Correlation of Heart Rate and Cardiac Dysfunction in Duchenne Muscular Dystrophy

Thomas, Tamara O.; Morgan, Thomas M.; Burnette, W. Bryan; Markham, Larry W.

doi:10.1007/s00246-012-0281-0

Cited by 52 publications

(56 citation statements)

References 26 publications

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“…For the calculation of the energy expenditure, the heart rates and the gait V ave were considered. In accordance with the literature 19 , the RHR of the evaluated patients was higher than in the healthy group studied by Rose et al 1 . This result may be explained by the autonomic nervous system dysfunction, which occurs precociously in patients with DMD, causing decreased parasympathetic activity and/or increased sympathetic activity with the progression of the disease 20 .…”

Section: Discussionsupporting

confidence: 92%

Gait energy expenditure in children with Duchenne muscular dystrophy: case study

Souza¹,

Ferreira²,

Baptista³

et al. 2014

Fisioter. Pesqui.

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| This case study aimed to verify the model of Rose et al. 1 as a feasible to assess energy expenditure in gait of children with Duchenne muscular dystrophy (DMD). Three DMD patients aged 6, 7 and 8 years old participated of this study. It was obtained weight, height, leg length measurement (LLM), resting and gait heart rate (HR) held on as 55-meter oval circuit performed during a two-minute test at each speed. Energy expenditure was calculated using the HR. It was performed a descriptive analysis (average) and these were compared, individually, to normative data. The average gait speed of these three patients was similar to the normative data for slow speed and lower considering comfortable and fast speed. The energy expenditure to slow speed of the patients 2 and 3 was similar to the normality, and lowest for patient 1; at comfortable speed, the energy expenditure obtained for all patients was similar; at fast speed, the patients 1 and 2 presented similar to normal values, but the patient 3 presented higher energy expenditure. It was concluded that the energy expenditure evaluation using HR was easily executed in the clinical practice and it can help therapeutic choices. For patient 3, an aerobic training could be indicated and for the others, they could keep the routine assessments.

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Section: Discussionsupporting

confidence: 92%

Gait energy expenditure in children with Duchenne muscular dystrophy: case study

Souza¹,

Ferreira²,

Baptista³

et al. 2014

Fisioter. Pesqui.

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“…The average heart rate at enrollment and the reduction of average heart rate correlated with a positive change in EF. 124 Thomas et al 229 initially showed that boys with DMD with normal LV function but with elevated heart rate were more likely to progress to cardiomyopathy than those in the lower quartiles for heart rate. This was followed by the finding that autonomic dysfunction before the onset of HF was associated with myocardial fibrosis, which suggests a role for earlier treatment.…”

Section: β-Adrenergic Blockadementioning

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

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211

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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“…An elevated resting heart rate (HR) is frequently observed early in the natural history of DMD associated cardiac disease [5,19], prior to the onset of changes in ejection fraction (EF), and this elevated resting HR may be a form of abnormal heart rate variability (HRV) implicating autonomic dysfunction. HRV has been used in multiple diseases as a measure of cardiac autonomic activity and has been associated with various morbidity and mortality outcomes in acute myocardial infarction [1,2,20], congenital heart disease [10], diabetic neuropathy [15], congestive heart failure, [4,17] and amyotrophic lateral sclerosis [16] but limited data are available evaluating HRV variables in DMD patients.…”

Section: Introductionmentioning

confidence: 99%

Autonomic Dysfunction: A Driving Force for Myocardial Fibrosis in Young Duchenne Muscular Dystrophy Patients?

et al. 2014

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Cardiac manifestations of Duchenne muscular dystrophy (DMD) include progressive cardiac dysfunction and an elevated resting heart rate (HR). We hypothesized this elevated HR reflects autonomic dysfunction that can be identified by heart rate variability (HRV) analyses which will be associated with myocardial fibrosis by cardiac magnetic resonance imaging (cMR). DMD patients (N = 74) and controls (N = 17) had time and frequency domain HRV analyses calculated via Holter monitoring. Cardiac magnetic resonance imaging was performed on DMD cases only. χ (2) test, T test, ANOVA, and logistic regression were used to perform comparisons between groups. A p value of <0.05 was used for statistical significance. DMD cases had higher resting average HR than controls (99.4 ± 8.9, 85.4 + 6.2, p < 0.001). Among HRV variables, decreases were seen in the following: standard deviation of R to R intervals, the percent RR intervals differing by >50 ms from previous RR interval, the root-meansquare of successive differences of RR intervals, the standard deviation of the mean R to R segment (SDANN), low frequency, and high frequency domain, all p values 0.001. Maximum HR and SDANN most significantly associated with positive LGE on cMR (p = 0.008, p = 0.016). DMD cases on beta blocker had an average HR lower than those not on beta blocker (p = 0.009), but with no difference in HRV analysis. DMD patients have reduced HRV and therefore autonomic dysfunction prior to the onset of heart failure which is associated with myocardial fibrosis.

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Correlation of Heart Rate and Cardiac Dysfunction in Duchenne Muscular Dystrophy

Cited by 52 publications

References 26 publications

Gait energy expenditure in children with Duchenne muscular dystrophy: case study

Gait energy expenditure in children with Duchenne muscular dystrophy: case study

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

Autonomic Dysfunction: A Driving Force for Myocardial Fibrosis in Young Duchenne Muscular Dystrophy Patients?

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