About Congenital Heart Defects and Our Program

Recent Publications

Raskind-Hood CL, Kancherla V, Ivey LC, Rodriguez FH, Sullivan AM, Lui GK, Botto L, Feldkamp M, Li JS, D’Ottavio A, et al. Racial and Ethnic Disparities in Healthcare Utilization and Mortality by Neighborhood Poverty among Individuals with Congenital Heart Defects, four U.S. Surveillance Sites, 2011-2013. medRxiv. 2024. doi: 10.1101/2023.12.12.23299887

Ivey LC, Ahmad A, Chen J, Rodriguez Iii FH, Raskind-Hood C, Book WM. Anatomic and physiologic classification of adults with congenital heart disease to predict adverse outcomes: Use of administrative codes compared to clinical staging. American Heart Journal. 2024;271:12-19. doi: https://doi.org/10.1016/j.ahj.2024.02.007

Ivey LC, Rodriguez FH, Shi H, Chong C, Chen J, Raskind‐Hood CL, Downing KF, Farr SL, Book WM. Positive Predictive Value of International Classification of Diseases, Ninth Revision, Clinical Modification, and International Classification of Diseases, Tenth Revision, Clinical Modification, Codes for Identification of Congenital Heart Defects. Journal of the American Heart Association. 2023;12:e030821. doi: 10.1161/JAHA.123.030821

Guo Y, Al‐Garadi MA, Book WM, Ivey LC, Rodriguez FH, Raskind‐Hood CL, Robichaux C, Sarker A. Supervised Text Classification System Detects Fontan Patients in Electronic Records With Higher Accuracy Than ICD Codes. Journal of the American Heart Association. 2023;12:e030046. doi: 10.1161/JAHA.123.030046

Rodriguez FH, 3rd, Raskind-Hood CL, Hoffman T, Farr SL, Glidewell J, Li JS, D'Ottavio A, Botto L, Reeder MR, Hsu D, et al. How Well Do ICD-9-CM Codes Predict True Congenital Heart Defects? A Centers for Disease Control and Prevention-Based Multisite Validation Project. J Am Heart Assoc. 2022;11:e024911. doi: 10.1161/jaha.121.024911

Glidewell J, Farr SL, Book, WM, Botto L, Li JS, Soim AS, Downing K, Riehle-Colarusso T, D’Ottavio AA, Feldkamp ML, Khanna AD, Raskind-Hood CL, Sommerhalter KM, Crume TI. Individuals aged 1-64 years with documented congenital heart defects at healthcare encounters, five U.S. surveillance sites, 2011-2013. American Heart Journal. 2021 May 2;S0002-8703(21):00109-5. https://doi.org/10.1016/j.ahj.2021.04.007

Raskind-Hood C, Gray K, Morgan J, Book W. Hypertension and Heart Failure as Predictors of Mortality in the Adult Congenital Heart Defect (ACHD) Population. Congenital Heart Disease. 2021 April;16(4): 333-355. doi:10.32604/CHD.2021.014384

Everitt I, Hoffman T, Raskind-Hood C, Saraf A, Rodriguez F, Hogue C, Book W. Predictors of 30-Day Readmission Following Congenital Heart Surgery across the Lifespan. Cardiology in the Young. 2020 Sep;30(9):1297-1304. https://doi:10.1017/S1047951120002012. Epub 2020 Aug 5. PMID: 32753074.

Gurvitz M, Dunn J, Bhatt A, Book W, Glidewell J, Hogue C, Lin A, Lui G, McGarry C, Raskind-Hood C, Van Zutphen A, Zaidi A, Jenkins K, Riehle-Colarusso T. Characteristics of Adults with Congenital Heart Defects at Three U.S. Surveillance Sites. Journal of the American College of Cardiology. 2020 July; 76(2): 175-182. https://doi.org/10.1016/j.jacc.2020.05.025

Raskind-Hood C, Saraf A, Colarusso T, Glidewell J, Van Zutphen A, Gurvitz M, Dunn J, Lui G, McGarry C, Hogue C, Hoffman T, Rodriguez F, Book W. Assessing Pregnancy, Gestational Complications, and Comorbidities in Women with Congenital Heart Defects (Data from ICD-9-CM Codes in Three U.S. Surveillance Sites). American Journal of Cardiology. 2020 March; 125(5): 812-819. https://doi.org/10.1016/j.amjcard.2019.12.001

Gaydos, L, Sommerhalter K, Raskind-Hood C, Fapo O, Lui G, Hsu D, Van Zutphen A, Glidewell J, Farr S, Rodriguez F, Hoffman T, Book W. Health Care Transition Perceptions among Parents of Adolescents with Congenital Heart Defects in Georgia and New York. Pediatric Cardiology. 2020; 41:1220-1230. https://doi.org/10.1007/s00246-020-02378-z

Gerardin JF, Raskind-Hood C, Hoffman T, Well A, Rodriguez F, Kalogeropoulos A, Hogue, C, Book W. Lost in the system? Transfer to adult congenital heart disease care - Challenges and solutions. Congenital Heart Disease. 2019 July; 14(4): 541-548. https://doi:org/10.1111/chd.12780

Raskind-Hood C, John K, Book W, Hogue C. Estimates of Adolescent and Adult Congenital Heart Defect Prevalence in Metropolitan Atlanta, 2010, Using Capture–Recapture Applied to Administrative Records. Annals of Epidemiology. 2019 April; 32: 72-77. https://doi.org/10.1016/j.annepidem.2018.11.012

Lui G, McGarry C, Bhatt, A, Book, W., Riehle-Colarusso T, Dunn J, Glidewell J, Gurvitz M, Hoffman T, Hogue C, Hsu D, Obenhaus S, Raskind-Hood C, Rodriguez F, Zaidi A, Van Zutphen A. Surveillance of Congenital Heart Defects among Adolescents at Three Sites in the U.S. Sites. 2019 April. American Journal of Cardiology; 124(1): 137-143. https://doi.org/10.1016/j.amjcard.2019.03.044

