Suboptimal Imaging on Obstetric Ultrasound Should Prompt Early Referral for Fetal Echocardiography.

Recent studies suggest that suboptimal cardiac imaging on routine obstetric anatomy ultrasound (OB-scan) is not associated with a higher risk for congenital heart disease (CHD) and, therefore, should not be an indication for fetal echocardiography (F-echo). We aim to determine the incidence of CHD in patients referred for suboptimal imaging in a large catchment area, including regions that are geographically distant from a tertiary care center. We conducted a retrospective chart review of patients referred to Seattle Children's Hospital (SCH) and SCH Regional Cardiology sites (SCH-RC) from 2011 to 2021 for F-echo with the indication of suboptimal cardiac imaging by OB-scan. Of 454 patients referred for suboptimal imaging, 21 (5%) of patients were diagnosed with CHD confirmed on postnatal echo. 10 patients (2%) required intervention by age one. Mean GA at F-echo was significantly later for suboptimal imaging compared to all other referral indications (27.5 ± 3.9 vs 25.2 ± 5.2 weeks, p < 0.01). Mean GA at F-echo was also significantly later at SCH-RC compared to SCH (29.2 ± 4.6 vs 24.2 ± 2.9 weeks; p < 0.01). In our experience, CHD in patients referred for suboptimal imaging is higher (5%) than previously described, suggesting that routine referral for is warranted. Furthermore, while suboptimal imaging was associated with a delayed F-echo compared to other indications, this delay was most striking for those seen at regional sites. This demonstrates a potential disparity for these patients and highlights opportunities for targeted education in cardiac assessment for primary providers in these regions.

Prospective Evaluation of High Titer Autoantibodies and Fetal Home Monitoring in the Detection of Atrioventricular Block Among Anti-SSA/Ro Pregnancies.

OBJECTIVE: This prospective study of pregnant patients, Surveillance To Prevent AV Block Likely to Occur Quickly (STOP BLOQ), addresses the impact of anti-SSA/Ro titers and utility of ambulatory monitoring in the detection of fetal second-degree atrioventricular block (AVB). METHODS: Women with anti-SSA/Ro autoantibodies by commercial testing were stratified into high and low anti-52-kD and/or 60-kD SSA/Ro titers applying at-risk thresholds defined by previous evaluation of AVB pregnancies. The high-titer group performed fetal heart rate and rhythm monitoring (FHRM) thrice daily and weekly/biweekly echocardiography from 17-26 weeks. Abnormal FHRM prompted urgent echocardiography to identify AVB. RESULTS: Anti-52-kD and/or 60-kD SSA/Ro met thresholds for monitoring in 261 of 413 participants (63%); for those, AVB frequency was 3.8%. No cases occurred with low titers. The incidence of AVB increased with higher levels, reaching 7.7% for those in the top quartile for anti-60-kD SSA/Ro, which increased to 27.3% in those with a previous child who had AVB. Based on levels from 15 participants with paired samples from both an AVB and a non-AVB pregnancy, healthy pregnancies were not explained by decreased titers. FHRM was considered abnormal in 45 of 30,920 recordings, 10 confirmed AVB by urgent echocardiogram, 7 being second-degree AVB, all <12 hours from normal FHRM and within another 0.75 to 4 hours to echocardiogram. The one participant with second/third-degree and two participants with third-degree AVB were diagnosed by urgent echocardiogram >17 to 72 hours from an FHRM. Surveillance echocardiograms detected no AVB when the preceding interval FHRM recordings were normal. CONCLUSION: High-titer antibodies are associated with an increased incidence of AVB. Anti-SSA/Ro titers remain stable over time and do not explain the discordant recurrence rates, suggesting that other factors are required. Fetal heart rate and rhythm (FHRM) with results confirmed by a pediatric cardiologist reliably detects conduction abnormalities, which may reduce the need for serial echocardiograms.

Parents' Psychological and Decision-Making Outcomes following Prenatal Diagnosis with Complex Congenital Heart Defect: An Exploratory Study.

