The Patterns and Public Health Impact of Heart Defects in Texas Pediatric Cardiac Care Conference VI Dell Children s Medical Center, Feb.

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1 The Patterns and Public Health Impact of Heart Defects in Texas Pediatric Cardiac Care Conference VI Dell Children s Medical Center, Feb. 7-8, 2013 Mark Canfield, Ph.D. Manager, Birth Defects Epidemiology and Surveillance Branch Texas Department of State Health Services

2 Topics Texas Birth Defects Registry Prevalence of Heart Defects, by Race/Ethnicity Maternal Age Infant Sex Public Health Region Body Mass Index and Diabetes Status Prevalence of Critical Congenital Heart Defects TX, US Mortality and Survival Hospital Charges and Length of Stay

3 Birth Defects Epidemiology & Surveillance Branch, Texas DSHS: 2 Components Texas Birth Defects Registry (TBDR) One of largest birth defects surveillance systems globally Monitor and describe the occurrence of birth defects in TX Conduct cluster investigations Work with others in research and prevention Family outreach Texas Center for Birth Defects Research and Prevention One of 10 in U.S. funded by CDC Collaborate in the National Birth Defects Prevention Study: largest of its kind to date Establish collaborative epidemiologic-genetic research - TX

4 15 Most Common Birth Defects Texas Atrial septal defect* Hypospadias (among males) Ventricular septal defect* Patent ductus arteriosus* Pyloric stenosis Talipes Equinovarus/clubfoot Trisomy 21 (Down syndrome) Cleft lip with/without cleft palate Pulmonary valve atresia/stenosis* Hydrocephaly (w/o spina bifida) Cleft palate alone (w/o cleft lip) Renal agenesis or dysgenesis Stenosis or atresia of large intestine Gastroschisis Transposition of the great vessels* 7,399 5,795 5,081 4,163 3,404 2,665 2,283 2,155 2,034 1,939 1,929 21,431 10,903 21,137 20, Prevalence (cases per 10,000 live births)

5 Cases per 10,000 Live Births Prevalence of Selected Heart Defects, by Maternal Race/Ethnicity, Texas, NH White NH Black Hispanic NH White NH Black Hispanic Hypoplastic Left Heart Syndrome Aortic Valve Stenosis

6 Cases per 10,000 Live Births Prevalence of Hypoplastic Left Heart Syndrome by Maternal Age and Sex of Infant/Fetus, Texas, < Male Female Mother s Age Sex of Infant/Fetus

7 Hypoplastic Left Heart Syndrome State rate = Statistically Significant or

8 Prevalence of Heart Defects by Body Mass Index and Diabetes Status

9 Percentage Obese Pre-pregnancy Obesity Reported on Texas Birth Certificates, % 20% 15% 10% 5% 0% Delivery Year

10 Body Mass Index (BMI) Maternal BMI = (weight [lb] 703)/height [in] 2 Categorized as: Underweight (BMI < 18.5) Normal weight ( ) Overweight ( ) Obese (BMI 30.0) Class I obese ( ) Class II obese ( ) Class III obese (40.0+)

11

12 Which Heart Defects Are Associated With BMI? Tetralogy of Fallot Ventricular Septal Defect* Atrial Septal Defect Pulmonary Valve Atresia/Stenosis Patent ductus arteriosis* Coarctation of the aorta Marengo L et al Body mass index and birth defects: Texas, Mat Child Health J (DOI /s )

13 Adjusted Prevalence Ratio Association Of BMI With Ventricular Septal Defect, By Diabetes Status, Texas, * * Ref. * * * * * 0.50 without diabetes with diabetes 0.00 adjusted for maternal race/ethnicity and maternal age *= statistically different than the referent of normal weight BMI Category Marengo L et al Body mass index and birth defects: Texas, Mat Child Health J (DOI /s )

14 Adjusted Prevalence Ratio Association Of BMI With Patent Ductus Arteriosus, By Diabetes Status, Texas, Ref. * * * * * * * 0.50 without diabetes with diabetes 0.00 adjusted for maternal race/ethnicity and maternal age *= statistically different than the referent of normal weight BMI Category Marengo L et al Body mass index and birth defects: Texas, Mat Child Health J (DOI /s )

