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Despite the overall improvement in life expectancy of patients living with congenital heart disease (congenital HD), disparities in morbidity and mortality remain throughout the lifespan. Longstanding systemic inequities, disparities in the social determinants of health, and the inability to obtain quality lifelong care contribute to poorer outcomes. To work toward health equity in populations with congenital HD, we must recognize the existence and strategize the elimination of inequities in overall congenital HD morbidity and mortality, disparate health care access, and overall quality of health services in the context of varying social determinants of health, systemic inequities, and structural racism. This requires critically examining multilevel contributions that continue to facilitate health inequities in the natural history and consequences of congenital HD. In this scientific statement, we focus on population, systemic, institutional, and individual‐level contributions to health inequities from prenatal to adult congenital HD care. We review opportunities and strategies for improvement in lifelong congenital HD care based on current public health and scientific evidence, surgical data, experiences from other patient populations, and recognition of implicit bias and microaggressions. Furthermore, we review directions and goals for both quantitative and qualitative research approaches to understanding and mitigating health inequities in congenital HD care. Finally, we assess ways to improve the diversity of the congenital HD workforce as well as ethical guidance on addressing social determinants of health in the context of clinical care and research.

Background Prenatal diagnosis of congenital heart disease has been associated with early‐term delivery and cesarean delivery (CD). We implemented a multi‐institutional standardized clinical assessment and management plan (SCAMP) through the University of California Fetal‐Maternal Consortium. Our objective was to decrease early‐term (37–39 weeks) delivery and CD in pregnancies complicated by fetal congenital heart disease using a SCAMP methodology to improve practice in a high‐risk and clinically complex setting. Methods and Results University of California Fetal‐Maternal Consortium site‐specific management decisions were queried following SCAMP implementation. This contemporary intervention group was compared with a University of California Fetal‐Maternal Consortium historical cohort. Primary outcomes were early‐term delivery and CD. A total of 496 maternal–fetal dyads with prenatally diagnosed congenital heart disease were identified, 185 and 311 in the historical and intervention cohorts, respectively. Recommendation for later delivery resulted in a later gestational age at delivery (38.9 versus 38.1 weeks, P=0.01). After adjusting for maternal age and site, historical controls were more likely to have a CD (odds ratio [OR],1.8; 95% CI, 2.1–2.8; P=0.004) and more likely (OR, 2.1; 95% CI, 1.4–3.3) to have an early‐term delivery than the intervention group. Vaginal delivery was recommended in 77% of the cohort, resulting in 61% vaginal deliveries versus 50% in the control cohort (P=0.03). Among pregnancies with major cardiac lesions (n=373), vaginal birth increased from 51% to 64% (P=0.008) and deliveries ≥39 weeks increased from 33% to 48% (P=0.004). Conclusions Implementation of a SCAMP decreased the rate of early‐term deliveries and CD for prenatal congenital heart disease. Development of clinical pathways may help standardize care, decrease maternal risk secondary to CD, improve neonatal outcomes, and reduce healthcare costs.

Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10‐year period were collected. Primary outcome measures included fetal demise and overall mortality. Of 100 fetuses, pregnancy termination/postnatal nonintervention was elected in 22. Of 78 with intention to treat, 7 (9%) died in utero and 21 (27%) died postnatally. With median follow‐up of 32.9 months, no deaths occurred after 13 months. Of 80 fetuses with genetic testing, 46% had chromosomal abnormalities, with 22q11.2 deletion in 35%. On last fetal echocardiogram, at a median of 34.6 weeks, left ventricular dysfunction independently predicted fetal demise (odds ratio [OR], 7.4; 95% CI 1.3, 43.0; P=0.026). Right ventricular dysfunction independently predicted overall mortality in multivariate analysis (OR, 7.9; 95% CI 2.1–30.0; P=0.002). Earlier gestational age at delivery, mediastinal shift, left ventricular/right ventricular dilation, left ventricular dysfunction, tricuspid regurgitation, and Doppler abnormalities were associated with fetal and postnatal mortality, although few tended to progress throughout gestation on serial evaluation. Pulmonary artery diameters did not correlate with outcomes. Conclusions Perinatal mortality in tetralogy of Fallot with absent pulmonary valve remains high, with overall survival of 64% in fetuses with intention to treat. Right ventricular dysfunction independently predicts overall mortality. Left ventricular dysfunction predicts fetal mortality and may influence prenatal management and delivery planning. Mediastinal shift may reflect secondary effects of airway obstruction and abnormal lung development and is associated with increased mortality.