Glidewell J, Book, W., Raskind-Hood C, Hogue C, Dunn J, Gurvitz M, Ozonoff A, McGarry C, Van Zutphen A, Lui G, Downing K, Riehle-Colarusso T. Population-Based Surveillance of Congenital Heart Defects among Adolescents and Adults. Birth Defects Research. 2018 November 15; 110(19): 1395-1403. https://doi.10.1002/bdr2.1400

Rodriguez F, Ephrem G, Gerardin J, Raskind-Hood C, Hogue C, Book W. The 745.5 Issue in Code-based, Adult Congenital Heart Disease Population Studies: Relevance to Current and Future ICD-9-CM and ICD-10-CM Studies. Congenital Heart Disease. 2018 January; 13(1): 59-64. https://DOI.10.1111/chd.12563

Shi H, Book W, Raskind-Hood C, Downing KF, Farr SL, Bell MN, Sameni R, Rodriguez Iii FH, Kamaleswaran R. A machine learning model for predicting congenital heart defects from administrative data. Birth Defects Research. 2023;115:1693-1707. doi: https://doi.org/10.1002/bdr2.2245

Manuscripts in Progress

Lui GK, Sommerhalter K, Xi Y, Botto L, Crume T., Farr S, Feldkamp M, Glidewell J, Hsu D, Khanna A, Krikov S, Li J, Raskind-Hood C, Sarno L, Van Zutphen AR, Zaidi A, Soim A, Book W. Healthcare Utilization among Adolescents with Congenital Heart Defects at Five Sites in the United States, 2011-2013

Spears T, D’Ottavio A, Book W, Raskind-Hood C, … Whitehead K, Hoskoppal A, Feldkamp M, Botto L. Pregnancy and Pregnancy Outcomes in Women with Congenital Heart Disease: A Population-based Assessment from Five Sites in the U.S.

Health Care Utilization Patterns among Adults with Congenital Heart Defects from an Integrated Surveillance System.

Spectrum and Prevalence of Heart Failure in Children and Adults with Congenital Heart Disease.

Prevalence of Co-morbidities in Congenital Heart Disease: An Analysis of Congenital Heart Defects Across the Lifespan.

Cognitive Disorders Among Individuals with Congenital Heart Defects.

Goldstein S, Li JS, D’Ottavio A, Spears T, Chiswell K, Hartman RJ, Kemper AR, Forestieri N, Hoffman TM, Walsh MJ, Sang CJ, Welke K, Krikov S, Raskind-Hood CL, Book WM, Botto L. Neonatal Outcomes of Pregnancy in Women with Congenital Heart Disease.

Healthcare Expenditures in Children with Congenital Heart Defects Insured by Medicaid in Georgia and New York.

Reynolds E, Raskind-Hood CL, Book W, Blake S. Mental Health Service Utilization among Congenital Heart Defect Medicaid Patients Experiencing Pregnancy.

Rink D, Kancherla V, Raskind-Hood C, Rodriguez F, … Book, W Association between Combined Anatomic and Physiologic Classification of Adults with Congenital Heart Defects and Selected Healthcare Utilization and Clinical Outcomes.

Alam Z, Raskind-Hood C, …. Book W. The Risk of Cerebrovascular Accident among Patients with Congenital Heart Disease.

Graham N, Raskind-Hood C, Book W, Rodriguez, F. Association between Diabetes Mellitus and Hospitalization in the Adult Congenital Heart Disease Population.

Licitra G, Raskind-Hood C, Ivey, L, Hoffman T, Hogue C, Rodriguez F, Book W. Predictors Associated with 30-day Readmission among a Cohort of Adult Congenital Heart Disease Patients, Medicaid Claims Data 2010-2013.

Raskind-Hood C, Lowe J, Ivey L, Hogue C, Book W. Severity of Congenital Heart Disease as a Predictor for Preterm Birth.

Kancherla V, Raskind-Hood CL, Elani, H, Book W. Dental care utilization among individuals with congenital heart defects enrolled in Medicaid.

Validation of CCS Codes 177- 196 to Detect Pregnancy in the Georgia Congenital Heart Defect Repository, 2011-2013.

Joshi, K.  Preventive Dental Care Utilization and Infective Endocarditis among Medicaid Beneficiaries with Congenital Heart Defects in Georgia, 2008-2019 (RSPH, Department of Epidemiology, 2024)

Children with CHD are recommended to receive preventive dental care due to their susceptibility to infective endocarditis (IE). Understanding the association of preventive dental care utilization and IE is crucial for informing targeted interventions that could improve oral health outcomes and mitigate IE burden for children with CHD. This retrospective cohort study assessed the association between preventive dental care utilization and IE among 61,024 children with CHD, aged 1 to 18 years, enrolled in Georgia Medicaid between 2008-2019. Logistic regression models estimated crude and adjusted risk ratios (RRs) and 95% confidence intervals (CIs) for IE risk, controlling for CHD anatomic severity, sex, race and ethnicity, birth year cohort, Social Deprivation Index (SDI), and rurality. Among all CHD patients, 181 IE (0.30%) cases were identified, and 65.64% had at least one annual preventative dental visit. For subgroups with valve lesions or shunt lesions, having at least one annual preventive dental care visit showed a significantly lower risk of IE (aRR=0.32, 95% CI: 0.18-0.56, and aRR=0.17, 95% CI: 0.07-0.38, respectively). At least one annual preventive dental care visit reduced the risk of IE among children with CHD. Despite Medicaid dental coverage, a large proportion of children with CHD did not receive annual preventive dental care visits. Future research should focus on developing and evaluating interventions to increase dental care access and improve utilization among this vulnerable population.

