Jane A. Correia

Hospitalizations, costs, and mortality among infants with critical congenital heart disease: How important is timely detection?

BACKGROUND Critical congenital heart disease (CCHD) was recently added to the U.S. Recommended Uniform Screening Panel for newborns. States considering screening requirements may want more information about the potential impact of screening. This study examined potentially avoidable mortality among infants with late detected CCHD and assessed whether late detection was associated with increased hospital resource use during infancy. METHODS This was a state-wide, population-based, observational study of infants with CCHD (n = 3603) born 1998 to 2007 identified by the Florida Birth Defects Registry. We examined 12 CCHD conditions that are targets of newborn screening. Late detection was defined as CCHD diagnosis after the birth hospitalization. Deaths potentially avoidable through screening were defined as those that occurred outside a hospital following birth hospitalization discharge and those that occurred within 3 days of an emergency readmission. RESULTS For 23% (n = 825) of infants, CCHD was not detected during the birth hospitalization. Death occurred among 20% (n = 568/2,778) of infants with timely detected CCHD and 8% (n = 66/825) of infants with late detected CCHD, unadjusted for clinical characteristics. Potentially preventable deaths occurred in 1.8% (n = 15/825) of infants with late detected CCHD (0.4% of all infants with CCHD). In multivariable models adjusted for selected characteristics, late CCHD detection was significantly associated with 52% more admissions, 18% more hospitalized days, and 35% higher inpatient costs during infancy. CONCLUSION Increased CCHD detection at birth hospitals through screening may lead to decreased hospital costs and avoid some deaths during infancy. Additional studies conducted after screening implementation are needed to confirm these findings.

Factors associated with late detection of critical congenital heart disease in newborns.

OBJECTIVES: Critical congenital heart disease (CCHD) was recently added to the US Recommended Uniform Screening Panel for newborns. This study assessed whether maternal/household and infant characteristics were associated with late CCHD detection. METHODS: This was a statewide, population-based, retrospective, observational study of infants with CCHD born between 1998 and 2007 identified by using the Florida Birth Defects Registry. We examined 12 CCHD conditions that are primary and secondary targets of newborn CCHD screening using pulse oximetry. We used Poisson regression models to analyze associations between selected characteristics (eg, CCHD type, birth hospital nursery level [highest level available in the hospital]) and late CCHD detection (defined as diagnosis after the birth hospitalization). RESULTS: Of 3603 infants with CCHD and linked hospitalizations, CCHD was not detected during the birth hospitalization for 22.9% (n = 825) of infants. The likelihood of late detection varied by CCHD condition. Infants born in a birth hospital with a level I nursery only (adjusted prevalence ratio: 1.9 [95% confidence interval: 1.6–2.2]) or level II nursery (adjusted prevalence ratio: 1.5 [95% confidence interval: 1.3–1.7]) were significantly more likely to have late-detected CCHD compared with infants born in a birth hospital with a level III (highest) nursery. CONCLUSIONS: After controlling for the selected characteristics, hospital nursery level seems to have an independent association with late CCHD detection. Thus, perhaps universal newborn screening for CCHD could be particularly beneficial in level I and II nurseries and may reduce differences in the frequency of late diagnosis between birth hospital facilities.

Associations between exposure to ambient benzene and PM2.5 during pregnancy and the risk of selected birth defects in offspring

OBJECTIVE A growing number of studies have investigated the association between air pollution and the risk of birth defects, but results are inconsistent. The objective of this study was to examine whether maternal exposure to ambient PM2.5 or benzene increases the risk of selected birth defects in Florida. METHODS We conducted a retrospective cohort study of singleton infants born in Florida from 2000 to 2009. Isolated and non-isolated birth defect cases of critical congenital heart defects, orofacial clefts, and spina bifida were identified from the Florida Birth Defects Registry. Estimates of maternal exposures to PM2.5 and benzene for all case and non-case pregnancies were derived by aggregation of ambient measurement data, obtained from the US Environmental Protection Agency Air Quality System, during etiologically relevant time windows. Multivariable Poisson regression was used to estimate adjusted prevalence ratios (aPRs) and 95% confidence intervals (CIs) for each quartile of air pollutant exposure. RESULTS Compared to the first quartile of PM2.5 exposure, higher levels of exposure were associated with an increased risk of non-isolated truncus arteriosus (aPR4th Quartile, 8.80; 95% CI, 1.11-69.50), total anomalous pulmonary venous return (aPR2nd Quartile, 5.00; 95% CI, 1.10-22.84), coarctation of the aorta (aPR4th Quartile, 1.72; 95% CI, 1.15-2.57; aPR3rd Quartile, 1.60; 95% CI, 1.07-2.41), interrupted aortic arch (aPR4th Quartile, 5.50; 95% CI, 1.22-24.82), and isolated and non-isolated any critical congenital heart defect (aPR3rd Quartile, 1.13; 95% CI, 1.02-1.25; aPR4th Quartile, 1.33; 95% CI, 1.07-1.65). Mothers with the highest level of exposure to benzene were more likely to deliver an infant with an isolated cleft palate (aPR4th Quartile, 1.52; 95% CI, 1.13-2.04) or any orofacial cleft (aPR4th Quartile, 1.29; 95% CI, 1.08-1.56). An inverse association was observed between exposure to benzene and non-isolated pulmonary atresia (aPR4th Quartile, 0.19; 95% CI, 0.04-0.84). CONCLUSION Our results suggest a few associations between exposure to ambient PM2.5 or benzene and specific birth defects in Florida. However, many related comparisons showed no association. Hence, it remains unclear whether associations are clinically significant or can be causally related to air pollution exposures.