Background Impairments in fetal oxygen delivery have been implicated in brain dysmaturation seen in congenital heart disease (CHD), suggesting a role for in utero transplacental oxygen therapy. We applied a novel imaging tool to quantify fetal cerebral oxygenation by measuring T2* decay. We compared T2* in fetuses with CHD with controls with a focus on cardiovascular physiologies (transposition or left‐sided obstruction) and described the effect of brief administration of maternal hyperoxia on T2* decay. Methods and Results This is a prospective study performed on pregnant mothers with a prenatal diagnosis of CHD compared with controls in the third trimester. Participants underwent a fetal brain magnetic resonance imaging scan including a T2* sequence before and after maternal hyperoxia. Comparisons were made between control and CHD fetuses including subgroup analyses by cardiac physiology. Forty‐four mothers (CHD=24, control=20) participated. Fetuses with CHD had lower total brain volume (238.2 mm3, 95% CI, 224.6–251.9) compared with controls (262.4 mm3, 95% CI, 245.0–279.8, P=0.04). T2* decay time was faster in CHD compared with controls (beta=−14.4, 95% CI, −23.3 to −5.6, P=0.002). The magnitude of change in T2* with maternal hyperoxia was higher in fetuses with transposition compared with controls (increase of 8.4 ms, 95% CI, 0.5–14.3, P=0.01), though between‐subject variability was noted. Conclusions Cerebral tissue oxygenation is lower in fetuses with complex CHD. There was variability in the response to maternal hyperoxia by CHD subgroup that can be tested in future larger studies. Cardiovascular physiology is critical when designing neuroprotective clinical trials in the fetus with CHD.

BACKGROUND The development of congenital heart disease (CHD) is multifactorial with genetic and environmental influences. We sought to determine the relationship between socioeconomic and environmental factors with the incidence of CHD among live‐born infants in California and to determine whether maternal comorbidities are in the causal pathway. METHODS AND RESULTS This was a population‐based cohort study in California (2007–2012). The primary outcome was having significant CHD. Predictors included socioeconomic status and environmental exposure to pollutants determined by U.S. Census data. A social deprivation index and environmental exposure index was assigned based on neighborhood socioeconomic variables, categorized into 4 quartiles. Quartile 1 was the best with the least exposure to pollutants and social deprivation, and quartile 4 was the worst. Multivariate logistic regression and mediation analyses were performed. Among 2 419 651 live‐born infants, the incidence of CHD was 3.2 per 1000 live births. The incidence of CHD was significantly higher among those in quartile 4 compared with quartile 1 (social deprivation index: 0.35% versus 0.29%; odds ratio [OR], 1.31; 95% CI, 1.21–1.41; environmental exposure index: 0.35% versus 0.29%; OR, 1.23; 95% CI, 1.15–1.31) after adjusting for maternal race/ethnicity and age and accounting for the relationship between the 2 primary predictors. Maternal comorbidities explained 13% (95% CI, 10%–20%) of the relationship between social deprivation index and environmental exposure index with the incidence of CHD. CONCLUSIONS Increased social deprivation and exposure to environmental pollutants are associated with the incidence of live‐born CHD in California. Maternal comorbidities explain some, but not all, of this relationship. These findings identify targets for social policy initiatives to minimize health disparities.