Patel, S.  Women with Severe Congenital Heart Disease Have Increased Risk of Offspring Death  (School of Medicine, 2024)

Women with the most severe CHD now routinely survive to reproductive age. In the U.S., pregnancy-related complications and death are more common in Black women and their offspring compared to White women and their offspring, and for those residing in rural areas compared to urban ones. This retrospective cohort study examined offspring outcomes among women with CHD covered by Georgia Medicaid at the time of pregnancy for racial, geographic, and CHD severity group differences and included 4581 women with CHD aged 11-50 with 6522 pregnancies (28.5% with severe CHD, 50.9% Black, 23.2% rural). Multivariate logistic regression models were conducted adjusting for age at first documented pregnancy, social deprivation index (SDI), number of pregnancies, and multiple gestations. Of 6522 pregnancies, 3.0% resulted in offspring death. Offspring death was more common in women with severe CHD compared to those with non-severe CHD (4.1% severe vs. 2.6% non-severe [aOR=1.39, 95%CI 1.02-1.85]. No significant differences were revealed in offspring death between racial (3.3% Black vs. 2.8% White) or geographic residence (2.9% rural vs. 3.1% urban) after controlling for other variables. Compared to fetal and neonatal death rates among U.S. pregnancies in 2019, women with CHD experienced a higher rate of offspring death, and this was more  prevalent for women with severe CHD (4.1%) versus women with non-severe CHD (2.6%).

Wilson, T. The Effects of Preexisting Heart Failure on Pregnancy Complications in Women with Congenital Heart Disease (School of Medicine, 2023)

This study aimed to explore the effects of pre-existing HF in women with CHD on pregnancy outcomes and inform the development of secondary prevention strategies for this population. Using a cross-sectional retrospective cohort study of 829 female patients with CHD, 11-50 years who sought care at either Emory Healthcare or Children’s Healthcare of Atlanta between 2010-2019, the association between women with CHD and non-gestational, pre-existing HF with maternal and fetal/neonatal complications was assessed by multivariate logistic regression. Pre-gestational HF increased the risk of maternal complications (aOR=1.8, 95% CI: 1.09-2.86). Women in their second pregnancy had 5.1 times greater odds (95% CI: 2.94-8.69) and those in their third or higher pregnancy having 5.2 times greater odds (95% CI: 2.52-10.54) of experiencing maternal complications. Those aged 11-19 and 40-50 years were more likely to experience maternal complications compared to those in their 20s. Non-Hispanic Black women had a higher risk of maternal complications than non-Hispanic White women (aOR=2.14, 95% CI: 1.39-3.30). Non-severe CHD reduced the risk for fetal/neonatal complications (aOR=0.56, 95% CI: 0.35-0.90), while increasing the risk for maternal complications (aOR = 2.32, 95% CI: 1.45–3.71) compared to those with severe CHD. Additionally, uninsured/self-payers and those with other insurance coverage had greater odds for fetal/neonatal complications than those with private insurance.

With advances in medical technology, most females born with CHD now live to reach child-bearing age. Safe reproduction and pregnancy in this population have become central concerns as the impact of pregnancy on maternal and infant outcomes is only vaguely understood for those with complex and non-complex defects. This retrospective analysis assessed the relationship of select covariates including CHD severity, age, race, and insurance coverage with maternal and infant complications of pregnancy in 3719 women with CHD who had at least one pregnancy during the 6-year study window, 2008-2013. The majority were 21-30 years of age (42.7%), White (46.0%), non-Hispanic (87.5%) and covered by public insurance (96.0%) with 32.4% residing in rural Georgia counties. Most  experienced obstetric complications (75.8%) and over half of their offspring experienced infant-associated complications (68.4%). While no differences in maternal-associated complications for those with complex CHD were found compared to those with non-complex CHD, offspring of women with complex CHD were more likely to have infant-associated complications compared to those born to women with non-complex CHD, 71.9% vs. 67.4%, (X²=5.85, p=0.0156, respectively). It is critical for pregnant women with CHD to see a specialist who can best manage their complex needs. With the increased incidence of pregnancy-associated complications affecting both mother and infant, standardized preconception counseling should be routine for all women with CHD of child-bearing age.

Chen, J. Risk of Hospitalization and Emergency Department Visits among Congenital Heart Defect Patients with Mental Health Disorders. (RSPH, Department of Epidemiology, 2022)

Concurrent CHD with MHD has been linked to poorer physical health, lower quality of life, and increased hospitalizations. This retrospective cohort study included 13512 patients with CHD, aged 14-55, seen at one or both Atlanta healthcare tertiary referral centers between 1/1/2010-12/31/2019. CHDs were determined by at least 1 ICD-9-CM/ICD-10 CHD-related codes, and MHD was defined by at least 1 ICD-9-CM/ICD-10 codes for mood, anxiety, psychotic, traumatic and personality disorders. At least one MHD was reported for 20.4% of patients, with anxiety disorders the most common, 10.6%. Individuals with CHD and co-occurring MHD had higher rates for: 1) hospitalization (aOR 4.01), with females higher than males (aOR 4.64 vs. aOR 3.22), and 2) ED visits (aOR 2.74), with those from urban areas higher than those from rural areas (aOR 2.85 vs. aOR 1.39). 14.5% had a conotruncal abnormality with a quarter with MHD (X2=37.17, p<0.0001). Individuals with conotruncal defects and MHD also had higher rates for: 1) hospitalizations (aOR 3.92), with females higher than males (aOR 4.99 vs. aOR 3.39), and 2) ED visits (aOR 2.70), with Hispanics higher than non-Hispanic blacks and non-Hispanic whites (aOR 8.39 vs. 2.83 and aOR 2.28, respectively).

Zhang, Y. Association between Influenza and Healthcare Utilization among Children and Adolescents Aged 1-18 years with Congenital Heart Defects (RSPH, Department of Epidemiology, 2021)

To assess the association between CHD anatomic group and 30-day outpatient healthcare utilization among a pediatric and adolescent cohort with CHD and influenza, clinical and administrative electronic healthcare records (eHR) between 2008-2013 were examined for 2,184 children and adolescents aged 1-19 years with CHD and an influenza diagnosis. Poisson regression models revealed that occurrence of any or no outpatient encounters within 30-days of an influenza diagnosis differed across CHD anatomic groups (shunt: 31.6% vs 32.1%; valve: 20.0% vs 26.7%; shunt+valve: 22.0% vs 17.4%; complex: 26.4% vs 23.8%). There was no association between CHD anatomic group and outpatient utilization after adjusting for age, race, ethnicity, hypertension, and heart failure, aside from comparison of the shunt+valve group with the shunt group. Patients with shunt+valve lesions were at a slightly increased risk of having outpatient visits within 30-days of an influenza diagnosis compared to patients with shunt lesions (aRR: 1.09; 95% CI:1.00-1.19).

