TY - JOUR
T1 - The Pediatric Cardiomyopathy Registry and Heart Failure
T2 - Key Results from the First 15 Years
AU - Wilkinson, James D.
AU - Landy, David C.
AU - Colan, Steven D.
AU - Towbin, Jeffrey A.
AU - Sleeper, Lynn A.
AU - Orav, E. John
AU - Cox, Gerald F.
AU - Canter, Charles E.
AU - Hsu, Daphne T.
AU - Webber, Steven A.
AU - Lipshultz, Steven E.
PY - 2010/10/1
Y1 - 2010/10/1
N2 - 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.
AB - 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.
KW - Cardiomyopathy
KW - Heart failure
KW - Pediatric cardiomyopathy registry
KW - Pediatrics
UR - http://www.scopus.com/inward/record.url?scp=77956893147&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77956893147&partnerID=8YFLogxK
U2 - 10.1016/j.hfc.2010.05.002
DO - 10.1016/j.hfc.2010.05.002
M3 - Review article
C2 - 20869642
AN - SCOPUS:77956893147
VL - 6
SP - 401
EP - 413
JO - Heart Failure Clinics
JF - Heart Failure Clinics
SN - 1551-7136
IS - 4
ER -