Outcomes of Restrictive Cardiomyopathy in Childhood and the Influence of Phenotype

• DOI: 10.1161/circulationaha.112.104638
• Publication Date: 2012-07-28T11:50:47Z
• Authors: Steven A. Webber; Steven E. Lipshultz; Lynn A. Sleeper; Minmin Lu; James D. Wilkinson; Linda J. Addonizio; Charles E. Canter; Steven D. Colan; Melanie D. Everitt; John Lynn Jefferies; Paul F. Kantor; Jacqueline M. Lamour; Renee Margossian; Elfriede Pahl; Paolo G. Rusconi; Jeffrey A. Towbin
• Keywords: Restrictive cardiomyopathy

Restrictive cardiomyopathy (RCM) has been associated with poor prognosis in childhood. The goal of the present analysis was to use Pediatric Cardiomyopathy Registry analyze outcomes childhood RCM, a focus on impact phenotype comparing pure RCM cases that have additional features hypertrophic (HCM).We analyzed database (1990-2008; N=3375) for RCM. Cases were defined as when only assigned diagnosis. Additional documentation HCM at any time used criterion RCM/HCM phenotype. accounted 4.5% cardiomyopathy. In 101 (66%), diagnosed; 51 (34%), there mixed Age diagnosis not different between groups, but 10% group diagnosed infancy versus 24% group. Freedom from death comparable groups 1-, 2-, and 5-year survival 82%, 80%, 68% 77%, 74%, 68%. Transplant-free 48%, 34%, 22% 65%, 53%, 43%, respectively (P=0.011). Independent risk factors lower transplant-free heart failure (hazard ratio 2.20, P=0.005), fractional shortening z score 1.12 per 1 SD decrease score, P=0.014), higher posterior wall thickness 1.32, P<0.001). Overall, worse than all other forms cardiomyopathy.Transplant-free is Survival independent phenotype; however, significantly better survival.URL: http://www.clinicaltrials.gov. Unique Identifier: NCT00005391.