Pediatrics Academy Publishes Heart
Care Guidelines for Duchenne, Becker MDs

The Section on Cardiology and Cardiac Surgery of the American Academy of Pediatrics published its recommendations for cardiac care in DMD and BMD and carriers of those dystrophies in the December issue of its journal, Pediatrics.

In summary, here are the recommendations.

• Cardiac care of the patient with DMD or BMD should begin after confirmation of the diagnosis. The patient should be referred for evaluation to a cardiac specialist with an interest in the management of cardiac dysfunction and/or neuromuscular disorders.
• A complete cardiac evaluation should include (but not be limited to) a history and physical examination, electrocardiogram and echocardiogram. Consideration should be given to other imaging studies, such as magnetic resonance imaging (MRI).
• Clinicians should be aware that the typical signs and symptoms of cardiac dysfunction may not be present because of the patient’s mobility limitations. The development of dilated cardiomyopathy usually precedes the development of heart failure symptoms by years and must be identified at its earliest onset.
• Signs and symptoms of cardiac dysfunction should be treated. Consideration should be given to the use of diuretics (medications to increase fluid excretion), angiotensin converting enzyme inhibitors and/or beta blockers.
• Abnormalities of cardiac rhythm should be promptly investigated and treated. Periodic Holter monitoring (24- to 48-hour mobile EKG) should be considered.
• Respiratory abnormalities contribute to cardiovascular dysfunction in DMD and BMD. Evaluation and treatment of respiratory abnormalities is recommended.
• Patients undergoing treatment with corticosteroids (such as prednisone) warrant increased cardiac surveillance with specific monitoring for weight gain and high blood pressure.
• Complete cardiac evaluation should be undertaken before scoliosis surgery or other major surgical procedures. Risks and benefits of the procedure should be discussed in detail with the patient and the family.
• Cardiac monitoring should be performed in DMD or BMD patients during major surgical procedures and should continue in the postoperative period. Specific anesthetic techniques and decisions about ventilation during the operation should depend on the patient and the procedure.
• Anticoagulation therapy (to prevent blood clots) should be considered in patients with severe cardiac dysfunction.
• Clinicians who are experienced in the care of patients with DMD or BMD and are knowledgeable about these diseases should be actively involved when patients are treated in an intensive care setting.
• Nutritional needs should be met.

Specific Recommendations for DMD Patients

• Patients should be routinely managed in early childhood with a complete cardiac evaluation at least every other year.
• Yearly complete cardiac evaluations should begin at approximately 10 years of age or at the onset of cardiac signs and symptoms.
  

Specific Recommendations for BMD Patients

• Complete cardiac evaluations should begin at approximately 10 years of age or at the onset of signs and symptoms. Evaluations should continue at least every other year.
  

Recommendations for DMD and BMD Carriers

• Carriers of DMD or BMD should be made aware of the risk of developing cardiomyopathy and educated about the signs and symptoms of heart failure.
• Carriers of DMD or BMD should be referred to a cardiac specialist with experience in the treatment of heart failure and/or neuromuscular disorders. Patients should undergo initial complete cardiac evaluation in late adolescence or early adulthood or at the onset of cardiac signs and symptoms, if these signs or symptoms appear earlier.
• Carriers should be screened with a complete cardiac evaluation at a minimum of every 5 years starting at 25 to 30 years of age.
• Treatment of cardiac disease is similar to that outlined for boys with DMD or BMD.
  

Adapted from “Cardiovascular Health Supervision for Individuals Affected by Duchenne or Becker Muscular Dystrophy,” Pediatrics, December 2005.