Background Infants with critical congenital heart disease (CCHD) are more likely to be small for gestational age (SGA) or born to mothers with maternal placental syndrome. The objective of this study was to investigate the relationship between maternal placental syndrome, SGA, and gestational age (GA) on 1‐year mortality in infants with CCHD. Methods and Results In a population‐based administrative database of all live‐born infants in California (2007–2012) we identified all infants with CCHD without chromosomal anomalies. Our primary predictor was an impaired fetal environment (IFE), defined as presence of maternal placental syndrome or SGA. We calculated hazard ratios to quantify the association between different components of IFE and 1‐year mortality and conducted a causal mediation analysis to assess GA at birth as a mediator. We identified 6863 infants with CCHD. IFE was present in 25.1%. Infants with IFE were more likely to die than infants without IFE (16.6% versus 11.1%; hazard ratios 1.55, 95% CI 1.34–1.78). Only SGA (hazard ratios 1.76, 95% CI 1.50–2.05) and placental abruption (hazard ratios 1.70, 95% CI 1.17–2.48) were significantly associated with mortality; preeclampsia and gestational hypertension had no significant association with mortality. The mediation analysis showed that 32.8% (95% CI 24.9–47.0%) of the relationship between IFE and mortality is mediated through GA. Conclusions IFE is a significant contributor to outcomes in the CCHD population. SGA and placental abruption are the main drivers of postnatal mortality while other maternal placental syndrome components had much less of an impact. Only one third of the effect between IFE and mortality is mediated through GA.

Background Tube‐assisted feeding in infancy is common in patients with single‐ventricle physiology (SVP). Postnatal brain development is delayed, and injury is common, in patients with SVP. The role of brain findings in feeding outcomes remains unclear. We sought to determine the association between neonatal perioperative brain injury and postnatal brain maturation with feeding‐tube dependency in patients with SVP at neonatal discharge and just before the stage‐2 palliation. Methods and Results We evaluated a cohort of 48 term neonates with SVP who underwent pre‐ and postoperative brain magnetic resonance imaging. Perioperative brain injury and microstructural brain development were measured with diffusion tensor imaging including fractional anisotropy in white matter and apparent diffusion coefficient in gray matter. The primary outcome was defined as being 100% orally fed (binary). Of the patients 79% (38/48) were tube fed at hospital discharge, and 27% (12/45) were tube fed before stage‐2 palliation. Perioperative brain injury did not differ by group. Orally fed patients had a faster rate of decrease in apparent diffusion coefficient (3%, 95% CI 1.7% to 4.6%, P

Background Racial/ethnic and socioeconomic disparities exist in outcomes for children with congenital heart disease. We sought to determine the influence of race/ethnicity and mediating socioeconomic factors on 1‐year outcomes for live‐born infants with hypoplastic left heart syndrome and dextro‐Transposition of the great arteries. Methods and Results The authors performed a population‐based cohort study using the California Office of Statewide Health Planning and Development database. Live‐born infants without chromosomal anomalies were included. The outcome was a composite measure of mortality or unexpected hospital readmissions within the first year of life defined as >3 (hypoplastic left heart syndrome) or >1 readmissions (dextro‐Transposition of the great arteries). Hispanic ethnicity was compared with non‐Hispanic white ethnicity. Mediation analyses determined the percent contribution to outcome for each mediator on the pathway between race/ethnicity and outcome. A total of 1796 patients comprised the cohort (n=964 [hypoplastic left heart syndrome], n=832 [dextro‐Transposition of the great arteries]) and 1315 were included in the analysis (n=477 non‐Hispanic white, n=838 Hispanic). Hispanic ethnicity was associated with a poor outcome (crude odds ratio, 1.72; 95% confidence interval [CI], 1.37–2.17). Higher maternal education (crude odds ratio 0.5; 95% CI, 0.38–0.65) and private insurance (crude odds ratio, 0.65; 95% CI, 0.45–0.71) were protective. In the mediation analysis, maternal education and insurance status explained 33.2% (95% CI, 7–66.4) and 27.6% (95% CI, 6.5–63.1) of the relationship between race/ethnicity and poor outcome, while infant characteristics played a minimal role. Conclusions Socioeconomic factors explain a significant portion of the association between Hispanic ethnicity and poor outcome in neonates with critical congenital heart disease. These findings identify vulnerable populations that would benefit from resources to lessen health disparities.

Background Infants with critical congenital heart disease (CCHD) are more likely to be small for gestational age (GA). It is unclear how this affects mortality. The authors investigated the effect of birth weight Z score on 1‐year mortality separately in preterm (GA