- 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.
- 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.
- Scientific statement.
- Limited guidance because of a dearth of evidence.