Matthew E. Oster’s research while affiliated with Children's Healthcare of Atlanta and other places

BACKGROUND Disparities between metro and nonmetro areas exist in health outcomes. The effect of residing areas on mortality for patients with congenital heart disease remains unclear. We evaluated the relationship of residing areas with survival outcomes after congenital heart surgery (CHS). METHODS This is a retrospective cohort study of patients enrolled in the Pediatric Cardiac Care Consortium who had a history of CHS. Outcomes were tracked by the National Death Index through 2022. Logistic regression and Cox proportional hazards models were fitted to examine the associations between residence at CHS with in-hospital mortality and long-term survival after adjustment for covariates. RESULTS Among 28 504 eligible patients (47.0% female) with a history of CHS, 19 772 (69.4%) patients resided in metro areas. Patients with congenital heart disease living in nonmetro areas at CHS had a lower (86.5%) 30-year survival rate following discharge from initial CHS versus patients living in metro areas (88.4%). After adjustment for sex, birth era, congenital heart disease severity, and presence of chromosomal abnormality, residing in nonmetro areas was associated with an increased risk of long-term mortality (adjusted hazard ratio, 1.12 [95% CI, 1.03–1.21]). Further adjustment for the neighborhood socioeconomic status attenuated the observed reduction in risk of death between nonmetro and metro areas. Patients with mild congenital heart disease who resided in nonmetro and not adjacent to metro areas were independently associated with an increased risk of long-term death (adjusted hazard ratio, 1.34 [95% CI, 1.00–1.77]), after adjustment for covariates and neighborhood socioeconomic status. CONCLUSIONS Residence in nonmetro areas at CHS is associated with an increased risk of death both in the immediate postoperative period in-hospital and on the long-term up to 30 years after CHS discharge, but this association is explained by differential neighborhood socioeconomic status at the time of CHS. These findings provide opportunities for targeted interventions to reduce disparities and improve outcomes for all patients after CHS.

Background Sudden cardiac death is a significant concern among patients with congenital heart disease (CHD). We assessed the risk of remote sudden cardiac death after congenital heart surgery. Methods Patients undergoing congenital heart surgery before 21 years of age between 1982 and 2003 in the Pediatric Cardiac Care Consortium registry were linked to National Death Index data through 2019. Sudden cardiac death was defined as death associated with a cardiac arrest or ventricular fibrillation diagnosis code. Standardised mortality ratios relative to the general population were calculated using Centers for Disease Control and Prevention data. Results Among 30,566 patients discharged after their initial surgery, 2,718 deaths occurred over a median period of 23 years (IQR 19–27). Of 463 (17%) sudden cardiac deaths, the median age was 1.7 years (IQR 0.5–16.5). The mean incidence was 7 per 10,000 person-years (95% CI: 0.64–0.77), ranging from 2.7 for left-to-right shunt lesions to 37 for single-ventricle physiology. Cardiac comorbidities including heart failure (13.6%) and arrhythmias (7.1%) were more frequent among sudden cardiac death patients. Standard mortality ratios for sudden cardiac death were elevated across all CHD types, ranging from 8.0 (95% CI: 6.3–9.6) for left-to-right shunts to 107.7 (95% CI: 88.9–126.5) for single-ventricle physiology. Conclusion Sudden cardiac death risk is higher post-congenital heart surgery compared to the general population. Even patients with mild CHD are at risk, highlighting the need for long-term follow-up for all patients. Heart failure and arrhythmia prevalence suggest potential therapeutic targets to reduce sudden cardiac death risk.

Background Our objective was to characterize the education and employment history of young adults with congenital heart defects (CHD) living in the United States. Methods The 2016–2019 Congenital Heart Survey To Recognize Outcomes, Needs, and well‐beinG collected data from young adults (ages 19–38) with CHD identified from active birth defect in Arkansas, Arizona, and Atlanta, Georgia. Educational attainment, employment history, and special education between kindergarten and 12th grade were self‐/proxy‐reported. Respondent percentages were standardized to the eligible population by CHD severity, birth year, site, sex, and maternal race/ethnicity and compared by CHD severity using p values from Z ‐scores. Log‐binomial prevalence ratios (aPRs) assessed associations between respondent characteristics and outcomes, adjusting for CHD severity, age group, sex, race/ethnicity, and site. Employment models also adjusted for education. Point estimates were compared to the 2018 American Community Survey (ACS) 5‐year general population estimates. Results Among 1438 respondents, 28.3% attained ≥ bachelor's degree and 22.1% were unemployed for ≥ 12 months. Estimates were comparable by CHD severity (aPRs ~1.0) and similar to general population estimates (in ACS, 21% attained ≥ bachelor's degree and 26% were unemployed). About 25.3% of adults with CHD received special education, more commonly adults with severe (32.9%) than nonsevere CHD (23.5%, p = 0.01). Conclusions Among young adults with CHD, educational attainment and employment did not substantially differ by CHD severity or from general population rates. One in four used special education between kindergarten and 12th grade. Clinical guidelines recommend ongoing educational and vocational support to individuals with CHD as needed so this population continues to thrive.