Background. Parents with a fetus diagnosed with a complex congenital heart defect (CHD) are at high risk of negative psychological outcomes. Purpose. To explore whether parents' psychological and decision-making outcomes differed based on their treatment decision and fetus/neonate survival status. Methods. We prospectively enrolled parents with a fetus diagnosed with a complex, life-threatening CHD from September 2018 to December 2020. We tested whether parents' psychological and decision-making outcomes 3 months posttreatment differed by treatment choice and survival status. Results. Our sample included 23 parents (average Age[years]: 27 ± 4, range = 21-37). Most were women (n = 18), non-Hispanic White (n = 20), and married (n = 21). Most parents chose surgery (n = 16), with 11 children surviving to the time of the survey; remaining parents (n = 7) chose comfort-directed care. Parents who chose comfort-directed care reported higher distress (x¯ = 1.51, s = 0.75 v. x¯ = 0.74, s = 0.55; Mdifference = 0.77, 95% confidence interval [CI], 0.05-1.48) and perinatal grief (x¯ = 91.86, s = 22.96 v. x¯ = 63.38, s = 20.15; Mdifference = 27.18, 95% CI, 6.20-48.16) than parents who chose surgery, regardless of survival status. Parents who chose comfort-directed care reported higher depression (x¯ = 1.64, s = 0.95 v. x¯ = 0.65, s = 0.49; Mdifference = 0.99, 95% CI, 0.10-1.88) than parents whose child survived following surgery. Parents choosing comfort-directed care reported higher regret (x¯ = 26.43, s = 8.02 v. x¯ = 5.00, s = 7.07; Mdifference = 21.43, 95% CI, 11.59-31.27) and decisional conflict (x¯ = 20.98, s = 10.00 v. x¯ = 3.44, s = 4.74; Mdifference = 17.54, 95% CI; 7.75-27.34) than parents whose child had not survived following surgery. Parents whose child survived following surgery reported lower grief (Mdifference = -19.71; 95% CI, -39.41 to -0.01) than parents whose child had not. Conclusions. The results highlight the potential for interventions and care tailored to parents' treatment decisions and outcomes to support parental coping and well-being. Highlights: Question: Do the psychological and decision-making outcomes of parents differ based on their treatment decision and survival outcome following prenatal diagnosis with complex CHD?Findings: In this exploratory study, parents who decided to pursue comfort-directed care after a prenatal diagnosis reported higher levels of psychological distress and grief as well as higher decisional conflict and regret than parents who decided to pursue surgery.Meaning: The findings from this exploratory study highlight potential differences in parents' psychological and decision-making outcomes following a diagnosis of complex CHD for their fetus, which appear to relate to the treatment approach and the treatment outcome and may require tailoring of psychological and decision support.

Racial and Ethnic Disparities in Cardiac Reintervention After Pediatric Cardiac Surgical Procedures.

BACKGROUND: Children undergoing cardiac surgical procedures may require postoperative surgical or catheter-based reintervention before discharge. We examined racial/ethnic variations in reintervention and associated in-hospital death. METHODS: Children undergoing cardiac surgical procedures from 2004 to 2015 were identified in the Pediatric Health Information Systems (PHIS) database. Regression analysis measured associations between race/ethnicity, in-hospital death, and postoperative cardiac surgical or catheter-based reintervention (surgical/catheter reintervention). RESULTS: Of 124,263 patients, 8265 (6.7%) had a surgical/catheter reintervention. Black patients had fewer reinterventions (5.9% vs 6.7%) and higher in-hospital mortality (3.9% vs 2.7%, P < .01) than White patients. After adjusting for sociodemographic and illness severity indicators, Black patients remained less likely to receive surgical/catheter reintervention (adjusted hazard ratio [aHR], 0.89; 95% CI, 0.82-0.98) despite having similar risk of death after reintervention (adjusted odds ratio, 1.17; 95% CI, 0.98-1.41) compared with White patients. The risk of death without surgical/catheter reintervention was also higher for Black (aHR, 1.26; 95% CI, 1.08-1.47) and other race/ethnicity (aHR, 1.33; 95% CI, 1.13-1.57) patients than for White patients. Similar trends were demonstrated when mechanical circulatory support and cardiac transplantation were included as reinterventions. CONCLUSIONS: Patients of Black and other race/ethnicity undergoing pediatric cardiac surgical procedures are more likely to die without postoperative cardiac reintervention than White patients. Black patients are also less likely to receive reintervention despite no significant difference in mortality with reintervention. Further studies should evaluate etiologies and methods of addressing these disparities.

