Pediatric cardiomyopathy: AHA advice on diagnosis, treatment

  • Lipshultz SE & al.
  • Circulation
  • 28 May 2019

  • International Clinical Digest
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Takeaway

  • The American Heart Association (AHA) has released a scientific statement on pediatric cardiomyopathy that focuses on current understanding of its causes and optimal paths to diagnosis.

Why this matters

  • These conditions can carry dire outcomes, with 40% of children having a heart transplantation or dying within the first 2 years of being diagnosed.
  • Transplantation rates have not declined in the last decade.
  • Cause is often not established, despite a strong likelihood of a genetic component. 
  • Fewer studies cover these conditions than address lymphoma, Wilms, or neuroblastoma, despite similar incidences. 
  • Because of sparse evidence, the AHA decided on a scientific statement with recommendations instead of a guideline.

Key highlights

  • For classification, follow a morphofunctional top-down approach, working from structural and functional manifestations to genetic and nongenetic causes.
  • Causation is highly variable and poorly distinguished.
  • The statement offers a thorough discussion even of the rarest conditions for clinicians to use in diagnosis.
  • No diagnostic algorithm is provided. 
  • Attempting to find cause is important, especially in infants, to include thorough history and physical exam, detailed imaging, and accompanying abnormalities. 

Study design

  • Scientific statement.

Limitations

  • Limited guidance because of a dearth of evidence.