variable onset and progression. Currently we lack objective markers of the effect of therapies targeted towards preventing progression of subclinical cardiac disease. Thus, our aim was to compare the ability of native T1 and extracellular volume (ECV) measurements to differentiate risk of myocardial disease in DMD and controls.MethodsTwenty boys with DMD and 16 age/gender-matched controls without history predisposing to cardiac fibrosis, but with a clinical indication for cardiovascular magnetic resonance (CMR) evaluation, underwent CMR with contrast. Data points collected include left ventricular ejection fraction (LVEF), left ventricular mass, and presence of late gadolinium enhancement (LGE). Native T1, and ECV regional mapping were obtained using both a modified Look-Locker (MOLLI) and saturation recovery single shot sequence (SASHA) on a 1.5T scanner. Using ordinal logistic regression models, controlling for age and LVEF, LGE-free septal we evaluated the ability native T1 and ECV assessments to differentiate levels of cardiomyopathy.ResultsTwenty DMD subjects aged 14.4 ± 4 years had an LVEF of 56.3 ± 7.4 %; 12/20 had LGE, all confined to the lateral wall. Sixteen controls aged 16.1 ± 2.2 years had an LVEF 60.4 ± 5.1 % and no LGE. Native T1 and ECV values were significantly higher in the DMD group (p < 0.05) with both MOLLI and SASHA imaging techniques. Native T1 demonstrated a 50 % increase in the ability to predict disease state (control, DMD without fibrosis, DMD with fibrosis). ECV demonstrated only the ability to predict presence of LGE, but could not distinguish between controls and DMD without fibrosis.ConclusionsLGE-spared regions of boys with DMD have significantly different native T1 and ECV values compared to controls. Native T1 measurements can identify early changes in DMD patients without the presence of LGE and help predict disease severity more effectively than ECV. Native T1 may be a novel outcome measure for early cardiac therapies in DMD and other cardiomyopathies.

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“…148,[158][159][160] Myocardial fibrosis burden, quantified as number of LGE-positive myocardial segments, is associated with decline in EF, 161 and transmural LGE was also recently reported to have additive value above LVEF alone in predicting hospitalization for HF or occurrence of ventricular tachycardia. 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.…”

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

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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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Left ventricular systolic function and the pattern of late-gadolinium-enhancement independently and additively predict adverse cardiac events in muscular dystrophy patients

Cited by 92 publications

References 32 publications

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

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

Native T1 values identify myocardial changes and stratify disease severity in patients with Duchenne muscular dystrophy

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

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