Hospital use, associated costs, and payer status for infants born with spina bifida.

BACKGROUND Health care use and costs for children with spina bifida (SB) are significantly greater than those of unaffected children. Little is known about hospital use and costs across health insurance payer types. We examined hospitalizations and associated costs by sociodemographic characteristics and payer type during the first year of life among children with SB. We also examined changes in health insurance payer status. METHODS This study was a retrospective, statewide population-based analysis of infants with SB without anencephaly born in Florida during 1998-2007. Infants were identified by the Florida Birth Defects Registry and linked to hospital discharge records. Descriptive statistics on number of hospitalizations, length of stay, and estimated hospital costs per hospitalization and per infant were calculated during the first year of life. Results were stratified by selected sociodemographic variables and health insurance payer type. RESULTS Among 615 infants with SB, mean and median numbers of hospitalizations per infant were 2.4 and 2.0, respectively. Mean and median total days of hospitalization per infant were 25.2 and 14.0 days, respectively. Approximately 18% of infants were hospitalized more than three times. Among infants with multiple hospitalizations, 16.7% had a mix of public and private health insurance payers. Almost 60% of hospitalizations for infants were paid by public payer sources. Mean and median estimated hospital costs per infant were

Role of health insurance on the survival of infants with congenital heart defects.

39,059 and

Hospitalizations and Associated Costs in a Population-Based Study of Children with Down Syndrome Born in Florida

21,937, respectively. CONCLUSIONS Results suggest a small percentage of infants with SB have multiple hospitalizations with high costs. Further analysis on factors associated with length of stay, hospitalizations, and costs is warranted.

The Accuracy of Hospital Discharge Diagnosis Codes for Major Birth Defects: Evaluation of a Statewide Registry With Passive Case Ascertainment.

OBJECTIVES We examined the association between health insurance and survival of infants with congenital heart defects (CHDs), and whether medical insurance type contributed to racial/ethnic disparities in survival. METHODS We conducted a population-based, retrospective study on a cohort of Florida resident infants born with CHDs between 1998 and 2007. We estimated neonatal, post-neonatal, and infant survival probabilities and adjusted hazard ratios (AHRs) for individual characteristics. RESULTS Uninsured infants with critical CHDs had 3 times the mortality risk (AHR = 3.0; 95% confidence interval = 1.3, 6.9) than that in privately insured infants. Publicly insured infants had a 30% reduced mortality risk than that of privately insured infants during the neonatal period, but had a 30% increased risk in the post-neonatal period. Adjusting for insurance type reduced the Black-White disparity in mortality risk by 50%. CONCLUSIONS Racial/ethnic disparities in survival were attenuated significantly, but not eliminated, by adjusting for payer status.