Fetal Echocardiographic Z Score Pilot Project: Study Design and Impact of Gestational Age and Variable Type on Reproducibility of Measurements Within and Across Investigators.

BACKGROUND: Fetal echocardiography is widely available, but normative data are not robust. In this pilot study, the authors evaluated (1) the feasibility of prespecified measurements in a normal fetal echocardiogram to inform study design and (2) measurement variability to assign thresholds of clinical significance and guide analyses in larger fetal echocardiographic Z score initiatives. METHODS: Images from predefined gestational age groups (16-20, >20-24, >24-28, and >28-32 weeks) were retrospectively analyzed. Fetal echocardiography expert raters attended online group training and then independently analyzed 73 fetal studies (18 per age group) in a fully crossed design of 53 variables; each observer repeated measures for 12 fetuses. Kruskal-Wallis tests were used to compare measurements across centers and age groups. Coefficients of variation (CoVs) were calculated at the subject level for each measurement as the ratio of SD to mean. Intraclass correlation coefficients were used to show inter- and intrarater reliabilities. Cohen's d > 0.8 was used to define clinically important differences. Measurements were plotted against gestational age, biparietal diameter, and femur length. RESULTS: Expert raters completed each set of measurements in a mean of 23 ± 9 min/fetus. Missingness ranged from 0% to 29%. CoVs were similar across age groups for all variables (P < .05) except ductus arteriosus mean velocity and left ventricular ejection time, which were both higher at older gestational age. CoVs were >15% for right ventricular systolic and diastolic widths despite fair to good repeatability (intraclass correlation coefficient > 0.5); ductal velocities and two-dimensional measures, left ventricular short-axis dimensions, and isovolumic times all had high CoVs and high interobserver variability despite good to excellent intraobserver agreement (intraclass correlation coefficient > 0.6). CoVs did not improve when ratios (e.g., tricuspid/mitral annulus) were used instead of linear measurements. Overall, 27 variables had acceptable inter- and intraobserver repeatability, while 14 had excessive variability between readers despite good intraobserver agreement. CONCLUSIONS: There is considerable variability in fetal echocardiographic quantification in clinical practice that may affect the design of multicenter fetal echocardiographic Z score studies, and not all measurements may be feasible for standard normalization. As missingness was substantial, a prospective design will be needed. Data from this pilot study may aid in the calculation of sample sizes and inform thresholds for distinguishing clinically significant from statistically significant effects.

Parents' quality of life and health after treatment decision for a fetus with severe congenital heart defect.

PURPOSE: This exploratory study examines differences in parents' quality of life by treatment decision and the child's survival outcome in the context of life-threatening congenital heart disease (CHD). DESIGN AND METHODS: Parents of a fetus or neonate diagnosed with severe CHD enrolled in the observational control group of a clinical trial (NCT04437069) and completed quality of life (i.e., contact with clinicians, social support, partner relationship, state of mind), mental and physical health survey measures. Comparisons were made between parents who chose comfort-directed care or surgery and between those whose child did and did not survive. RESULTS: Parents who chose surgery and their child did not survive reported the most contact with their clinicians. Parents who chose comfort-directed care reported lower social support than parents who chose surgery and their child did not survive as well as poorer state of mind compared to parents who chose surgery. CONCLUSIONS: Some aspects of parents' quality of life differed based on their treatment decision. Parents who choose comfort-directed care are vulnerable to some negative outcomes. PRACTICE IMPLICATIONS: Decision support tools and bereavement resources to assist parents with making and coping with a complex treatment decision is important for clinical care.

Variation in provider compliance with sports restriction guidelines in children with an isolated bicuspid aortic valve.