Alam, Z. Risk of Cerebrovascular Accident among Patients with Congenital Heart Disease (School of Medicine, 2021)

To assess whether anatomically complex CHD increased subsequent risk of CVA, a retrospective cohort study examined 13971 patients, aged 11-64, who were seen at either Emory Healthcare, Children’s Healthcare of Atlanta, or Sibley Heart Center between 2008-2013. Patients were grouped based on CHD anatomy (27% complex, 24% shunt, 42% valve, 7% shunt+valve). Multivariate logistic regression models were used to estimate adjusted relative risk (aRR) and 95% confidence intervals (CIs). Among the 547 CHD patients (3.9%) who had any stroke within study period, it was found that CHD anatomic complexity did not affect the odds of developing a CVA (20.7% complex, 25.4% shunt, 46.6% valve, 7.3% shunt+valve) after controlling for age, insurance, geographic distribution, diabetes, hypertension, hyperlipidemia, endocarditis, HF, and cyanosis.

Kapera, O. Association of Heart Failure and Pulmonary Hypertension among Individuals with Coexisting Congenital Heart Defects and Down Syndrome (RSPH, Department of Epidemiology, 2021)

Individuals with CHD are more likely to have Down Syndrome (DS), yet complications of CHD, such as pulmonary hypertension (P-HTN) and heart failure (HF), has not been well examined with presence or absence of coexisting DS. A retrospective secondary data analysis was conducted with 22499 CHD patients, aged 1-64, identified using ICD-9 codes from encounters in 2008 - 2013 from 11 clinical and administrative data sources. Multivariate logistical regression was used to assess adjusted relative risks (aRR) and 95% confidence intervals (CIs) of P-HTN without HF and HF without P-HTN, among patients with CHD grouped by DS status. The regression analysis showed a 23% lower risk of P-HTN without HF among those with CHD and DS (aRR=0.77; 95% CI: 0.59-1.00) and found a significantly increased risk of HF without P-HTN in patients with CHD and DS (aRR=1.51; 95% CI: 1.04-2.18). It was found that co-occurring DS among individuals with CHD can impact the development of P-HTN and HF overtime.

Graham, N. Association between Diabetes Mellitus and Hospitalization in the Adult Congenital Heart Disease Population. (School of Medicine, 2021).

Concurrent CHD and diabetes mellitus (DM) has a recognized risk, yet medical consequences are not well quantified. For this retrospective cohort study of 4886 CHD patients, age 20-64, with 2008-2013 healthcare encounters from health records, the association between DM and hospitalizations among adults with CHD (ACHD) were assessed. Multivariate logistic regression was used to estimate the association. It was found that 17.1% of ACHD with at least 1 hospitalization had DM compared to 2.5% of their non-hospitalized counterparts (p<0.0001). When controlling for age, gender, race, insurance, CHD anatomic group, obesity hypertension, hyperlipidemia, and neighborhood poverty, odds of hospitalization for those with CHD and DM were 2.78 times greater (95% CI: 2.07-3.74) than those without DM. Along with DM, obesity (adjusted prevalence odds ratio (aPOR) 1.83; 95% CI: 1.46-2.29), hypertension (aPOR 2.64; 95% CI: 2.24-3.11), and hyperlipidemia (aPOR 1.23; 95% CI: 1.02-1.50) were comorbidities also associated with increased hospitalization odds among those with CHD.

Rink, D. Association between Combined Anatomic and Physiologic Classification of Adults with Congenital Heart Defects and Selected Healthcare Utilization and Clinical Outcomes. (RSPH, Department of Epidemiology and School of Medicine, 2021)

This study aims to examine the association between combined anatomic and physiologic classification of CHD complexity with healthcare utilization (i.e., hospitalizations and ED visits) and adverse clinical outcomes (i.e., transplantation and mortality) among adults. Data from Georgia Medicaid claims and Emory electronic health records was examined in 2,384 CHD patients, aged 18-45, from 2008-2013. Anatomic complexity was categorized as complex or shunt and/or valve, and physiological classification was categorized as A/B or C/D. Adjusted relative risks (aRR) and 95% confidence intervals (CI) were estimated using multivariate logistic regression. The risk of hospitalization and ED visits for those with complex CHD and C/D physiology was 31.2 (aRR 31.2, 95% CI: 11.9, 81.6) and 10.6 (aRR 10.6, 95% CI: 3.4, 33.5) times higher, respectively, than those with shunt and/or valve anatomy and A/B physiology over 1-year of follow-up.

Reynolds, E. The Effect of Rurality on Utilization of Psychotherapy for Perinatal Mood Disorders among Georgia Congenital Heart Defect Medicaid Patients (RSPH, Department of Health Policy and Management, 2020)

The number of people born with CHD living into adulthood is increasing, yet the receipt of treatment for perinatal mood disorders (PMD), the number one complication in pregnancy and childbirth, have not been explored in pregnant women living with CHD. This study uses Medicaid claims data to evaluate 5,235 women with CHD who had a delivery spanning the years 1999-2013. A two-part model using logistic regression analysis was used to assess the effect of rurality on the receipt of psychotherapy, a treatment for PMD, in a CHD population. Of pregnant women with CHD who delivered, 931 (17.8%) had a diagnosis of PMD and only approximately 15% of these received psychotherapy. Those living in rural areas were significantly less likely to receive any psychotherapy within 12 weeks of diagnosis of PMD compared to urban counterparts, even after accounting for differences in geographic access.

