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BACKGROUND: Studies of outcomes among adults with congenital heart defects (CHDs) have focused on those receiving cardiac care, limiting generalizability. The Congenital Heart Survey To Recognize Outcomes, Needs, and well-beinG (CH STRONG) will assess comorbidities, health care utilization, quality of life, and social and educational outcomes from a US population-based sample of young adults living with CHD. METHODS: Individuals with CHD born between 1980 and 1997 were identified using active, population-based birth defects surveillance systems from 3 US locations (Arkansas [AR]; Arizona [AZ]; and Atlanta, Georgia [GA]) linked to death records. Individuals with current contact information responded to mailed survey materials during 2016 to 2019. Respondents and nonrespondents were compared using χ(2) tests. RESULTS: Sites obtained contact information for 74.6% of the 9,312 eligible individuals alive at recruitment. Of those, 1,656 returned surveys, either online (18.1%) or via paper (81.9%), for a response rate of 23.9% (AR: 18.3%; AZ: 30.7%; Atlanta, GA: 28.0%; P value < .01). For 20.0% of respondents, a proxy completed the survey, with 63.9% reporting that the individual with CHD was mentally unable. Among respondents and nonrespondents, respectively, sex (female: 54.0% and 47.3%), maternal race/ethnicity (non-Hispanic white: 74.3% and 63.0%), CHD severity (severe: 33.8% and 27.9%), and noncardiac congenital anomalies (34.8% and 38.9%) differed significantly (P value < .01); birth year (1991–1997: 56.0% and 57.5%) and presence of Down syndrome (9.2% and 8.9%) did not differ. CONCLUSIONS: CH STRONG will provide the first multisite, population-based findings on long-term outcomes among the growing population of US adults with CHD.

Background: Hypospadias is a common male birth defect that has shown widespread variation in reported prevalence estimates. Many countries have reported increasing trends over recent decades.Objective: To analyze the prevalence and trends of hypospadias for 27 international programs over a 31-yr period.Design, setting, and participants: The study population included live births, stillbirths, and elective terminations of pregnancy diagnosed with hypospadias during 1980-2010 from 27 surveillance programs around the world.Outcome measurements and statistical analysis: We used joinpoint regression to analyze changes over time in international total prevalence of hypospadias across programs, prevalence for each specific program, and prevalence across different degrees of severity of hypospadias.Results and limitations: The international total prevalence of hypospadias for all years was 20.9 (95% confidence interval: 19.2-22.6) per 10 000 births. The prevalence for each program ranged from 2.1 to 39.1 per 10 000 births. The international total prevalence increased 1.6 times during the study period, by 0.25 cases per 10 000 births per year (p Conclusions: Although there have been changes in clinical practice and registry ascertainment over time in some countries, the consistency in the observed increasing trends across many programs and by degrees of severity suggests that the total prevalence of hypospadias may be increasing in many countries. This observation is contrary to some previous reports that suggested that the total prevalence of hypospadias was no longer increasing in recent decades.Patient summary: We report on the prevalence and trends of hypospadias among 27 birth defect surveillance systems, which indicate that the prevalence of hypospadias continues to increase internationally. (C) 2019 European Association of Urology. Published by Elsevier B.V. All rights reserved.

Few reports have suggested that non-Hispanic (NH) blacks may present with different symptoms for COVID-19 than NH-whites. The objective of this study was to investigate patterns in symptomatology and COVID-19 outcomes by race/ethnicity among adults in Arkansas. Data on COVID-19 symptoms were collected on day of testing, 7th and 14th day among participants at UAMS mobile testing units throughout the state of Arkansas. Diagnosis for SARS-CoV-2 infection was confirmed via nasopharyngeal swab and RT-PCR methods. Data analysis was conducted using Chi-square test and Poisson regression to assess the differences in characteristics by race/ethnicity. A total of 60,648 individuals were RT-PCR tested from March 29, 2020 through October 7, 2020. Among adults testing positive, except shortness of breath, Hispanics were more likely to report all symptoms than NH-whites or NH-blacks. NH-whites were more likely to report fever (19.6% vs. 16.6%), cough (27.5% vs. 26.1%), shortness of breath (13.6% vs. 9.6%), sore throat (16.7% vs. 10.7%), chills (12.5% vs. 11.8%), muscle pain (15.6% vs. 12.4%), and headache (20.3% vs. 17.8%). NH-blacks were more likely to report loss of taste/smell (10.9% vs. 10.6%). To conclude, we found differences in COVID-19 symptoms by race/ethnicity, with NH-blacks and Hispanics more often affected with specific or all symptoms, compared to NH-whites. Due to the cross-sectional study design, these findings do not necessarily reflect biological differences by race/ethnicity; however, they suggest that certain race/ethnicities may have underlying differences in health status that impact COVID-19 outcomes.

