What are the CDC guidelines for cardiac management of Duchenne muscular dystrophy (DMD)?

Updated: Aug 17, 2020
  • Author: Twee T Do, MD; Chief Editor: Jeffrey D Thomson, MD  more...
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Answer

Cardiac management

The CDC guidelines recommend that a cardiac specialist be involved with the patient and family after confirmation of the diagnosis to initiate a relationship to ensure long-term cardiovascular health. Baseline assessment of cardiac function should be done at diagnosis or by the age of 6 years, especially if this can be done without sedation. Clinical judgment should be used for patients under the age of 6 years who require sedation.

Echocardiographic screening at the time of diagnosis or by the age of 6 years is deemed necessary even though the incidence of echocardiographic abnormalities is low in children younger than 8 to 10 years. A baseline echocardiogram allows for screening for anatomic abnormalities (eg, atrial or ventricular septal defects, patent ductus arteriosus) that might affect long-term cardiovascular function. [73]

Additional recommendations for cardiac management include the following:

  • Minimum assessment should include electrocardiography (ECG) and a noninvasive cardiac imaging study (ie, echocardiography)
  • Assessment of cardiac function should be performed at least once every 2 years until the age of 10 years
  • Annual complete cardiac assessments should begin at the age of 10 years, or earlier if cardiac signs and symptoms arise
  • Abnormalities of ventricular function on non-invasive cardiac imaging studies warrant increased surveillance (at least every 6 months) and should prompt initiation of pharmacologic therapy, irrespective of the age at which they are detected
  • Consider the use of angiotensin-converting enzyme (ACE) inhibitors as first-line therapy; beta blockers and diuretics are also appropriate
  • Signs or symptoms of cardiac rhythm abnormalities should be investigated with Holter or event monitoring and should be treated
  • New-onset sinus tachycardia in the absence of a clear cause should prompt assessment, including that of left ventricular function
  • Patients receiving glucocorticoids need additional monitoring for hypertension, which might necessitate adjustment in the glucocorticoid dose; systemic arterial hypertension should be treated
  • Prevention of systemic thromboembolic events by anticoagulation therapy can be considered in patients with severe cardiac dysfunction, but is inappropriate in earlier cardiac dysfunction

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