Gray, K. Hypertension and Heart Failure as Predictors of Mortality in the Adult Congenital Heart Defect (ACHD) Population. (Mercer University College of Health Professions Department of Public Health, 2019)

Since hypertension (HTN) and heart failure (HF), comorbid conditions among the most prevalent causes of death in the United States, commonly accompany CHD, it is crucial to evaluate whether they are reliable predictors of mortality for adults with CHD (ACHD) independent of anatomic CHD complexity. A retrospective cross-sectional analysis of ACHD, aged 18-64, with concomitant HTN and/or HF and at least one health care encounter during 2008-2010 were assessed. Of 7,061 ACHD patients (18.3% HTN without HF, 5.6% HF without HTN, 12.4% with both), 3.5% died (n=244) during the study period. Overall, the sample was 50.6% white, 55.2% female, and 20.3% had a severe CHD. Among those who died, 20.1% had HTN without HF, 17.6% had HF without HTN, and 46.7% had both. Crude analyses revealed that older age and black race were associated with increased mortality during the three-year study period compared to those of younger age and white race. After adjustment for age, race and CHD complexity, ACHD patients with HF with or without HTN were equally likely to die during the study period. However, ACHD patients with shunt and valve defects, also diagnosed with HF without HTN, were more likely to die during the three-year study period compared to patients with complex CHDs.

Lowe, J. Severity of Congenital Heart Disease as a Predictor for Preterm Birth. (RSPH, Department of Epidemiology, 2019)

To assess whether preterm birth risk varies by CHD severity, a retrospective cohort design study used an Emory CHD surveillance repository to examine the association between severity of CHD and preterm birth from 2011-2013. Among initial cohort of 2,523 women aged 12-55, 823 were retained for analysis after excluding those without a birth certificate match, without a pregnancy diagnosis code, or who only had a 745.5 code in isolation. Both crude and adjusted analyses revealed that the preterm birth (23.9%) was not significantly different for women who had a severe CHD (43.4%) compared to those who did not have severe CHD. CHD severity may not be associated with preterm birth risk, however, failure to match a large segment of the initial cohort (60.4%) with their birth outcomes may have biased the results toward the null.

Everitt, I. Predictors of 30-Day Readmission Following Congenital Heart Surgery across the Lifespan. (School of Medicine, 2019).

Hospital readmission is an important driver of costs among patients with congenital heart disease (CHD). We assessed predictors of 30-day rehospitalization between 2011-2012 of 981 patients with CHD following cardiac surgery in CHD patients of any age. A multivariate logistic regression model was used to identify demographic, clinical, and surgical predictors of 30-day readmission. Risk factors for readmission include a history of cardiac surgery, longer length of stay, and comorbid conditions. This information may serve to guide efforts to prevent readmission and inform resource allocation in the transition of care to the outpatient setting. This study also demonstrates the feasibility of linking a national subspecialty registry to a clinical and administrative data repository to follow longitudinal outcomes of interest.

Licitra, G. Predictors associated with 30-day Readmission among a Cohort of Adult Congenital Heart Disease Patients, Medicaid Claims Data 2010-2013. (RSPH, Department of Epidemiology, 2018).

This study aimed to identify common diagnoses and socio-contextual factors at index admission associated with readmissions within 30-days of a cardiac-related hospital admission among ACHD patients. Of the 1,697 patients included in the analysis, about 22% had a 30-day hospital readmission. The remaining 78% had no recorded hospital readmissions within 30 days. Readmission was higher for those at initial admission with comorbid congestive heart failure (CHF) non-hypertensive (13% higher in readmitted patients compared to non-readmitted patients), fluid and electrolyte disease (15% higher), and diabetes mellitus (DM) with complications (~7% higher). Readmission was also higher for black patients compared to white (~6% higher).

Josias Sejour, D. Prevalence of Pregnancy-related Complications among CHD Patients Enrolled in Medicaid during 1998-2013 (RSPH, Department of Behavioral Sciences and Health Education, 2017)

The current study determined whether prevalence of pregnancy-related complications differed by history of Medicaid enrollment among pregnant women with CHD. Using multivariable logistic regression, odds ratios were calculated between Medicaid enrollment history and pregnancy-related complications for female patients who were coded as having at least one CHD diagnosis in the years 1999-2007 with at least one pregnancy-related diagnosis in 2008-2013. Of 1799 females, 557 (31%) were continuously enrolled in Medicaid from 1999-2007, while 1,242 (69.0%) were occasionally enrolled in Medicaid from 1999-2007. 206 (11.5%) had cardiovascular complications, 476 (26.5%) experienced neonatal/fetal loss, 1,426 (79.3%) had maternal complications and 419 (23.3%) experienced complications in pregnancy. Pregnant women > 19 with CHD who were only occasionally enrolled in Medicaid were more likely to have complications during delivery than those who were continuously enrolled in Medicaid.

Gerardin, J. Lost in the system? Transfer to adult congenital heart disease care – challenges and solutions (CHD Fellow, Emory School of Medicine, 2015-2017)

The objective was to determine what percentage of patients aged 16-30 with congenital heart disease transferred to adult congenital cardiac care between 2008-2010. Among 1514 initial patients (39% severe complexity), 24% were beyond the recommended transfer age of 21 years. Overall, only 12.1% transferred care to the referral affiliated adult hospital. 90% of these adults that successfully transferred were seen by an adult congenital cardiologist, with an average of 33.9 months between last pediatric visit and first adult visit. Distance to referral center contributed to delayed transfer to adult care. Those with severe congenital heart disease were more likely to transfer (18.7% vs 6.2% for not severe).

Claxton, J. Estimate of Care Non-Continuity among Medicaid Beneficiaries Diagnosed with Congenital Heart Defects in Five Metropolitan Georgia Counties: 1999-2010 (RSPH, Department of Epidemiology, 2016)

The aim of this study was to estimate the percentage of Georgia Medicaid beneficiaries diagnosed with a CHD sometime during 1999-2007 who also had a Medicaid-paid claim during 2008-2010. Using multivariable logistic regression, odds ratios were calculated between age and having a Medicaid-paid claim during 2008-2010 and, among those with a claim, between age and having a CHD-related diagnosis on the claim. 5,944 patients had a CHD-related diagnosis on their Medicaid claim during 1999-2007; only 46% also had a Medicaid-paid claim during 2008-2010. Females were most likely to have a Medicaid claim with a CHD diagnosis.