BACKGROUND/OBJECTIVE. Research suggests gestational exposure to particulate matter ≤ 2.5 μm (PM(2.5)) and extreme heat may independently increase risk of birth defects. We investigated whether duration of gestational extreme heat exposure modifies associations between PM(2.5) exposure and specific congenital heart defects (CHDs). We also explored nonlinear exposure-outcome relationships. METHODS. We identified CHD case children (n = 2,824) and non-malformed live-birth control children (n = 4,033) from pregnancies ending between 1999 and 2007 in the National Birth Defects Prevention Study, a U.S. population-based multicenter case-control study. We assigned mothers 6-week averages of PM(2.5) exposure during the cardiac critical period (postconceptional weeks 3–8) using the closest monitor within 50 kilometers of maternal residence. We assigned a count of extreme heat days (EHDs, days above the 90th percentile of daily maximum temperature for year, season, and weather station) during this period using the closest weather station. Using generalized additive models, we explored logit-nonlinear exposure-outcome relationships, concluding logistic models were reasonable. We estimated joint effects of PM(2.5) and EHDs on six CHDs using logistic regression models adjusted for mean dewpoint and maternal age, education, and race/ethnicity. We assessed multiplicative and additive effect modification. RESULTS. Conditional on the highest observed EHD count (15) and at least one critical period day during spring/summer, each 5 μg/m³ increase in average PM(2.5) exposure was significantly associated with perimembranous ventricular septal defects (VSDpm; OR: 1.54 [95% CI: 1.01, 2.41]). High EHD counts (8+) in the same population were positively, but non-significantly, associated with both overall septal defects and VSDpm. Null or inverse associations were observed for lower EHD counts. Multiplicative and additive effect modification estimates were consistently positive in all septal models. CONCLUSIONS. Results provide limited evidence that duration of extreme heat exposure modifies the PM(2.5)-septal defects relationship. Future research with enhanced exposure assessment and modeling techniques could clarify these relationships.

To determine the effects of maternal periconceptional supplementation with folic acid or multiple micronutrients containing folic acid on the prevention of fetal congenital heart defects (CHDs).Data were drawn from a Prenatal Health Care System and a Birth Defects Surveillance System in a district of Beijing, China. A total of 63 969 singleton births, live or stillborn, 308 CHDs among them, during 2013 to 2018 were included. Associations between different patterns of supplementation and risk for total CHDs or main types of CHDs were evaluated with risk ratios (RRs).For folic acid or multiple micronutrients containing folic acid users compared with nonusers, the adjusted RRs (ARRs) for total CHDs, critical CHD, and ventricular septal defect (VSD) were 0.60 (95% CI, 0.44-0.83), 0.41 (95% CI, 0.26-0.67), and 0.47 (95% CI, 0.30-0.74), respectively. When we compared multiple micronutrients containing folic acid users with folic acid users, the ARRs were 0.84 (95% CI, 0.66-1.09), 0.64 (95% CI, 0.41-1.00), and 0.94 (95% CI, 0.63-1.41) for total CHDs, critical CHD, and VSD, respectively. We also found that, compared with supplementation initiated after conception, supplementation initiated before conception was associated with a lower risk for CHDs: the ARRs were 0.68 (95% CI, 0.48-0.95) for total CHDs and 0.26 (95% CI, 0.10-0.71) for critical CHD, but 1.08 (95% CI, 0.63-1.83) for VSD.Maternal periconceptional supplementation with folic acid or multiple micronutrients containing folic acid seems to decrease the risk for CHDs, especially critical CHD, in offspring. Supplementation confers a greater protective effect when it is initiated before conception. We did not find any difference between folic acid and multiple micronutrients containing folic acid in terms of preventing CHDs.

Information on dietary intakes of Jamaican immigrants in the United States is sparse. Understanding factors that influence diet is important since diet is associated with chronic diseases. This study examined the association between acculturation, socio-cultural factors, and dietary pattern among Jamaican immigrants in Florida. Jamaican persons 25–64 years who resided in two South Florida counties were recruited for participation. A health questionnaire that assessed acculturation, dietary pattern, and risk factors for cardiovascular disease was administered to participants. Generalized Estimating Equations were used to determine associations. Acculturation score was not significantly associated with dietary intake pattern (β = − 0.02 p = 0.07). Age at migration was positively associated with traditional dietary pattern (β = 0.02 p, Highlights • Age at migration was associated with traditional diet among Jamaican immigrants. • Persons with lower educational attainment were more likely to eat traditional foods. • Residing in an ethnic enclave was not associated with having a traditional diet.

