Guideline:
Cardiovascular health supervision for individuals affected by Duchenne or Becker muscular dystrophy
Bibliographic Source(s)
- American Academy of Pediatrics Section on Cardiology and Cardiac Surgery. Cardiovascular health supervision for individuals affected by Duchenne or Becker muscular dystrophy. Pediatrics 2005 Dec;116(6):1569-73. [60 references] PubMed
Guideline Status
This is the current release of the guideline.
All policy statements from the American Academy of Pediatrics automatically expire 5 years after publication unless reaffirmed revised or retired at or before that time.
Guideline Category
Evaluation
Management
Risk Assessment
Screening
Treatment
Intended Users
Physicians
Guideline Objective(s)
To provide recommendations for optimal cardiovascular evaluation to health care specialists caring for individuals with Duchenne or Becker muscular dystrophy
Target Population
Individuals with Duchenne or Becker muscular dystrophy
Interventions and Practices Considered
- Complete cardiac evaluation including:
- History and physical examination
- Electrocardiogram
- Transthoracic echocardiogram
- Multigated acquisition study (MUGA) or cardiac magnetic resonance imaging (MRI) for patients with limited echocardiographic acoustic windows
- Pharmacologic treatment of cardiac dysfunction (e.g. diuretics angiotensin-converting enzyme inhibitors beta-blockers); rhythm abnormalities; and respiratory abnormalities
- Increased cardiovascular surveillance for patients undergoing treatment with glucocorticoids
- Pre-operative evaluation of baseline cardiac and pulmonary function
- Intraoperative cardiac monitoring
- Postoperative maintenance of fluid balance and cardiopulmonary monitoring
- Optimization of nutrition status
- Anticoagulation therapy
- Optimizing pulmonary care
- Cardiovascular screening of female carriers of Duchenne or Becker muscular dystrophy
Major Outcomes Considered
Morbidity and mortality associated with cardiovascular complications of Duchenne and Becker muscular dystrophy
Methods Used to Collect/Select Evidence
Searches of Electronic Databases
Description of Methods used to Collect/Select the Evidence
Not stated
Number of Source Documents
Not stated
Methods Used to Assess the Quality and Strength of the Evidence
Not stated
Rating Scheme for the Strength of the Evidence
Not applicable
Methods Used to Analyze the Evidence
Review
Description of the Methods Used to Analyze the Evidence
Not stated
Methods Used to Formulate the Recommendations
Expert Consensus
Description of Methods Used to Formulate the Recommendations
Not stated
Rating Scheme for the Strength of the Recommendations
Not applicable
Cost Analysis
A formal cost analysis was not performed and published cost analyses were not reviewed.
Method of Guideline Validation
Peer Review
Description of Method of Guideline Validation
Not stated
Major Recommendations
Recommendations for Cardiac Care in Patients With Duchenne Muscular Dystrophy (DMD) or Becker Muscular Dystrophy (BMD)
- 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 transthoracic echocardiogram. Consideration should be given to a multigated acquisition study (MUGA) or cardiac magnetic resonance imaging (MRI) in patients with limited echocardiographic acoustic windows.
- Clinicians should be aware that the typical signs and symptoms of cardiac dysfunction may not be present secondary to the patient's musculoskeletal limitations. Weight loss cough nausea and vomiting orthopnea and increased fatigue with a decreased ability to tolerate the daily regimen may represent cardiac impairment and should be investigated. However 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 angiotensin-converting enzyme inhibitors and/or beta-blockers.
- Abnormalities of cardiac rhythm should be promptly investigated and treated. Periodic Holter monitoring should be considered for patients with demonstrated cardiac dysfunction.
- Respiratory abnormalities contribute to the cardiovascular morbidity and mortality of the disease. Concurrent evaluation and treatment of respiratory abnormalities are recommended.
- Individuals undergoing treatment with glucocorticoids warrant increased cardiac surveillance with specific monitoring for weight gain and hypertension.
- Complete cardiac evaluation should be undertaken before scoliosis surgery or other major surgical procedures. Consideration should be given to cardiac stress testing (such as a dobutamine stress echocardiogram) if abnormalities of cardiac function are present during resting evaluation. Medical therapy should be optimized before surgery and the risks and benefits of the procedure should be discussed in detail with the patient and the family.
- Intraoperative cardiac monitoring should be undertaken in individuals with DMD or BMD during major surgical procedures. Specific anesthetic techniques and decisions about intraoperative ventilation will depend on the patient and the procedure. Agents known to trigger hyperkalemia (e.g. succinylcholine chloride) or a hypermetabolic state (e.g. inhaled anesthetic agents) should be avoided. Cardiac monitoring should continue in the postoperative period.
