Comparison of right and left ventricular function and size in Duchenne muscular dystrophy

Mehmood, Muddassir; Hor, Kan N.; Al‐Khalidi, Hussein R.; Benson, D. Woodrow; Jefferies, John L.; Taylor, Michael D.; Egnaczyk, Gregory F.; Raman, Subha V.; Basu, Sanmit K.; Cripe, Linda H.; Germann, Joshua; Mazur, Wojciech

doi:10.1016/j.ejrad.2015.07.007

Cited by 22 publications

(18 citation statements)

References 30 publications

(24 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Compared to other causes of dilated cardiomyopathy, DMD and BMD have less ventricular dilation early in the course of disease, simply beginning with dysfunction without dilation. [11][12][13] For patients with DMD, cardiac disease is the primary cause of mortality in >20%, 14 and previous observational studies have shown that increasing cardiac dysfunction is correlated with increasing age and severity of skeletal muscle disease. 15,16 Recent studies have suggested that the average age for development of abnormal left ventricular ejection fraction (LVEF) is 14.3 years.…”

Section: 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

Self Cite

211

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

Section: 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

Self Cite

211

195

View full text Add to dashboard Cite

show abstract

“…Twenty-seven boys and adolescent and young adult males (median age 15 years; range, [9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26] were included in the study. Most of the participants had 3 or 4 clinic visits (median, 3; range, 1-10).…”

Section: Resultsmentioning

confidence: 99%

“…Mehmood et al . conducted a cross‐sectional study using cardiac MRI to compare LV and right ventricular (RV) systolic function in children with DMD and found that LV estimates were superior diagnostically to RV variables because of a greater prediction ability of mortality and an increased rate of decline.…”

Section: Discussionmentioning

confidence: 99%

“…Mehmood et al 26 conducted a cross-sectional study using cardiac MRI to compare LV and right ventricular (RV) systolic function in children with DMD and found that LV estimates were superior diagnostically to RV variables because of a greater prediction ability of mortality and an increased rate of decline. Moreover, at most centers, transthoracic ECHO performed as part of standard clinical care does not include RV functional assessment because RV variables are difficult to assess because of geometrical complexities.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Association between pulmonary function and left ventricular volume and function in duchenne muscular dystrophy

et al. 2019

View full text Add to dashboard Cite

Introduction: Duchenne muscular dystrophy (DMD) is characterized by absence of the subsarcolemmal protein dystrophin, present in skeletal muscles and cardiomyocytes. We hypothesized that progressive respiratory and left ventricular (LV) insufficiencies in DMD could be parallel and interrelated phenomena. Methods: We conducted a retrospective chart review of 27 patients with DMD. Our primary objective was to compare the rates of decline between pulmonary function test (PFT) measures (forced expiratory volume in the first second, forced vital capacity, peak expiratory flow rate, maximal inspiratory/expiratory pressure) and echocardiographic estimates of LV end‐diastolic volume and LV ejection fraction. Results: The rates of decline/year of PFTs and LV estimates were not significantly different. Pulmonary function test measures of ventilatory efficiency and strength had strong intercorrelations. Pulmonary function tests and LV estimates had weak but statistically significant correlations. Discussion: A comparable rate of decline in PFTs and LV indices in DMD provides evidence for concurrently progressive deterioration in respiratory and LV functions. Muscle Nerve, 2019

show abstract

“…However, in a larger series of DMD patients right ventricular function and ventricular size was relatively preserved [5]. Not only the myocardiocytes but also the cells of the cardiac conduction system may be involved thus leading to arrhythmias and conduction disturbances.…”

mentioning

confidence: 99%

Expanded Diagnostic and Therapeutic Options for Cardiac Disease in Duchenne Muscular Dystrophy

Stöllberger

Finsterer

2018

JND

View full text Add to dashboard Cite

With interest we read the article by Fayssoil et al. about cardiac involvement classification and therapy in patients with Duchenne muscular dystrophy (DMD) [1]. We agree with the authors that search for cardiac involvement and cardiac therapy are important in the care of patients with DMD, however we want to add some comments and suggestions.Regarding symptoms, it is mentioned that "classical" symptoms of heart failure like exertional dyspnea or symptoms of arrhythmias like palpitations may be absent in DMD because of the limited mobility. Thus, instrumental investigations to assess the cardiac function are important. The authors recommend electrocardiogram, echocardiography and cardiac magnetic resonance imaging. However, the armamentarium of the cardiologists to detect cardiac dysfunction is larger. First, we recommend measurement of the serum levels of brain natriuretic peptide (BNP) which is a robust indicator for cardiac dysfunction, useful for diagnostic purposes and for control of therapeutic effects, as recommended by current heart failure guidelines [2]. Secondly, we suggest that in patients with suspected arrhythmia 24-Holter monitoring or, if the arrhythmias occur only rarely, implantable loop recorders (ILRs) should be considered. ILRs are small devices, implanted or injected subcutaneously in the left side of the chest under local anaesthesia. They continuously record and delete a

show abstract

Comparison of right and left ventricular function and size in Duchenne muscular dystrophy

Cited by 22 publications

References 30 publications

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

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

Association between pulmonary function and left ventricular volume and function in duchenne muscular dystrophy

Expanded Diagnostic and Therapeutic Options for Cardiac Disease in Duchenne Muscular Dystrophy

Contact Info

Product

Resources

About