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The Pediatric Cardiomyopathy Registry: 1995-2007
James D Wilkinson1, Lynn A Sleeper, Jorge A Alvarez
1Division of Pediatric Clinical Research, Department of Pediatrics, Miller School of Medicine, University of Miami, Miami, FL.
Progress in Pediatric Cardiology
|April 4, 2009
Summary
The Pediatric Cardiomyopathy Registry (PCMR) tracks over 3,000 children with heart muscle disorders, revealing an incidence of 1.13 per 100,000 children. Etiology is often unknown at diagnosis, impacting prognosis for conditions like dilated and hypertrophic cardiomyopathy.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Epidemiology
Background:
- Cardiomyopathy (CM) is a severe heart muscle disease in children, with rare but devastating potential.
- The Pediatric Cardiomyopathy Registry (PCMR) has collected data since 1994 to understand pediatric CM epidemiology and clinical course.
- Over 3,000 children's data are in the PCMR database, with ongoing annual follow-up.
Purpose of the Study:
- To describe the epidemiology and clinical course of selected cardiomyopathies in children (≤18 years).
- To promote the development of etiology-specific prevention and treatment strategies for pediatric cardiomyopathy.
- To analyze risk factors and outcomes associated with different types of pediatric cardiomyopathy.
Main Methods:
- Utilized data from the multi-center Pediatric Cardiomyopathy Registry (PCMR).
- Included annual follow-up data until death, heart transplant, or loss-to-follow-up for over 3,000 children.
- Analyzed patient characteristics, family history, echocardiographic findings, laboratory tests, and biopsy results.
Main Results:
- Estimated incidence of cardiomyopathy in US children is 1.13 per 100,000.
- Etiology was unknown in approximately two-thirds of diagnosed children.
- Higher incidence observed in boys and infants (<1 yr) for dilated (DCM) and hypertrophic (HCM) cardiomyopathy, and in Black children for DCM.
- Worse prognosis in DCM linked to older age at diagnosis (>1 yr), heart failure at diagnosis, or idiopathic etiology.
- Worse prognosis in HCM associated with inborn errors of metabolism or combined cardiomyopathy types.
- Best outcomes in HCM seen in children >1 yr with idiopathic HCM.
Conclusions:
- The PCMR is a crucial resource for understanding pediatric cardiomyopathy etiology, clinical course, and outcomes.
- Further research through collaborations and specimen repositories will enhance understanding and treatment strategies.
- Identifying specific risk factors and prognostic indicators is vital for improving outcomes in children with cardiomyopathy.
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