15 Live Birth Prevalence of 7 Critical Congenital Heart Defects, Texas and US Average (Mean), Data from the Texas Birth Defects Registry (Provisional) and NBDPN Tetralogy of Fallot dextro-transposition of Great Arteries (d-tga) n=702 n=787 Hypoplastic Left Heart Syndrome Total Anomalous Pulmonary Venous Return (TAPVR) Pulmonary Valve Atresia Tricuspid Valve Atresia Common Truncus n=417 n=354 n=285 n=155 n= Live Born Cases Per 10,000 Live Births US Mean Texas Mai CT et al Selected birth defects data from population-based birth defects surveillance programs in the United States, : Featuring critical congenital heart defects targeted for pulse oximetry screening. BDRA; 94:

16 Mortality and Survival of Infants and Children in Texas with Selected Heart Defects

17 Effect of Acculturation and Distance from Cardiac Center on Infant Mortality from Severe Congenital Heart Disease Texas, Births during Eight severe heart defects* combined First year survival=59.9% No racial/ethnic differences in survival Lower survival among residents of Texas counties bordering Mexico Not significant: Parental birth country (Latin America- vs. US-born) Not significant: Distance from home to cardiac center Hypoplastic left heart syndrome Lower survival in Hispanics (compared to non-hispanic (NH) whites) *Hypoplastic Left Heart Syndrome, Single Ventricle, Pulmonary Valve Atresia with Intact Ventricular Septum, Pulmonary Valve Atresia with VSD, Tricuspid Atresia, Interrupted Aortic Arch, Ebstein s Malformation of the Tricuspid Valve, Truncus Arteriosus Fixler et al Effect of acculturation and distance from cardiac center on congenital heart disease mortality. Pediatrics

18 Racial/Ethnic Disparity in Early Childhood Mortality from Heart Defects Texas, Births during Higher risk of death in non-hispanic blacks (compared to NH whites): Transposition of the great arteries (adj. HR=2.04, 95% CI= ) Tetralogy of Fallot (adj. HR=1.85, 95% CI= ) Pulmonary valve atresia w/o VSD (adj. HR=2.60, 95% CI= ) Ventricular septal defect (adj. HR=1.56, 95% CI= ) Atrial septal defect (adj. HR=1.34, 95% CI= ) Higher risk of death in Hispanics (compared to NH whites): Pulmonary valve atresia w/o VSD (adj. HR=1.76, 95% CI= ) Hypoplastic left heart syndrome (adj. HR=1.51, 95% CI= ) Nembhard WN et al Racial/Ethnic disparities in risk of early childhood mortality among children with congenital heart defects. Pediatrics

19 Effect of Maternal Race/Ethnicity, Birth Era, and Extracardiac Defects on Five-Year Mortality from Functional Single Ventricle Defects, Births Defects Studied: Hypoplastic left heart syndrome, single ventricle, pulmonary atresia with intact ventricular septum, tricuspid atresia Race/Ethnicity: Non-Hispanic blacks had a 41% higher risk of death than NH whites (adj. HR=1.41, 95% CI= ) Hispanics had a 26% higher risk of death (adj HR=1.26, 95% CI= ) Birth Era: 47% lower risk of death for patients born vs (adj HR=0.53, 95% CI= ) Extracardiac Defects: 84% higher risk of death with extracardiac defects (adj HR=1.84, 95% CI= ) Fixler et al Mortality in first 5 years in infants with functional single ventricle born in Texas, 1996 to Circulation.

20 Five-year Survival for Texas Children with Defects resulting in Functional Single Ventricle, Fixler et al Mortality in first 5 years in infants with functional single ventricle born in Texas, 1996 to Circulation.

21 Hospital Charges and Median Length of Stay for Texas Children with Heart and Other Birth Defects

22 Total Hospital Charges Highest Total Hospital Charges for Congenital Anomalies (as Primary Diagnosis), Children 0-17 years, Texas, $500,000,000 $450,000,000 $400,000,000 $350,000,000 $300,000,000 $250,000,000 $200,000,000 $150,000,000 $100,000,000 $50,000,000 $0

23 Length of Stay (Days) Highest Median Length of Hospital Stay for Congenital Anomalies (as Primary Diagnosis), Children 0-17 years, Texas,

24 Mark A Canfield, PhD, Manager Birth Defects Epidemiology and Surveillance Branch Texas Department of State Health Services Phone: (512) THANK YOU Web site:

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