Background Almost half of individuals born with Down syndrome (DS) have congenital heart defects (CHDs). Yet, little is known about the health and healthcare needs of adults with CHDs and DS. Therefore, we examined comorbidities and healthcare utilization of this population. Methods Data were from the 2016–2019 Congenital Heart Survey to Recognize Outcomes, Needs, and well‐beinG (CH STRONG), a survey of 19–38‐year‐olds with CHDs identified through birth defects registries in Arkansas, Arizona, and Atlanta. Outcome estimates were standardized to the CH STRONG eligible population. Multivariable Poisson regression generated adjusted prevalence ratios (aPRs) for associations between DS and each outcome, adjusting for covariates, including CHD severity. Results Among 1500 respondents, 9.1% had DS. Compared to those without DS, respondents with DS were more commonly male (55.5% vs. 45.0%), < 25 years old (51.8% vs. 42.7%), non‐Hispanic White (72.3% vs. 69.3%), and publicly insured (77.4% vs. 22.8%; all p < 0.05). Of adults with CHDs and DS, 5.5% had cardiac comorbidities, 19.3% had emergency room (ER) visits, 6.2% had hospital admissions, and 1.2% had cost‐related delays in care in the last year; 0.1 to 0.6 times lower than adults with CHDs without DS. Additionally, 26.7% had non‐cardiac comorbidities (aPR = 1.25 [0.92–1.72]), most commonly sleep apnea (13.7% vs. 3.2%, aPR = 3.67 [2.02–6.67]). Receipt of cardiology care in the last 2 years was similarly low (52.7% vs. 44.7%). Conclusions Adults with CHDs and DS comprise a substantial percentage of adults with CHDs and have unique health and healthcare needs. Half of adults with CHDs and DS are not receiving recommended routine cardiac care.

The initial and updated Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STAT and STAT 2020) and Risk Adjusted Classification for Congenital Heart Surgery-1 and Risk Adjusted Classification for Congenital Heart Surgery-2 scoring systems are validated to predict early postoperative mortality following congenital heart surgery in children; however, their ability to predict long-term mortality has not been examined. We performed a retrospective cohort study using data from the Pediatric Cardiac Care Consortium, a US-based registry of cardiac interventions in 47 participating centres between 1982 and 2011. Patients included in this cohort analysis had select congenital heart surgery representing the spectrum of severity as determined by STAT and Risk Adjusted Classification for Congenital Heart Surgery-1 and were less than 21 years of age. We applied STAT, STAT 2020, Risk Adjusted Classification for Congenital Heart Surgery-1, and Risk Adjusted Classification for Congenital Heart Surgery-2 for prediction of early mortality and long-term postoperative survival probability by surgical risk category. Long-term outcomes were obtained by matching Pediatric Cardiac Care Consortium patients with deaths reported in the National Death Index through 2021. Of 20,753 eligible patients, 18,755 survived the postoperative period and 2,058 deaths occurred over a median follow up of 24.4 years (Interquartile Range: 21–28.4). Each scoring system performed well for predicting early postoperative mortality with the following c-statistics: STAT: 0.7872, Risk Adjusted Classification for Congenital Heart Surgery-1: 0.7872, STAT 2020: 0.7724 and Risk Adjusted Classification for Congenital Heart Surgery-2: 0.7668. The predictive ability for long-term risk of death was as follows: STAT: 0.6995, Risk Adjusted Classification for Congenital Heart Surgery-1 c = 0.6741, Risk Adjusted Classification for Congenital Heart Surgery-2: 0.7156 and STAT 2020: c = 0.7156. Risk-adjusted score systems for congenital heart surgery maintain adequate but diminishing discriminative power to predict long-term mortality. Future efforts are warranted to develop a tool with improved long-term survival prediction.