Purpose The purpose of this study was to estimate the effect of cigarette smoking and electronic nicotine delivery systems (ENDS) use during pregnancy on small for gestational age (SGA). Methods We used data from the 2016-2017 Arkansas Pregnancy Risk Assessment Monitoring System, estimated the risk ratio (RR) for SGA for maternal prenatal cigarette smoking–only use, cigarette smoking, and ENDS use (i.e., dual use), with no prenatal tobacco use as referent, accounting for the complex survey design. We also explored these effects for women who stopped smoking cigarettes during pregnancy among cigarette smokers and dual users. Results Estimated adjusted RR for SGA for cigarette-only users was 1.7 (95% confidence interval [CI]: 1.1, 2.7), and 1.8 (95% CI: 1.0, 3.4) for dual users. These RR estimates increased after correcting for tobacco use misclassification. Women who were dual users (cigarette smokers and ENDS users) and continued using ENDS but stopped smoking cigarettes had an increased risk for SGA compared with nontobacco users, 3.2 (95% CI: 1.5, 6.6). Conclusions Our results in a population representative sample are consistent with the hypothesis that exposure to both maternal cigarette smoking and ENDS use increased the risk of SGA. Dual users still had an elevated risk of SGA after smoking cessation.

Background Although there is evidence in experimental model systems that exposure to polycyclic aromatic hydrocarbons (PAHs) is linked with congenital heart defects (CHDs), few studies have examined the association in humans. We conducted a case-control study to examine the association between maternal exposure to PAHs and CHDs in offspring using data from the National Birth Defects Prevention Study (NBDPS) (1997–2011). Methods We obtained detailed information on maternal occupation during the month before to three months after conception. Expert raters, masked to case-control status, assessed job descriptions to assign categorical levels of exposure. Categories were quantitatively mapped to estimate cumulative exposure to PAHs, incorporating exposure intensity, frequency, work duration, and work hours. Quartiles were generated for cumulative maternal exposure to PAHs. Crude and adjusted odds ratios (ORs) with 95% confidence intervals (CIs) were estimated using unconditional logistic regression for quartiles of PAH exposure and six CHD groupings (e.g. conotruncal) and specific subtypes (e.g. tetralogy of Fallot [ToF]). Final models were adjusted for maternal age, race/ethnicity, education, smoking, anticonvulsant use, folic acid supplementation, and study center. Results There were 4,775 case and 7,734 control infants eligible for the study. The prevalence of occupational exposure to PAHs was 10.2% among both case and control mothers. In adjusted analysis, compared to mothers with no occupational PAH exposure, those in the highest quartile of exposure were more likely to have offspring in the conotruncal heart defects group (OR 1.41; 95% CI 1.00–2.00), and with ToF (OR 1.83; 95% CI 1.21–2.78). Conclusions Women in the highest quartile of estimated cumulative occupational PAH exposure during early pregnancy were more likely to have offspring with conotruncal heart defects, specifically ToF, compared to women with no occupational PAH exposure. Other comparisons between PAHs and other CHDs subgroups did not show any statistically precise associations.

Purpose Studies suggest exposure to ambient particulate matter less than 2.5 μg/m3 in aerodynamic diameter (PM2.5) may be associated with preterm birth (PTB), but few have evaluated how this is modified by ambient temperature. We investigated the relationship between PM2.5 exposure during pregnancy and PTB in infants without birth defects (1999–2006) and enrolled in the National Birth Defects Prevention Study and how it is modified by concurrent temperature. Methods PTB was defined as spontaneous or iatrogenic delivery before 37 weeks. Exposure was assigned using inverse distance weighting with up to four monitors within 50 kilometers of maternal residence. To account for state-level variations, a Bayesian two-level hierarchal model was developed. Results PTB was associated with PM2.5 during the third and fourth months of pregnancy (range: (odds ratio (95% confidence interval) = 1.00 (0.35, 2.15) to 1.49 (0.82, 2.68) and 1.31 (0.56, 2.91) to 1.62 (0.7, 3.32), respectively); no week of exposure conveyed greater risk. Temperature may modify this relationship; higher local average temperatures during pregnancy yielded stronger positive relationships between PM2.5 and PTB compared to nonstratified results. Conclusions Results add to literature on associations between PM2.5 and PTB, underscoring the importance of considering co-exposures when estimating effects of PM2.5 exposure during pregnancy.