Factors associated with high hospital resource use in a population-based study of children with orofacial clefts

BACKGROUND Our objective was to examine differences in hospital resource usage for children with Down syndrome by age and the presence of other birth defects, particularly severe and nonsevere congenital heart defects (CHDs). METHODS This was a retrospective, population-based, statewide study of children with Down syndrome born 1998 to 2007, identified by the Florida Birth Defects Registry (FBDR) and linked to hospital discharge records for 1 to 10 years after birth. To evaluate hospital resource usage, descriptive statistics on number of hospitalized days and hospital costs were calculated. Results were stratified by isolated Down syndrome (no other coded major birth defect); presence of severe and nonsevere CHDs; and presence of major FBDR-eligible birth defects without CHDs. RESULTS For 2552 children with Down syndrome, there were 6856 inpatient admissions, of which 68.9% occurred during the first year of life (infancy). Of the 2552 children, 31.7% (n = 808) had isolated Down syndrome, 24.0% (n = 612) had severe CHDs, 36.3% (n = 927) had nonsevere CHDs, and 8.0% (n = 205) had a major FBDR-eligible birth defect in the absence of CHD. Infants in all three nonisolated DS groups had significantly higher hospital costs compared with those with isolated Down syndrome. From infancy through age 4, children with severe CHDs had the highest inpatient costs compared with children in the other sub-groups. CONCLUSION Results support findings that for children with Down syndrome the presence of other anomalies influences hospital use and costs, and children with severe CHDs have greater hospital resource usage than children with other CHDs or major birth defects without CHDs.

Birth defects surveillance in Florida: infant death certificates as a case ascertainment source.

CONTEXT Birth defects prevention, research, education, and support activities can be improved through surveillance systems that collect high-quality data. OBJECTIVE To estimate the overall and defect-specific accuracy of Florida Birth Defects Registry (FBDR) data, describe reasons for false-positive diagnoses, and evaluate the impact of statewide case confirmation on frequencies and prevalence estimates. DESIGN Retrospective cohort evaluation study. PARTICIPANTS A total of 8479 infants born to Florida resident mothers between January 1, 2007, and December 31, 2011, and diagnosed with 1 of 13 major birth defects in the first year of life. MAIN OUTCOME MEASURES Positive predictive value: calculated overall (proportion of FBDR-identified cases confirmed by medical record review, regardless of which of the 13 defects were confirmed) and defect-specific (proportion of FBDR-identified cases confirmed by medical record review with the same defect) indices. RESULTS The FBDRs overall positive predictive value was 93.3% (95% confidence interval, 92.7-93.8); however, there was variation in accuracy across defects, with positive predictive values ranging from 96.0% for gastroschisis to 54.4% for reduction deformities of the lower limb. Analyses suggested that International Classification of Diseases, Ninth Edition, Clinical Modification, codes, upon which FBDR diagnoses are based, capture the general occurrence of a defect well but often fail to identify the specific defect with high accuracy. Most infants with false-positive diagnoses had some type of birth defect that was incorrectly documented or coded. If prevalence rates reported by the FBDR for these 13 defects were adjusted to incorporate statewide case confirmation, there would be an overall 6.2% rate reduction from 82.6 to 77.5 per 10 000 live births. CONCLUSIONS A statewide birth defects surveillance system, relying on linkage of administrative databases, is capable of achieving high accuracy (>93%) for identifying infants with any one of the 13 major defects included in this study. However, the level of accuracy and the ability to minimize false-positive diagnoses vary depending on the defect.

Uncertainty in maternal exposures to ambient PM2.5 and benzene during pregnancy: Sensitivity to exposure estimation decisions.

BACKGROUND Little is known about population-based maternal, child, and system characteristics associated with high hospital resource use for children with orofacial clefts (OFC) in the US. METHODS This was a statewide, population-based, retrospective observational study of children with OFC born between 1998 and 2006, identified by the Florida Birth Defects Registry whose records were linked with longitudinal hospital discharge records. We stratified the descriptive results by cleft type [cleft lip with cleft palate, cleft lip, and cleft palate] and by isolated versus nonisolated OFC (accompanied by other coded major birth defects). We used Poisson regression to analyze associations between selected characteristics and high hospital resource use (≥90th percentile of estimated hospitalized days and inpatient costs) for birth, postbirth, and total hospitalizations initiated before age 2 years. RESULTS Our analysis included 2,129 children with OFC. Infants who were born low birth weight (<2500 grams) were significantly more likely to have high birth hospitalization costs for CLP (adjusted prevalence ratio: 1.6 [95% confidence interval: 1.0-2.7]), CL (adjusted prevalence ratio: 3.0 [95% confidence interval: 1.1-8.1]), and CP (adjusted prevalence ratio: 2.3 [95% confidence interval: 1.3-4.0]). Presence of multiple birth defects was significantly associated with a three- to eleven-fold and a three- to nine-fold increase in the prevalence of high costs and number of hospitalized days, respectively; at birth, postbirth before age 2 years and overall hospitalizations. CONCLUSION Children with cleft palate had the greatest hospital resources use. Additionally, the presence of multiple birth defects contributed to greater inpatient days and costs for children with OFC.