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Review
. 2010 Oct;6(4):401-13, vii.
doi: 10.1016/j.hfc.2010.05.002.

The pediatric cardiomyopathy registry and heart failure: key results from the first 15 years

James D Wilkinson  1 , David C Landy, Steven D Colan, Jeffrey A Towbin, Lynn A Sleeper, E John Orav, Gerald F Cox, Charles E Canter, Daphne T Hsu, Steven A Webber, Steven E Lipshultz
Affiliations
Review

The pediatric cardiomyopathy registry and heart failure: key results from the first 15 years

James D Wilkinson et al. Heart Fail Clin. 2010 Oct.

Abstract

Cardiomyopathy is a serious disorder of the heart muscle and, although rare, is a common cause of heart failure in children and the most common cause for heart transplantation in children older than 1 year of age. Funded by the National Heart Lung and Blood Institute since 1994, the Pediatric Cardiomyopathy Registry (PCMR) has followed more than 3500 North American children with cardiomyopathy. Early analyses determined estimates for the incidence of pediatric cardiomyopathy (1.13 cases per 100,000 children per year), risk factors for cardiomyopathy (age <1 year, male sex, black race, and living in New England as opposed to the central southwestern states), the prevalence of heart failure at diagnosis (6%-84% depending on cause), and 10-year survival (29%-94% depending on cause). More recent analyses explored cause-specific functional status, survival and transplant outcomes, and risk factors in greater detail. For many topics these analyses are based on the largest and best-documented samples of children with disease such as the muscular dystrophies, mitochondrial disorders, and Noonan syndrome. Data from the PCMR continue to provide valuable information that guides clinical management and the use of life-saving therapies, such as cardiac transplantation and approaches to treating heart failure, and prepares children, their families, and their caregivers to deal with this serious condition.

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Figures

Figure 1
Figure 1
Freedom from death or transplant for 491 children in the retrospective cohort and 935 children in the prospective cohort with pure dilated cardiomyopathy (P= 0.71). Data are from the Pediatric Cardiomyopathy Registry for the period between 1990 and 2002. [From Towbin JA, Lowe AM, Colan SD, et al. Incidence, causes, and outcomes of dilated cardiomyopathy in children. JAMA 2006;296:1869, with permission.]
Figure 2
Figure 2
Freedom from death or transplantation for 1423 children with pure dilated cardiomyopathy, by cause. Data are from the Pediatric Cardiomyopathy Registry for the period between 1990 and 2002. [From Towbin JA, Lowe AM, Colan SD, et al. Incidence, causes, and outcomes of dilated cardiomyopathy in children. JAMA 2006;296:1873, with permission.]
Figure 3
Figure 3
Freedom from death or transplantation for 855 children with idiopathic hypertrophic cardiomyopathy, by age at diagnosis. Data are from the Pediatric Cardiomyopathy Registry for the period between 1990 and 2002. [From Colan SD, Lipshultz SE, Lowe AM, et al. Epidemiology and cause specific outcome of hypertrophic cardiomyopathy in children: findings from the Pediatric Cardiomyopathy Registry. Circulation 2007;115:777, with permission.]
Figure 4
Figure 4
Risk of death or transplant in 882 children with hypertrophic cardiomyopathy, by number of cause-specific risk factors. Data are from the Pediatric Cardiomyopathy Registry for the period between 1990 and 2002. [From Lipshultz SE, Orav EJ, Wilkinson JD, Towbin JA, Messere J, Lowe AM, Sleeper LA, Clunie SK, Cox GF, Lurie PR, Hsu DT, Canter CE, Colan SD. A risk stratification analysis of predictors of death or transplant in children with hypertrophic cardiomyopathy. Circulation Supplement. 2008;118:4956.]
Figure 5
Figure 5
Physical and psychosocial function, as measured by the Child Health Questionnaire, of 294 children with cardiomyopathy, by functional type. Data are from the Pediatric Cardiomyopathy Registry for the period between 1990 and 2002. [From Sleeper LA, Towbin JA, Colan SD, et al. Functional Status is Impaired and Correlated with Clinical Status in Pediatric Cardiomyopathy [abstract]. In: Proc 5th World Cong Pediatr Cardiol and Cardiac Surg 2009;5:134.]
Figure 6
Figure 6
Child Health Questionnaire domain mean z scores for 303 children with cardiomyopathy in the Pediatric Cardiomyopathy Registry. Scores for the physical domains, general health, self-esteem, and parental impact-emotional domains were significantly below the average for healthy children. Scores for mental health and behavior did not differ significantly from those of healthy children. (PF = physical functioning; RE = role/social limits-emotional; RP = role/social limits-physical; PAIN= bodily pain; BEH = behavior; MH = mental health; SE = self-esteem; GH = general health perception; PE = parental impact-emotional; PT= parental impact-time). Data are from the Pediatric Cardiomyopathy Registry for the period between 1990 and 2002. [From Sleeper LA, Towbin JA, Colan SD, et al. Functional Status is Impaired and Correlated with Clinical Status in Pediatric Cardiomyopathy [abstract]. In: Proc 5th World Cong Pediatr Cardiol and Cardiac Surg 2009;5:134.]
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