Abstract 145 Poster Session III, Monday, 5/3 (poster 128)

Background: Large prospective studies of pediatric cardiomyopathy (CM) have not previously been conducted, resulting in little population-based data.

Methods: The NHLBI-sponsored Pediatric CM Registry (PCMR) collected data on all patients ≤ 18 years of age newly diagnosed by a pediatric cardiologist with CM meeting study criteria during 1996 in 2 geographically distinct regions, New England (NE: MA< ME, NH, VT, RI, CT) and the Central Southwest (CSW: TX, OK, AR).

Results: We identified 130 cases in both regions for an overall incidence of 1.31/100,000 children. There was a significantly higher incidence in NE compared to the CSW, 1.79 vs. 1.13 respectively (P=0.022). Younger age (0-1 yr.) at diagnosis had a significantly higher incidence rate than older age (1-8 yrs.) at diagnosis (13.0 vs. 0.732, P value <0.001). The incidence of CM was lower in whites (0.62) than in African-American (1.60, P value <0.001) or Hispanic children (1.42, P value <0.001). Boys had a higher incidence of CM than girls (1.52 vs. 1.10, P value = 0.070). The incidence rates for different functional types of CM were hypertrophic = 47%, dilated = 46%, restrictive = 3%, and arrhythmia = 5%. The incidence rate for pts with mixed CM was 6%.

Conclusion: The incidence of pediatric CM has been established. Most cases are identified at an early age and regional, gender, and ethnic origin differences are suggested. Further studies are essential.