- Anticoagulation therapy should be considered in patients with severe cardiac dysfunction to prevent systemic thromboembolic events.
- Clinicians who are experienced in the care of patients with DMD or BMD and are knowledgeable about the pathogenesis of the disease should be actively involved when patients are treated in an intensive care setting.
- Nutritional status should be optimized to the special needs of patients with DMD or BMD.
Recommendations Specific for Cardiac Care in Patients with DMD
- Patients should be routinely managed in early childhood with a complete cardiac evaluation at least biannually.
- Yearly complete cardiac evaluations should begin at approximately 10 years of age or at the onset of cardiac signs and symptoms. However individuals demonstrating these signs and symptoms are relatively late in their course.
Recommendations Specific for Cardiac Care in Patients with BMD
- Complete cardiac evaluations should begin at approximately 10 years of age or at the onset of signs and symptoms. Evaluations should continue at least biannually.
Recommendations for Cardiac Care in Carriers of DMD or BMD
- 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 for evaluation by 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.
Clinical Algorithm(s)
None provided
Type of Evidence supporting the Recommendations
The type of evidence supporting the recommendations is not specifically stated.
Potential Benefits
- Appropriate cardiovascular monitoring and evaluation of patients with Duchenne or Becker muscular dystrophy may lead to decreased morbidity and mortality in these patients.
- Investigation of cardiomyopathy in patients with Duchenne muscular dystrophy and Becker muscular dystrophy as well as carriers will benefit affected individuals and new knowledge may lead to the elucidation of novel treatment strategies for dilated cardiomyopathy.
Potential Harms
Not stated
Description of Implementation Strategy
An implementation strategy was not provided.
IOM Care Need
Living with Illness
IOM Domain
Effectiveness
Bibliographic Source(s)
- American Academy of Pediatrics Section on Cardiology and Cardiac Surgery. Cardiovascular health supervision for individuals affected by Duchenne or Becker muscular dystrophy. Pediatrics 2005 Dec;116(6):1569-73. [60 references] PubMed
Adaptation
Not applicable: The guideline was not adapted from another source.
Source(s) of Funding
American Academy of Pediatrics
Guideline Committee
Section on Cardiology and Cardiac Surgery
Composition of Group that Authored the Guideline
Section on Cardiology and Cardiac Surgery 2004-2005: Thomas S. Klitzner MD PhD Chairperson; Robert H. Beekman III MD; Frank M. Galioto Jr MD; Thomas K. Jones MD; Peter B. Manning MD; W. Robert Morrow MD; Jane Newburger MD MPH; John W. M. Moore MD MPH Immediate Past Chairperson
Invited Contributor: *Linda H. Cripe MD
Staff: Lynn Colegrove MBA
*Lead author
Financial Disclosures/Conflicts of Interest
Not stated
Guideline Status
This is the current release of the guideline.
All policy statements from the American Academy of Pediatrics automatically expire 5 years after publication unless reaffirmed revised or retired at or before that time.
Guideline Availability
Electronic copies: Available from the American Academy of Pediatrics (AAP) Policy Web site.
Print copies: Available from American Academy of Pediatrics 141 Northwest Point Blvd. P.O. Box 927 Elk Grove Village IL 60009-0927.
Availability of Companion Documents
None available
Patient Resources
None available
NGC STATUS
This NGC summary was completed by ECRI on January 9 2006. The information was verified by the guideline developer on January 12 2006.
COPYRIGHT STATEMENT
This NGC summary is based on the original guideline which is subject to the guideline developer's copyright restrictions. Please contact the Permissions Editor American Academy of Pediatrics (AAP) 141 Northwest Point Blvd Elk Grove Village IL 60007.
NGC Disclaimer
The National Guideline Clearinghouse™ (NGC) does not develop produce approve or endorse the guidelines represented on this site.
All guidelines summarized by NGC and hosted on our site are produced under the auspices of medical specialty societies relevant professional associations public or private organizations other government agencies health care organizations or plans and similar entities.
Guidelines represented on the NGC Web site are submitted by guideline developers and are screened solely to determine that they meet the NGC Inclusion Criteria which may be found at http://www.guideline.gov/about/inclusion.aspx .
NGC AHRQ and its contractor ECRI Institute make no warranties concerning the content or clinical efficacy or effectiveness of the clinical practice guidelines and related materials represented on this site. Moreover the views and opinions of developers or authors of guidelines represented on this site do not necessarily state or reflect those of NGC AHRQ or its contractor ECRI Institute and inclusion or hosting of guidelines in NGC may not be used for advertising or commercial endorsement purposes.
Readers with questions regarding guideline content are directed to contact the guideline developer.
Tools
No Quick Reference tools have been developed.