John, K. Congenital Heart Defect Prevalence and Capture-Recapture Methodology (RSPH, Department of Epidemiology, 2016)

Using data from a repository of administrative and billing data collected on congenital heart disease individuals, capture-recapture (CR) methodology and logistic regression were employed to estimate the prevalence of CHD for both adolescents, aged 11-20, and adults aged 21-64, in five metropolitan Atlanta, Georgia counties. Altogether 1,858 adolescent cases were captured from at least one "adolescent" database, and 3,183 adult cases were captured from at least one "adult" database. The estimated number of adolescents (aged 11-20 years) with CHD and living in the 5 metropolitan Atlanta counties in Georgia was 3,718 (95%CI: 3,471 - 4,004) for a prevalence estimate of 7.85 per 1,000 population aged 10-19 in 2010. The number of adults with CHD aged 21-64 years was estimated to be 12,969 (95%CI: 13,873 -18,915) for a prevalence estimate of 6.08 per 1,000 population aged 20-64 in 2010.

Deshmukh, A. In or Out of Care: Congenital Heart Defects and the Impact on Health Outcomes (RSPH, Department of Epidemiology, 2015)

Study goals were to determine 1) how many Georgia adolescents with a Congenital Heart Defect (CHD) received continuous care from 2008-2010; 2) how many of them successfully transitioned into adult congenital care; and 3) a predictive model of risk factors for loss to follow-up and successful transition. Administrative healthcare data was used to identify adolescent patients (16-21 years old) and multivariable logistic regression was used to determine odds ratios. After controlling for age, sex, insurance, proximity, CHD severity, number of procedures, and comorbidities, more than half (53.6%) of the adolescents were lost to follow-up and only about 20% successfully transitioned into adult congenital care. Being older and female predicted loss to follow-up, while severity, procedure history and having a comorbidity were protective. Being older, and having public insurance, a severe CHD, a non-CHD birth defect, and a respiratory/pulmonary comorbidity predicted successful transitioning.

Oandasan, P. Evaluating the Cardiovascular Risk Factors of Adult Congenital Heart Defects (RSPH, Career MPH, Epidemiology, 2015)

Diabetes Mellitus (DM) patients and Adult Congenital Heart Defect (ACHD) patients are high utilizers of hospital resources, specifically through hospitalizations, however, there has been no research assessing the risk of increased hospitalization among diabetic ACHD patients. Using a cross-sectional study design with 2008-2010 ACHD administrative data, a logistic regression analysis was employed to calculate odds ratios to assess the association between DM and hospitalizations in an ACHD cohort. After controlling for CHD severity, hypertension, and hyperlipidemia, the adjusted odds of being hospitalized among diabetic ACHD patients were four times greater compared to non-DM ACHD patients (aOR=4.2, 95%CI: 2.9, 6.2).

> CHD Education Module

We have partnered with The Georgia Department of Public Health, The Centers for Disease Control and Prevention, community organizations, healthcare systems, patient advocacy groups, patients and parents across Georgia.

Funding/Support: Funding/Support received by Centers for Disease Control and Prevention, Grant/Award Number: CDC‐RFA‐DD12-1207

Surveillance period
2008 - 2010

Collaborators (CDC and 3 sites: GA, MA & NY)
Centers for Disease Control and Prevention (CDC): National Center on Birth Defects and Developmental Disabilities (NCBDDD), Atlanta, GA

1. Emory University School of Medicine and Rollins School of Public Health, Atlanta, GA collaborated with:
   • Metropolitan Atlanta Congenital Defects Program (MACDP) - pediatric
   • Georgia Department of Public Health (GADPH) - pediatric and adult
   • Sibley Heart Center - pediatric
   • Pediatric Cardiology Services (now part of Sibley Heart Center) - pediatric
   • Children’s Healthcare of Atlanta (CHOA) - pediatric
   • Emory Healthcare (EHC) - adult
   • St. Joseph’s Hospital (now part of Emory Healthcare) - adult
   • Grady Health - adult
   • Centers for Medicare and Medicaid Services (CMS) (via Research Data Assistance Center (ResDAC) - pediatric and adult
2. Massachusetts Department of Health, Boston, MA
3. New York State Department of Health, Albany, NY

Priorities

• To determine feasibility of population-based CHD surveillance across multiple sites
• To estimate prevalence of CHD among adolescents and adults
• To examine survival, healthcare utilization, longer term outcomes among adolescents and adults with CHD

What we Learned

• Many U.S. children, teens, and adults live with and receive care for CHD
• 20-33% of adolescents and adults with CHD have been diagnosed with a mental health condition
• Many women with CHD become pregnant and experience pregnancy-related health complications
• Severe CHD ranged from 11% (NY) to 20% (GA)
• Medicaid Coverage: > 75% (MA), 31% (GA), 28% (NY)
• Capture of comorbid conditions varied across sites
• Older patients and those with non-complex CHD contribute to health burden
• Cardiac complications and comorbid conditions are common

Estimates of Adolescent and Adult Congenital Heart Defect Prevalence in Metropolitan Atlanta, 2010, Using Capture-Recapture Applied to Administrative Records (Raskind-Hood et al. Annuals of Epidemiology. 2019 Apr;32:72-77.e2)

The 745.5 Issue in Code-based, Adult Congenital Heart Disease Population Studies: Relevance to Current and Future ICD-9-CM and ICD-10-CM Studies (Rodriguez et al. Congenital Heart Disease. 2018 January; 13(1): 59-64).