BACKGROUND: Published guidelines for sports restriction for children with a bicuspid aortic valve remain controversial. We sought to describe practice variation and factors influencing sports restrictions in these children. METHODS: This retrospective single-centre study included children (7-18 years old) with an isolated bicuspid aortic valve at baseline from 1 January, 2005 to 31 December, 2014. Sports restrictions, factors potentially influencing decision-making, and outcomes were collected. Descriptive statistics and multivariable mixed-effects logistic regression models were performed with providers and patients as random effects. Provider variation was estimated using intraclass correlation coefficients. Odds ratios, 95% confidence intervals, and p-values were reported from the models. RESULTS: In 565 encounters (253 children; 34 providers), 41% recommended no sports restrictions, 40% recommended high-static and high-dynamic restrictions, and 19% had no documented recommendations. Based on published guidelines, 22% of children were inappropriately restricted while 30% were not appropriately restricted. The paediatric cardiology provider contributed to 37% of observed practice variation (p < 0.001). Sports restriction was associated with older age, males, greater ascending aorta z-score, and shorter follow-up interval. There were no aortic dissections or deaths and one cardiac intervention. CONCLUSION: Physicians frequently fail to document sports restrictions for children with a bicuspid aortic valve, and documented recommendations often conflict with published guidelines. Despite this, no adverse outcomes occurred. Providers accounted for a significant proportion of the variation in sports restrictions. Further research to provide evidence-based guidelines may improve provider compliance with activity recommendations in this population.

Prenatally diagnosed congenital heart disease: the cost of maternal care.

OBJECTIVE: Little is known regarding the effects of a prenatal diagnosis of congenital heart disease (CHD) on the cost of antenatal and delivery care. We sought to compare the maternal costs of care in pregnancies where the fetus or child was diagnosed prenatally vs. postnatally. METHODS: Costs of maternal care were determined for pregnancies in which the fetus or child was diagnosed with CHD between 1997 and 2012 in the state of Utah. Cases of CHD were identified via a statewide birth defect surveillance program which included data on the timing of diagnosis, maternal demographic and clinical data, and linked to statewide inpatient maternal hospital discharge records. Antenatal testing costs were determined using Medicaid fee estimates and total facility costs were determined for all hospitalizations including delivery. The association of timing of diagnosis of CHD with costs was analyzed using univariable and multivariable models. RESULTS: Of 2128 pregnancies included in the study, 36% had a fetus prenatally diagnosed with CHD. The prenatal diagnosis group was more likely to have a termination or stillbirth and were younger at delivery (gestational age 37.3 vs 38.0 weeks, p < .001). Labor induction and cesarean delivery rates were similar between groups. Antenatal testing and delivery hospitalization costs were higher in the prenatal diagnosis group: $5819 vs $4041 (p < .001) and $10,509 vs $7802 (p < .001), respectively. Patients in the prenatal diagnosis group had longer lengths of hospital stays (3.5 vs 2.4 d, p > .001). After controlling for significant differences between the groups, including lesion severity, the prenatal diagnosis remained directly associated with antenatal testing costs (+$1472), maternal hospitalization costs (+$2713), and maternal hospital length of stay (+1.0 d). CONCLUSION: A prenatal diagnosis of fetal CHD was associated with increased prenatal costs, hospitalization costs, and hospital length of stay for affected pregnant patients.

Parents' decision-making for their foetus or neonate with a severe congenital heart defect.

BACKGROUND: Parents who receive a diagnosis of a severe, life-threatening CHD for their foetus or neonate face a complex and stressful decision between termination, palliative care, or surgery. Understanding how parents make this initial treatment decision is critical for developing interventions to improve counselling for these families. METHODS: We conducted focus groups in four academic medical centres across the United States of America with a purposive sample of parents who chose termination, palliative care, or surgery for their foetus or neonate diagnosed with severe CHD. RESULTS: Ten focus groups were conducted with 56 parents (Mage = 34 years; 80% female; 89% White). Results were constructed around three domains: decision-making approaches; values and beliefs; and decision-making challenges. Parents discussed varying approaches to making the decision, ranging from relying on their "gut feeling" to desiring statistics and probabilities. Religious and spiritual beliefs often guided the decision to not terminate the pregnancy. Quality of life was an important consideration, including how each option would impact the child (e.g., pain or discomfort, cognitive and physical abilities) and their family (e.g., care for other children, marriage, and career). Parents reported inconsistent communication of options by clinicians and challenges related to time constraints for making a decision and difficulty in processing information when distressed. CONCLUSION: This study offers important insights that can be used to design interventions to improve decision support and family-centred care in clinical practice.

Cumulative In-Hospital Costs Associated With Single-Ventricle Palliation.

