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Duchenne Muscular Dystrophy: Cardiac Management

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Duchenne Muscular Dystrophy: Cardiac Management Introduction Aim: early detection and treatment of deterioration in heart muscle function Cardiac disease most often ... – PowerPoint PPT presentation

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Title: Duchenne Muscular Dystrophy: Cardiac Management


1
Duchenne Muscular DystrophyCardiac Management
2
Introduction
  • Aim early detection and treatment of
    deterioration in heart muscle function
  • Cardiac disease most often manifests as
  • Cardiomyopathy and/or
  • Cardiac arrhythmia
  • Progressive cardiomyopathy a major source of
    DMD/BMD morbidity warrants further study
  • Clear onset of disease in myocardium before
    clinical symptoms
  • Surveillance and proactive management are key a
    cardiologist must be in the care team
  • Family should have a copy of the latest
    assessments to show attending doctors

3
Surveillance Baselines
  • Baseline evaluation of cardiac function at
    diagnosis or by 6 years at the latest, especially
    if possible without sedation
  • Clinical judgement should be used for patients
    under 6 requiring sedation
  • Incidence of echocardiograph abnormalities is low
    in children under 8-10 years, but where they do
    exist can affect clinical decision-making
  • Baseline echocardiogram also allows for screening
    for anatomical abnormalities(e.g.
    atrial/ventricular septal defects, patent ductus
    arteriosis) which may affect long-term
    cardiovascular function
  • Minimal evaluation should include, but is not
    limited to, and electrocardiogram (ECG) and
    echocardiogram

4
Surveillance Annual Evaluation
  • Evaluation at least once every 2 years to the age
    of 10.
  • Annual complete cardiac assessments should begin
    at 10, or at onset of cardiac signs or symptoms
    if these occur earlier
  • Increased surveillance (at least every 6 months)
    required if non-invasive cardiac tests show
    abnormalities of ventricular function.
  • Drug treatment should be initiated, irrespective
    of the age at which they are detected

5
Treatment
  • First-line therapy angiotension converting
    enzyme (ACE) inhibitors
  • Beta-blockers and diuretics also appropriate, and
    should follow published guidelines for management
    of heart failure
  • Recent clinical trial evidence supports the
    prophylactic treatment of cardiomyopathy with ACE
    inhibitors prior to signs of abnormal
    functioning further studies awaited to allow
    firm recommendations.

6
Abnormalities (1)
  • Abnormalities of cardiac rhythm should be
    promptly investigated with Holder or event
    monitor recording, and should be treated
  • Sinus tachycardia is common in DMD, but also
    noted in systolic dysfunction.
  • New onset in absence of clear cause should prompt
    assessment including that of left-ventricular
    function
  • Those on steroids need additional cardiovascular
    attention, especially for hypertension which may
    necessitate an adjustment of steroid dose TLN
    table 2 part 1

7
Abnormalities (2)
  • Systemic arterial hypertension should be treated
  • Prevention of systemic thromboembolic events by
    anticoagulation therapy can be considered in
    severe cardiac dysfunction, but inappropriate in
    earlier cardiac dysfunction
  • Usefulness of internal cardiac defibrillator not
    established
  • With generally improved fitness of DMD patients,
    cardiac transplants may require consideration in
    future

8
References Resources
  • The Diagnosis and Management of Duchenne Muscular
    Dystrophy, Bushby K et al, Lancet Neurology 2010
    9 (1) 77-93 Lancet Neurology 2010 9 (2) 177-189
  • Particularly references, p186-188
  • The Diagnosis and Management of Duchenne Muscular
    Dystrophy A Guide for Families
  • TREAT-NMD website www.treat-nmd.eu
  • CARE-NMD website www.care-nmd.eu
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