Background Substance use during pregnancy is a major medical and public health concern. Determination of the most appropriate screening protocol remains a clinical conundrum. Interviews and/or laboratory drug screens may be costly, inaccurate, and are frequently inadequate to identify patterns of substance use for a given population or geographic area. We compared commercially available urine “dip cup” toxicology screens obtained in the clinic to university hospital drug toxicology results. Methods 267 observed urine samples were collected from pregnant women with known substance use disorders enrolled in a specialized treatment program that included access to buprenorphine medication-assisted treatment. Each urine sample was tested by commercial dip cup with temperature confirmation and then sent to the university hospital laboratory for analyses. The number of substances detected and cost for each screening method were compared. Results Uniformly, the dip cup had comparable detection of amphetamines, barbiturates, cocaine, methadone, opiates, and tetrahydrocannabinol to the university hospital laboratory with the exception of benzodiazepines. In addition, the dip cup detected use of buprenorphine (a commonly misused opiate receptor ligand not included in the hospital screen) and was significantly less expensive. Conclusions Commercially available urine dip cups are cost-effective, equally comparable to hospital based screening, and provide ‘real time’ results germane to clinical care and treatment planning.

Objective To determine the prevalence of major congenital heart defects (CHD) by ethnicity and sex. Study design Data from the Florida Birth Defects Registry was used to conduct a retrospective cohort study with 8029 singleton infants with 11 CHDs born 1998–2003 to resident non-Hispanic (NH) white, NH-black, and Hispanic women aged 15 to 49. Defect-specific prevalence rates, ratios, and 95% confidence intervals were calculated. Poisson regression was used to calculate adjusted ethnic-specific rate ratios (RR) for each CHD. Statistical significance was P Results Compared with NH-whites, NH-black males had significantly increased rates of pulmonary valve atresia/stenosis (RR = 1.66) but lower prevalence of aortic valve atresia/stenosis (RR = 0.33) and ventricular septal defect (VSD; RR=0.78). Hispanic males had lower rates of aortic valve atresia/stenosis (RR = 0.28), coarctation of the aorta (RR = 0.61) and VSD (RR = 0.79). NH-black females had statistically significantly lower rates of VSD (RR = 0.75), and Hispanic females had lower rates of tetralogy of Fallot (RR = 0.54), VSD (RR = 0.84) and atrioventricular septal defects (RR = 0.53) compared with NH-whites. Conclusions We found differences in ethnic susceptibilities to CHD by sex, but the cause remains unclear.

PURPOSE: Congenital heart defects (CHDs) are the most prevalent birth defects. Infants with CHDs more often are small-for-gestational age (SGA) than infants without CHD; however, little is known about racial/ethnic variations in prevalence of SGA or large-for-gestational age (LGA) for infants born with CHDs. This study determined the risk of SGA and LGA for non-Hispanic (NH)-black and Hispanic infants with CHDs. METHODS: Data from the Florida Birth Defects Registry were used in a retrospective cohort study of 10,027 live-born infants to resident NH-White, NH-Black, and Hispanic women ages 15-49 years from January 1, 1998, to December 31, 2003, and diagnosed with 11 CHDs. Defect-specific odds ratios and 95% confidence intervals were computed for risk of SGA and LGA by race/ethnicity and adjusted for covariates using multinomial logistic regression. RESULTS: After adjusting for covariates, we found there were no statistically significant racial/ethnic differences in risk of SGA. However, NH-Blacks with ventricular septal defect had increased risk of LGA and NH-Blacks with tetralogy of Fallot had decreased risk of LGA compared to NH-Whites. CONCLUSIONS: Very few racial/ethnic differences in fetal growth are present among infants with CHD. Further elucidation of the factors involved in fetal growth and the impact of CHD itself on fetal development is needed.

Objectives To investigate survival up to early adulthood for children with intellectual disability and compare their risk of mortality with that of children without intellectual disability. Study design This was a retrospective cohort study of all live births in Western Australia between January 1, 1983 and December 31, 2010. Children with an intellectual disability (n = 10 593) were identified from the Western Australian Intellectual Disability Exploring Answers Database. Vital status was determined from linkage to the Western Australian Mortality database. Kaplan-Meier product limit estimates and 95% CIs were computed by level of intellectual disability. Hazard ratios (HRs) and 95% CIs were calculated from Cox proportional hazard regression models adjusting for potential confounders. Results After adjusting for potential confounders, compared with those without intellectual disability, children with intellectual disability had a 6-fold increased risk of mortality at 1-5 years of age (adjusted HR [aHR] = 6.0, 95%CI: 4.8, 7.6), a 12-fold increased risk at 6-10 years of age (aHR = 12.6, 95% CI: 9.0, 17.7) and a 5-fold increased risk at 11-25 years of age (aHR = 4.9, 95% CI: 3.9, 6.1). Children with severe intellectual disability were at even greater risk. No difference in survival was observed for Aboriginal children with intellectual disability compared with non-Aboriginal children with intellectual disability. Conclusions Although children with intellectual disability experience higher mortality at all ages compared with those without intellectual disability, the greatest burden is for those with severe intellectual disability. However, even children with mild to moderate intellectual disability have increased risk of death compared with unaffected children.