Effects of Angiotensin-Converting Enzyme Inhibitors and/or Beta Blockers on the Cardiomyopathy in Duchenne Muscular Dystrophy

Viollet, Laurence; Thrush, Philip T.; Flanigan, Kevin M.; Mendell, Jerry R.; Allen, Hugh D.

doi:10.1016/j.amjcard.2012.02.064

Cited by 136 publications

(111 citation statements)

References 14 publications

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“…Several studies have demonstrated the importance of initiating pharmacotherapy for cardiomyopathy prior to the onset of symptoms, in order to improve the morbidity and mortality of DMD. 10,[12][13][14] We found that although cardiac assessment had been performed in over two-thirds of patients with DMD, few were currently undergoing annual assessments and the treatment regime could be improved in the majority, in line with the new international guidelines. 15 The ECG abnormalities identified, such as conduction defects and signs consistent with hypertrophic or dilated cardiomyopathy, were consistent with the existing literature.…”

Section: Discussionmentioning

confidence: 83%

“…16 Much of the existing data regarding prevalence and severity of cardiomyopathy was before ventilatory support was common for this group of patients, 7 and the literature regarding the pharmacological management of cardiomyopathy in DMD involves predominantly children and teenagers. 10,[12][13][14] Patients are now living into their 4th and 5th decades but to date there is little information about this group. One recent observational study, involving teenagers and young adults (mean age of 22 years), requiring ventilatory support reported a lower prevalence and progression of cardiomyopathy compared to other studies 17 raising a question regarding the common assumption that it was almost inevitable.…”

Section: Introductionmentioning

confidence: 99%

“…8,9 Several studies have found that nearly all patients with DMD develop cardiomyopathy, although the age of onset varies considerably. 7,10,11 The structural evidence of cardiomyopathy precedes symptoms by years. 7 Furthermore, it is difficult to differentiate the signs and symptoms of heart failure, from respiratory complications of DMD compounded by the severe restriction in activity level.…”

Section: Introductionmentioning

confidence: 99%

“…Pharmacological treatment, including angiotensin-converting enzyme (ACE) inhibitors with or without b blockers, for asymptomatic and symptomatic cardiomyopathy in DMD reduces both morbidity and mortality. 10,[12][13][14] 'Failure to see a cardiac specialist until late in the disease, after clinical manifestations of cardiac dysfunction are evident, have led to late treatment and poor outcomes'. 15 Regular assessment is thus necessary for early detection of asymptomatic cardiomyopathy in patients with DMD.…”

Section: Introductionmentioning

confidence: 99%

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Cardiac Management of Ventilator-Assisted Individuals with Duchenne Muscular Dystrophy

et al. 2014

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As life expectancy of patients with Duchenne muscular dystrophy (DMD) has increased to the 5th decade, in part due to improved ventilatory support, cardiomyopathy is projected to increase as a cause of death. International guidelines recommend an annual assessment of cardiac function and initiation of appropriate pharmacological treatment. We conducted an audit of the cardiac management in patients with DMD requiring ventilatory support and reported a case series of the collated cardiac investigations. Patients with DMD requiring ventilatory support were included in the study. The date of the last electrocardiogram (ECG), echocardiogram (ECHO), cardiology review and pharmacological management were retrieved from the medical records. If an annual cardiac assessment had not been performed this was requested and the latest ECGs and ECHO reports were collated. A total of 30 patients with DMD (29 males, mean (SD) age of 30 (7) years) met the inclusion criteria. Although there was ECG and ECHO documentation in 24 and 21 individuals, respectively, it was only recent in 10 and 6 individuals. In all, 60% of patients had been assessed by a cardiologist, but only 10% within the last year. Over half of the patients failed to attend their new appointments. From the available results, 18 of the 19 patients had an abnormal ECG, 11 of the 16 patients had left ventricular (LV) impairment and 55% of patients had a change in prescription following cardiac investigations. There is a need for a coordinated cardiorespiratory approach towards adult patients with DMD. Over a third of patients had normal LV function suggesting that cardiomyopathy is not inevitable in this group.

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Section: Discussionmentioning

confidence: 83%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Cardiac Management of Ventilator-Assisted Individuals with Duchenne Muscular Dystrophy

et al. 2014

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“…In the advanced stages, standard therapy for heart failure with diuretics, digitalis and anticoagulants is adopted, without forgetting to correct electrolyte imbalances. 10,45,46 …”

Section: Treatment Of Dystrophinopathic Cardiomyopathymentioning

confidence: 99%

Muscular Dystrophies: Key Elements for Everyday Diagnosis and Management

Palladino¹,

Nigro

Politano³

2013

Cardiogenetics

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Muscular dystrophies are a heterogeneous group of inherited disorders that share similar clinical features and dystrophic changes on muscle biopsy, associated with progressive weakness. Weakness may be noted at birth or develop in late adult life. In recent years, cardiac involvement has been observed in a growing number of genetic muscle diseases, and considerable progress has been made in understanding the relationships between disease skeletal muscle and cardiac muscle disease. This review will focus on the skeletal muscle diseases most commonly associated with cardiac complications that can be diagnosed by echocardiography, such as dystrophinopathies including Duchenne (DMD) and Becker (BMD) muscular dystrophies, cardiomyopathy of DMD/BMD carriers and X-L dilated cardiomyopathy.

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Interventions for preventing and treating cardiac complications in Duchenne and Becker muscular dystrophy and X-linked dilated cardiomyopathy

Bourke

Bueser

Quinlivan

2018

Cochrane Database of Systematic Reviews

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Effects of Angiotensin-Converting Enzyme Inhibitors and/or Beta Blockers on the Cardiomyopathy in Duchenne Muscular Dystrophy

Cited by 136 publications

References 14 publications

Cardiac Management of Ventilator-Assisted Individuals with Duchenne Muscular Dystrophy

Cardiac Management of Ventilator-Assisted Individuals with Duchenne Muscular Dystrophy

Muscular Dystrophies: Key Elements for Everyday Diagnosis and Management

Interventions for preventing and treating cardiac complications in Duchenne and Becker muscular dystrophy and X-linked dilated cardiomyopathy

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