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Fixler D.E.,University of Texas at Dallas | Nembhard W.N.,University of South Florida | Salemi J.L.,University of South Florida | Ethen M.K.,Texas Birth Defects Epidemiology and Surveillance Branch | Canfield M.A.,Texas Birth Defects Epidemiology and Surveillance Branch
Circulation | Year: 2010

BACKGROUND-: Infants with functional single ventricle have a high risk of death during the early years of life. Studies have reported improvement in postoperative survival, but they do not include preoperative deaths or those occurring before transfer. The purpose of this population-based study was to estimate 5-year survival in infants with functional single ventricle, to define factors associated with survival, and to estimate improvement in outcome. METHODS AND RESULTS-: Patients with hypoplastic left heart syndrome, pulmonary atresia intact ventricular septum, single ventricle, and tricuspid atresia born in 1996 to 2003 were identified from the Texas Birth Defects Registry and linked to state and national birth and death vital records. We examined the effects of defect type, birth era, birth weight, gestational age, maternal race/ethnicity, extracardiac anomalies, sex, and maternal age and education on survival. Five-year survival varied by defect type: hypoplastic left heart syndrome, 38.0% (95% confidence interval, 32.6 to 43.5); single ventricle, 56.1% (95% confidence interval, 49.9 to 61.7); pulmonary atresia intact ventricular septum, 55.7% (95% confidence interval, 45.8 to 64.4); and tricuspid atresia, 74.6% (95% confidence interval, 62.4 to 83.4). The presence of extracardiac defects increased the adjusted risk of death by 84%. Non-Hispanic blacks had an adjusted risk of death that was 41% higher than that for non-Hispanic whites, and Hispanics had a 26% higher risk. Patients born in 2001 to 2003 had a 47% lower risk than those born in 1996 to 2000. CONCLUSIONS-: This population-based study demonstrates significant improvement in overall 5-year survival, particularly in cases of hypoplastic left heart syndrome and single ventricle. Additional studies are needed to determine the factors causing racial/ethnic and regional differences in outcome. © 2010 American Heart Association, Inc.


Shin M.,Centers for Disease Control and Prevention | Shin M.,Oak Ridge Institute for Science and Education | Besser L.M.,Centers for Disease Control and Prevention | Siffel C.,Centers for Disease Control and Prevention | And 12 more authors.
Pediatrics | Year: 2010

OBJECTIVE: The goal was to estimate the number of children and adolescents, 0 to 19 years of age, living with spina bifida (SB) in the United States. METHODS: A retrospective study was conducted by using population-based, birth defect surveillance data from 10 US regions, with vital status ascertainment. Birth defect surveillance data were obtained from Arkansas, Georgia (5 central counties of metropolitan Atlanta), California (11 counties), Colorado, Iowa, New York (New York City excluded), North Carolina, Oklahoma, Texas, and Utah. We estimated the numbers of children 0 to 19 years of age who were living with SB in the 10 US regions in 2002, according to age group, race/ethnicity, and gender, and examined a long-term trend in the prevalence of SB among children 0 to 11 years of age in 1991-2002. RESULTS: The overall prevalence of SB among children and adolescents 0 to 19 years of age in the study regions was 3.1 cases per 10 000 in 2002. The prevalence of SB among children was lower among male and non-Hispanic black children. CONCLUSIONS: The prevalence estimates of SB among children and adolescents varied according to region, race/ethnicity, and gender, which suggests possible variations in prevalence at birth and/or inequities in survival rates. Additional studies are warranted to elucidate the reasons for these variations and to derive prevalence estimates of SB among adults. Copyright © 2010 by the American Academy of Pediatrics.

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