Background: In the SVR (Single Ventricle Reconstruction) Trial, 1-year survival in recipients of right ventricle to pulmonary artery shunts (RVPAS) was superior to that in those receiving modified Blalock-Taussig-Thomas shunts (MBTTS), but not in subsequent follow-up. Cost analysis is an expedient means of evaluating value and morbidity. Objectives: The purpose of this study was to evaluate differences in cumulative hospital costs between RVPAS and MBTTS. Methods: Clinical data from SVR and costs from Pediatric Health Information Systems database were combined. Cumulative hospital costs and cost-per-day-alive were compared serially at 1, 3, and 5 years between RVPAS and MBTTS. Potential associations between patient-level factors and cost were explored with multivariable models. Results: In total, 303 participants (55% of the SVR cohort) from 9 of 15 sites were studied (48% MBTTS). Observed total costs at 1 year were lower for MBTTS ($701,260 ± 442,081) than those for RVPAS ($804,062 ± 615,068), a difference that was not statistically significant (P = 0.10). Total costs were also not significantly different at 3 and 5 years (P = 0.21 and 0.32). Similarly, cost-per-day-alive did not differ significantly for either group at 1, 3, and 5 years (all P > 0.05). In analyses of transplant-free survivors, total costs and cost-per-day-alive were higher for RVPAS at 1 year (P = 0.05 for both) but not at 3 and 5 years (P > 0.05 for all). In multivariable models, aortic atresia and prematurity were associated with increased cost-per-day-alive across follow-up (P < 0.05). Conclusions: Total costs do not differ significantly between MBTTS and RVPAS. The magnitude of longitudinal costs underscores the importance of efforts to improve outcomes in this vulnerable population.

Study protocol for a randomised clinical trial of a decision aid and values clarification method for parents of a fetus or neonate diagnosed with a life-threatening congenital heart defect.

INTRODUCTION: Parents who receive the diagnosis of a life-threatening, complex heart defect in their fetus or neonate face a difficult choice between pursuing termination (for fetal diagnoses), palliative care or complex surgical interventions. Shared decision making (SDM) is recommended in clinical contexts where there is clinical equipoise. SDM can be facilitated by decision aids. The International Patient Decision Aids Standards collaboration recommends the inclusion of values clarification methods (VCMs), yet little evidence exists concerning the incremental impact of VCMs on patient or surrogate decision making. This protocol describes a randomised clinical trial to evaluate the effect of a decision aid (with and without a VCM) on parental mental health and decision making within a clinical encounter. METHODS AND ANALYSIS: Parents who have a fetus or neonate diagnosed with one of six complex congenital heart defects at a single tertiary centre will be recruited. Data collection for the prospective observational control group was conducted September 2018 to December 2020 (N=35) and data collection for two intervention groups is ongoing (began October 2020). At least 100 participants will be randomised 1:1 to two intervention groups (decision aid only vs decision aid with VCM). For the intervention groups, data will be collected at four time points: (1) at diagnosis, (2) postreceipt of decision aid, (3) postdecision and (4) 3 months postdecision. Data collection for the control group was the same, except they did not receive a survey at time 2. Linear mixed effects models will assess differences between study arms in distress (primary outcome), grief and decision quality (secondary outcomes) at 3-month post-treatment decision. ETHICS AND DISSEMINATION: This study was approved by the University of Utah Institutional Review Board. Study findings have and will continue to be presented at national conferences and within scientific research journals. TRIAL REGISTRATION NUMBER: NCT04437069 (Pre-results).

Provider insights on shared decision-making with families affected by CHD.

BACKGROUND AND OBJECTIVES: Little data exist on provider perspectives about counselling and shared decision-making for complex CHD, ways to support and improve the process, and barriers to effective communication. The goal of this qualitative study was to determine providers' perspectives regarding factors that are integral to shared decision-making with parents faced with complex CHD in their fetus or newborn; and barriers and facilitators to engaging in effective shared decision-making. METHODS: We conducted semi-structured interviews with providers from different areas of practice who care for fetuses and/or children with CHD. Providers were recruited from four geographically diverse centres. Interviews were recorded, transcribed, and analysed for key themes using an open coding process with a grounded theory approach. RESULTS: Interviews were conducted with 31 providers; paediatric cardiologists (n = 7) were the largest group represented, followed by nurses (n = 6) and palliative care providers (n = 5). Key barriers to communication with parents that providers identified included variability among providers themselves, factors that influenced parental comprehension or understanding, discrepant expectations, circumstantial barriers, and trust/relationship with providers. When discussing informational needs of parents, providers focused on comprehensive short- and long-term outcomes, quality of life, and breadth and depth that aligned with parental goals and needs. In discussing resources to support shared decision-making, providers emphasised the need for comprehensive, up-to-date information that was accessible to parents of varying situations and backgrounds. CONCLUSIONS: Provider perspectives on decision-making with families with CHD highlighted key communication issues, informational priorities, and components of decision support that can enhance shared decision-making.