Assessing Pregnancy, Gestational Complications, and Comorbidities in Women with Congenital Heart Defects (Data from ICD-9-CM Codes in Three U.S. Surveillance Sites). (Raskind-Hood et al. American Journal of Cardiology. 2020. Marv1; 125(5):812-819.

Funding/Support: Funding/Support received by Centers for Disease Control and Prevention, Grant/Award Number: CDC‐RFA‐DD15-1506

Surveillance period
2011 - 2013

Collaborators (CDC and 5 sites: CO, GA, NC, NY & UT)
Centers for Disease Control and Prevention (CDC): National Center on Birth Defects and Developmental Disabilities (NCBDDD), Atlanta, GA

1. Emory University School of Medicine and Rollins School of Public Health, Atlanta, GA collaborated with:
   1. Data Sources:
      • Metropolitan Atlanta Congenital Defects Program (MACDP) - pediatric
      • Georgia Department of Public Health (GADPH) – pediatric and adult
      • Children’s Healthcare of Atlanta (CHOA) - pediatric
      • Sibley Heart Center (now subsumed under CHOA) - pediatric
      • CHOA Society of Thoracic Surgeons (STS) – pediatric
      • Emory Healthcare (EHC) – adult
      • EHC Society of Thoracic Surgeons – adult
      • Grady Health System - adult
      • Piedmont Healthcare – pediatric and adult
      • Wellstar Health System - adult
      • Centers for Medicare and Medicaid Services (CMS) (via Research Data Assistance Center (ResDAC) - pediatric and adult
   2. Community Advisory Committee:
      • St. Joseph’s Hospital (now part of Emory Healthcare)
      • Georgia Department of Public Health (GA DPH)
      • Sibley Heart Center
      • The Children's Heart Foundation
      • Georgia Center for Learning Disabilities – GA State University
      • Adult Congenital Hearts of Greater Atlanta (patient advocacy)
      • Grady Healthcare
      • Wellstar Healthcare
      • Piedmont Healthcare
      • Children’s Healthcare of Atlanta
      • The Urban Health Initiative
      • CHD Healthcare providers
      • Parents of teens with CHD
2. Duke University School of Medicine, Duke Clinical Research Institute, Durham, NC
3. New York State Department of Health, Albany, NY
4. University of Colorado | Anschutz Medical Campus, Departments of Internal Medicine and Pediatrics, Divisions of Cardiology, Aurora, CO
5. University of Utah, Salt Lake City, UT

Priorities

• Prevalence of CHD across the lifespan, healthcare utilization among adolescents and adults, comorbidities, heart failure
• Assessment of barriers to appropriate transition of adolescents to adult cardiac care (NY & GA)
• Education Modules (GA)

What we Learned so Far

• 24% complex anatomy, 37% shunt, 53% valve
• 69% children
• 19% black or multi-racial, 15% Hispanic
• 48% have public insurance, more common for children
• Overall retention in care was low when transitioning from pediatric to adult care
• Severe CHD were more likely to transfer to ACHD care than those with shunt and/or valve lesion
• Transfer to adult care typically was to an ACHD care setting
• Barriers to care transition included concern with replacing a strong relationship with pediatric providers, concern with obtaining insurance coverage into adulthood, and inability to locate an appropriate adult provider.

Paper Topics/Projects in Progress

• Racial and socioeconomic differences in healthcare outcomes
• Comorbidities
• ICD-9-CM CHD code validation
• Pregnancy outcomes
• Healthcare utilization

Individuals aged 1-64 years with documented congenital heart defects at healthcare encounters, five U.S. surveillance sites, 2011-2013 (Glidewell et al. American Heart Journal, 2021. May 2;S0002-8703(21):00109-5.)

Percentage of CHD cases decreased with age until 20 to 44 years, and for non-severe CHD, the percentage increased slightly for those 45 to 64 years. These findings will inform planning for the needs of this growing population.

Health Care Transition Perceptions among Parents of Adolescents with Congenital Heart Defects in Georgia and New York (Gaydos et al. Pediatric Cardiology. 2020.41:1220–1230).

These findings suggest that there are significant concerns about the realities of the transition process. Future investment in adolescent transition should include educational interventions and training of maternal and child healthcare professionals who can assist with allaying fears about transition and ensuring that barriers to care remain low.

Hypertension and Heart Failure as Predictors of Mortality in an Adult Congenital Heart Defect Population. (Raskind-Hood et al. 2021. Congenital Heart Disease. 16(4):333–355).

To evaluate the impact of hypertension (HTN) and heart failure (HF) on mortality in adults with CHD, a retrospective cross-sectional analysis of 5,397 adults with CHD (ACHD), aged 18-64, was conducted. 18.3% of the sample had HTN without HF, 5.6% had HF without HTN, 12.4% had both. While overall mortality was 3.5%, older age and black race were associated with increased mortality during the three-year study period compared to those of younger age and white race. Also, valve lesion patients with HF, with or without HTN, were at increased risk of death. Findings suggest that vigilance for signs and symptoms of HF in adults with all heart defects, and treatment to mitigate comorbid HF and HTN may lessen mortality in ACHD regardless of the anatomic complexity of the heart defect, emphasizing the need for all ACHD to stay in specialty care.

Funding/Support: Funding/Support received by Centers for Disease Control and Prevention, Grant/Award Number: CDC‐RFA‐DD19-1902A and 1902B

Component A (in progress)

Surveillance period
2010 - 2019

Collaborators (CDC and 7 sites: AZ, GA, IA, NC, NY, SC & UT)
Centers for Disease Control and Prevention (CDC): National Center on Birth Defects and Developmental Disabilities (NCBDDD), Atlanta, GA

