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Cardiomyopathy in Children: Classification and Diagnosis: A Scientific Statement From the American Heart Association

Steven E Lipshultz, Yuk M Law, Alfred Asante-Korang

    Circulation
    |May 29, 2019
    PubMed

    Insights

    Pediatric cardiomyopathy, a serious heart muscle disease in children, requires better diagnostic approaches. Early diagnosis and research are crucial for improving outcomes and quality of life for affected children.

    Area of Science:

    • Pediatric Cardiology
    • Cardiomyopathy Research
    • Genetics of Heart Disease

    Background:

    • Cardiomyopathy (heart muscle disease) in children leads to poor outcomes, with nearly 40% dying or needing heart transplantation within two years of diagnosis.
    • Pediatric cardiomyopathy is the leading cause of heart transplantation for children over one year of age, with no decline in transplant rates over the past decade.
    • Despite a significant impact, research and focus on pediatric cardiomyopathy lag behind that of comparable childhood cancers.

    Purpose of the Study:

    • To provide the most current understanding of the causes of cardiomyopathy in children.
    • To outline optimal diagnostic approaches for pediatric cardiomyopathy.
    • To guide future research priorities for earlier diagnosis and improved outcomes in pediatric cardiomyopathy.

    Main Methods:

    • Review of current scientific understanding and expert consensus on pediatric cardiomyopathy.
    • Analysis of data from the National Heart, Lung, and Blood Institute-funded Pediatric Cardiomyopathy Registry.
    • Focus on diagnostic criteria and classification of pediatric cardiomyopathy.

    Main Results:

    • Genetic causes are suspected in most pediatric cardiomyopathy cases, though established in few.
    • The incidence of pediatric cardiomyopathy is approximately 1 in 100,000 children.
    • Current diagnostic understanding and research efforts are insufficient given the severity of the condition.

    Conclusions:

    • There is a critical need for enhanced focus on the diagnosis and classification of pediatric cardiomyopathy.
    • Earlier diagnosis and targeted research are essential to improve clinical outcomes and quality of life.
    • This statement aims to stimulate future research to address the challenges posed by pediatric cardiomyopathy.

    Keywords:
    AHA Scientific Statementscardiomyopathieschildgeneticsheart failureheart transplantationsudden death

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