Changes in Provider Prescribing Behavior for Infants with Single Ventricle Physiology After Evidence-Based Publications.

BACKGROUND: The impact of published evidence on clinical practice has been understudied in pediatric cardiology. OBJECTIVE: We sought to assess changes in prescribing behavior for angiotensin-converting enzyme inhibitor (ACEI) and digoxin at discharge after initial palliation of infants with single ventricle (SV) physiology following the publication of two large studies: The Pediatric Heart Network Infant Single Ventricle (PHN-ISV) trial showing no benefit with routine ACEI use and the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) analysis showing an association between digoxin and survival. METHODS: ICD-9-10 codes identified SV infants from the Pediatric Health Information System (1/2004 to 1/2018) and charge codes identified medications at discharge. Generalized estimating equations implementing segmented logistic regressions modeled medication use, before and after (with a 3-month washout period) the relevant publication (ACEI 7/1/2010; digoxin 4/1/2016). A subgroup analysis was performed for hypoplastic left heart syndrome (HLHS). RESULTS: ACEI use (37 centers, n = 4700) at discharge did not change over time during the pre-publication period. After publication of the PHN-ISV trial, ACEI use decreased (OR: 0.61, CI 0.44-0.84, p = 0.003). Digoxin use (43 centers, n = 4778) decreased by 1% monthly before publication. After the NPC-QIC publication, digoxin use increased (OR: 2.07, CI 1.05-4.08, p = 0.04) with an ongoing increase of 9% per month. Results were similar for the HLHS subgroup. CONCLUSIONS: Prescribing behavior changed congruently after the publication of evidence-based studies, with decreased ACEI use and increased digoxin use at discharge following initial palliation of SV infants. Our findings suggest scientific findings were rapidly implemented into clinical practice.

Impact of Socioeconomic Status, Race and Ethnicity, and Geography on Prenatal Detection of Hypoplastic Left Heart Syndrome and Transposition of the Great Arteries.

BACKGROUND: Prenatal detection (PND) has benefits for infants with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA), but associations between sociodemographic and geographic factors with PND have not been sufficiently explored. This study evaluated whether socioeconomic quartile (SEQ), public insurance, race and ethnicity, rural residence, and distance of residence (distance and driving time from a cardiac surgical center) are associated with the PND or timing of PND, with a secondary aim to analyze differences between the United States and Canada. METHODS: In this retrospective cohort study, fetuses and infants <2 months of age with HLHS or TGA admitted between 2012 and 2016 to participating Fetal Heart Society Research Collaborative institutions in the United States and Canada were included. SEQ, rural residence, and distance of residence were derived using maternal census tract from the maternal address at first visit. Subjects were assigned a SEQ z score using the neighborhood summary score or Canadian Chan index and separated into quartiles. Insurance type and self-reported race and ethnicity were obtained from medical charts. We evaluated associations among SEQ, insurance type, race and ethnicity, rural residence, and distance of residence with PND of HLHS and TGA (aggregate and individually) using bivariate analysis with adjusted associations for confounding variables and cluster analysis for centers. RESULTS: Data on 1862 subjects (HLHS: n=1171, 92% PND; TGA: n=691, 58% PND) were submitted by 21 centers (19 in the United States). In the United States, lower SEQ was associated with lower PND in HLHS and TGA, with the strongest association in the lower SEQ of pregnancies with fetal TGA (quartile 1, 0.78 [95% CI, 0.64-0.85], quartile 2, 0.77 [95% CI, 0.64-0.93], quartile 3, 0.83 [95% CI, 0.69-1.00], quartile 4, reference). Hispanic ethnicity (relative risk, 0.85 [95% CI, 0.72-0.99]) and rural residence (relative risk, 0.78 [95% CI, 0.64-0.95]) were also associated with lower PND in TGA. Lower SEQ was associated with later PND overall; in the United States, rural residence and public insurance were also associated with later PND. CONCLUSIONS: We demonstrate that lower SEQ, Hispanic ethnicity, and rural residence are associated with decreased PND for TGA, with lower SEQ also being associated with decreased PND for HLHS. Future work to increase PND should be considered in these specific populations.