1. Emory University School of Medicine and Rollins School of Public Health, Atlanta, GA collaborates with:
   1. Data Sources:
      • Children’s Healthcare of Atlanta (CHOA) – pediatric [case finding & encounters]
      • CHOA Society of Thoracic Surgeons (STS) – pediatric [gold standard case finding source]
      • Emory Healthcare (EHC) – adult
      • EHC Society of Thoracic Surgeons – adult [gold standard case finding source]
      • Emory ‘Archival’ CHD databases – adult
      • Centers for Medicare and Medicaid Services (CMS) (via Research Data Assistance Center (ResDAC) and/or GA Dept Community Health (GADCH) - pediatric and adult
      • Metropolitan Atlanta Congenital Defects Program (MACDP) - pediatric
      • Piedmont Healthcare -pediatric and adult
      • Wellstar Health System – adult
      • Northside Hospital - adult
      • GA Dept of Public Health (GADPH)
        • Vital records (birth and death certificates) – pediatric and adult
        • Critical Congenital Heart Disease (CCHD) Newborn Screening - pediatric
   2. Community Advisory Committee:
      • GA Dept of Public Health (GADPH)
      • GA Dept of Community Health (GADCH)
      • Children’s Healthcare of Atlanta (CHOA) & Sibley Heart Center
      • The Children's Heart Foundation
      • Georgia Center for Learning Disabilities – GA State University
      • Adult Congenital Hearts of Greater Atlanta (patient advocacy)
      • Grady Health System
      • Wellstar Health System
      • Piedmont Healthcare
      • CHD Healthcare providers
      • Parents of teens with CHD
2. University of Arizona, Department of Pediatrics, College of Medicine, Tucson, AZ
3. Duke University School of Medicine, Duke Clinical Research Institute, Durham, NC
4. University of Iowa, College of Public Health, Department of Epidemiology, Iowa City, IA
5. New York State Department of Health, Albany, NY
6. South Carolina Department of Health and Environmental Control, Columbia, SC
7. University of Utah, Salt Lake City, UT

Priorities

• Assess and conduct a population-based surveillance of individuals with CHD, ages 1-45 years
• Examine descriptive epidemiology of CHD, survival, healthcare utilization, and comorbidities over time
• Improve understanding of health outcomes of CHD among public health practitioners
• Estimate age-specific mortality

Improved treatment of congenital heart defects (CHD) in the recent decades has increased survival; however, little data exist on healthcare utilization, comorbidities, long-term health and non-health outcomes and mortality to inform quality of life and develop effective secondary prevention strategies to address health complications among those affected. Through expansion of an integrated surveillance system for CHD, using individual encounter level data for 2010-2019, we aim to:

1. Improve understanding of age-specific mortality, healthcare utilization, comorbidities, survival and other outcomes over time;
2. Improve understanding of racial/ethnic and socioeconomic patterns in healthcare usage, and their impact on long-term outcomes over time;
3. Gain a greater understanding of the strengths and limitations of databases used for CHD surveillance; and
4. Increase CHD awareness among the public and stakeholders

Our source population is the state of Georgia based on 2010 Census [9.7 million (30.5% black, 8.8% Hispanic)], and the 2017 American Community Survey (ACS) estimate [10.2 million (31.3% black, 9.3% Hispanic)].

• a case born between 1/1/1965 - 12/31/2019 with at least one of the included CHD codes found in an approved data source or cardiac encounter type, at any time, and with at least one encounter between 2010-2019, with or without an associated CHD code, and with residence in the state of Georgia at some point in the decade 2010-2019.

Regional and community partners enhance our surveillance system, guide education efforts, and ensure data quality and consistency. Through our previous work, we have already demonstrated success in conducting CHD surveillance in the proposed region, and have developed processes to assess data quality and performance measures applicable to the proposed project. Through this project, we will gain a greater understanding of the strengths and limitations of integrated CHD surveillance, increase awareness among stakeholders, and improve ability to estimate life expectancies. Achieving these aims should assist affected individuals and families in their expectations and healthcare decisions, improve decision-making among stakeholders, and ultimately lead to more effective secondary prevention strategies to reduce the public health impact of CHDs.

Component B (in progress)

Surveillance period
2010 - 2019

Collaborators (CDC and Emory)

1. Emory University School of Medicine and Rollins School of Public Health, Atlanta, GA collaborates with:
2. Emory University School of Medicine, Dept of Biomedical Informatics, Atlanta, GA whose team includes: Gari Clifford, Rishi Kamaleswaran, Chad Robichaux, Abeed Sarker, Reza Sameni, Qiao Li and Mary Bell (GA Tech student)

Priorities

• To validate billing codes for CHDs in healthcare claims data
• Determine best ways to use healthcare claims for CHD surveillance across the lifespan

Building on existing CHD surveillance infrastructure and using individual encounter level integrated electronic health record (eHR) and administrative data for 2010-2019, and through validation of healthcare claims data in an integrated surveillance system for CHDs, this project aims to:

1. Improve understanding of the validity and utility of healthcare claims data for surveillance of CHDs;
2. Improve the accuracy of identification and surveillance of CHDs; and
3. increase awareness among the public and stakeholders.

Achieving these aims should improve decision-making among stakeholders, and ultimately lead to more effective secondary prevention strategies to reduce the public health impact of CHDs. Improved accuracy in CHD surveillance will lead to improved understanding of healthcare outcomes over time for patients with CHD; racial/ethnic and socioeconomic patterns in healthcare usage, and their impact on outcomes over time; and age-specific mortality.

A minimum of 1500 cases identified by CHD ICD codes have already been validated via medical record review. A machine learning (ML) approach will be utilized to determine PPV of CHD ICD codes by CHD anatomic group, age, CHD type, and individual and healthcare characteristics. From these results, further development of algorithms to maximize PPV and sensitivity will be proposed. ML techniques developed together with colleagues in the Emory Biomedical Informatics Dept. will be tested to optimize algorithms for accurate detection of CHDs in healthcare claims data. In addition, algorithms will be tested in additional data sources such as GA Medicaid and Kaiser Permanente. In addition, we are exploring the role of natural language processing (NLP) for identifying congenital heart conditions that cannot be accurately characterized by claims data. Through this project, we will gain a greater understanding of the strengths and limitations of integrated CHD surveillance, increase awareness among stakeholders, and improve ability to calculate life expectancies. Regional and community partners enhance our surveillance system, guide education efforts, and ensure data quality and consistency.