Influence of Geographic Access on Surgical Center Readmissions After Index Congenital Heart Surgery.

OBJECTIVE: To assess the impact of geographic access to surgical center on readmission risk and burden in children after congenital heart surgery. STUDY DESIGN: Children <6 years old at discharge after congenital heart surgery (Risk Adjustment for Congenital Heart Surgery-1 score 2-6) were identified using Pediatric Health Information System data (46 hospitals, 2004-2015). Residential distance from the surgery center, calculated using ZIP code centroids, was categorized as <15, 15-29, 30-59, 60-119, and ≥120 miles. Rurality was defined using rural-urban commuting area codes. Geographic risk factors for unplanned readmissions to the surgical center and associated burden (total hospital length of stay [LOS], costs, and complications) were analyzed using multivariable regression. RESULTS: Among 59 696 eligible children, 19 355 (32%) had ≥1 unplanned readmission. The median LOS was 9 days (IQR 22) across the entire cohort. In those readmitted, median total costs were $31 559 (IQR $90 176). Distance from the center was inversely related but rurality was positively related to readmission risk. Among those readmitted, increased distance was associated with longer LOS, more complications, and greater costs. Compared with urban patients, highly rural patients were more likely to have an unplanned readmission but had fewer average readmission days. CONCLUSIONS: Geographic measures of access differentially affect readmission to the surgery center. Increased distance from the center was associated with fewer unplanned readmissions but more complications. Among those readmitted, the most isolated patients had the greatest readmission costs. Understanding the contribution of geographic access will aid in developing strategies to improve care delivery to this population.

Implications of Left Ventricular Dysfunction at Presentation for Infants with Coarctation of the Aorta.

Infants with aortic coarctation may present with left ventricular (LV) dysfunction which may complicate the postoperative course and lead to increased healthcare costs. We aimed to define the prevalence of moderate to severe left ventricular (LV) systolic dysfunction, evaluate time to recovery, and compare health care costs. Single-center retrospective cohort study at a tertiary care hospital was conducted. Infants < 6 months old at diagnosis with aortic coarctation were identified using surgical codes for coarctation repair between January 2010 and May 2018. Moderate to severe dysfunction was defined as ejection fraction (EF) < 40%. Of 160 infants studied, 18 (11%) had moderate to severe LV dysfunction at presentation. Compared to those with better LV function, infants with moderate to severe LV dysfunction were older at presentation (12 vs. 6 days, p = 0.004), had more postoperative cardiac intensive care unit (ICU) days (5 vs. 3, p < 0.001), and more ventilator days (3.5 vs. 1, p < 0.001). The median time to normal LV EF (≥ 55%) was 6 days postoperatively (range 1-230 days). Infants presenting with moderate to severe LV dysfunction had higher index hospitalization costs ($90,560 vs. $59,968, p = 0.02), but no difference in cost of medical follow-up for the first year following discharge ($3,078 vs. $2,568, p = 0.46). In the current era, > 10% of infants with coarctation present with moderate to severe LV dysfunction that typically recovers. Those with moderate to severe dysfunction had longer duration of mechanical ventilation and postoperative cardiac ICU stays, likely driving higher costs of index hospitalization.

Prenatal cardiac care: Goals, priorities & gaps in knowledge in fetal cardiovascular disease: Perspectives of the Fetal Heart Society.

Perinatal cardiovascular care has evolved considerably to become its own multidisciplinary field of care. Despite advancements, there remain significant gaps in providing optimal care for the fetus, child, mother, and family. Continued advancement in detection and diagnosis, perinatal care and delivery planning, and prediction and improvement of morbidity and mortality for fetuses affected by cardiac conditions such as heart defects or functional or rhythm disturbances requires collaboration between the multiple types of specialists and providers. The Fetal Heart Society was created to formalize and support collaboration between individuals, stakeholders, and institutions. This article summarizes the challenges faced to create the infrastructure for advancement of the field and the measures the FHS is undertaking to overcome the barriers to support progress in the field of perinatal cardiac care.