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OBJECTIVES: Obesity may pose additional cardiovascular risk to children with acquired and congenital heart disease. Many children with heart disease are sedentary as a result of physician-, parent-, and/or self-imposed restrictions. The aim of this study was to evaluate the impact of the epidemic of obesity on children with heart disease. PATIENTS AND METHODS: A cross-sectional review was performed of children evaluated in 2004 at 2 cardiology outpatient clinics. Differences in the prevalence of obese (BMI > or = 95%) and overweight (BMI 85%-95%) children were compared with national data and healthy control subjects. Dictated letters were reviewed to determine whether obesity was discussed with referring practitioners. RESULTS: Of 2921 patients assessed, 1523 had heart disease. Diagnostic subgroups included 'mild' heart disease (n = 401), arrhythmia (n = 447), biventricular repair (n = 511), univentricular palliation (Fontan; n = 108), and heart transplantation (n = 56). More than 25% of the patients with heart disease were obese or overweight; the prevalence of obese and overweight children was significantly lower only in the Fontan group (15.9%). Pediatric cardiologists failed to document obesity or weight counseling in the majority of clinic letters. CONCLUSIONS: Obesity is common in children with congenital and acquired heart disease. Pediatric cardiologists demonstrate inadequate communication regarding this problem to referring practitioners. Healthy-lifestyle counseling and routine exercise in children with heart disease may be underemphasized. [ABSTRACT FROM AUTHOR]

Background Most epidemiological studies in pediatric heart failure (HF) use administrative database sources, defining patient cohorts by presence of a single HF ICD code. However, the ability of ICD codes to identify true HF patients is unknown in pediatrics. Here we describe the accuracy of HF ICD-10-CM code search algorithms, in identifying pediatric patients with HF from electronic data sources. Methods Based on the adult HF literature, search algorithms were designed to incorporate HF ICD codes, imaging, and medications. Sensitivity, specificity, positive and negative predictive value and accuracy of the algorithms were tested among children in an advanced HF clinic ("Clinic cohort"). Top-performing algorithms were then tested in a large-scale regional electronic data warehouse (EDW), 01/2017 to 01/2020, generating the "EDW Cohort". False positive cases were identified and characterized by chart review. Results Within the Clinic Cohort, 78/378 patients (21%) had gold standard HF diagnoses. A search algorithm with one HF ICD coded visit was more sensitive but less specific than >1 HF ICD coded visit, (sensitivity 94% and specificity 89% versus 69% and 97%, respectively). Correspondingly, >1 ICD coded visit had a higher PPV than one ICD coded visit; 84% vs. 69%. Accuracy was similar (90% vs 91%). Presence of 1 HF ICD code combined with HF medication had high sensitivity, specificity, PPV, NPV and accuracy, all higher than the single ICD code algorithm. The "1 HF coded visit + any medication" algorithm resulted in highest accuracy (93%). Top-performing algorithms were tested in the EDW: the algorithm with > 1 HF ICD coded visit, and the algorithm with one HF ICD coded visit combined with HF medication. In the EDW Cohort, 133/248 (53.6%) patients had gold standard HF diagnoses though 115/248 (46.3%) were false positive cases; 41% of those had pulmonary over-circulation from congenital heart disease. Excluding children < 30 days old and those with a history of an isolated VSD repair, complete AVSD repair, or PDA closure further reduced the proportion of false positives to 50/248 (20%). Conclusions A search algorithm using a single HF ICD code can have acceptable sensitivity, specificity, PPV, NPV and accuracy in identifying children with HF from within electronic medical records. Similar to adult HF literature, specificity improves by including HF medication. When applied to large data sources, however, the search algorithms result in a high proportion of patients with pulmonary overcirculation related to congenital heart disease. To narrow the population to those with myocardial dysfunction, case identification may require use of ICD codes with linked of administrative, surgical, and/or imaging databases.

Background: Pulmonary rehabilitation (PR) is vital for managing chronic respiratory diseases. This study aimed to assess PR practices in India, focusing on quality standards, provider affiliations, service offerings, and structural components., Materials and Methods: A survey was conducted among Indian cardiopulmonary physiotherapists via WhatsApp, Facebook, and Gmail, covering demographics, structural, process, and outcome quality indicators, and PR delivery challenges., Results: Of 50 respondents, 54% were affiliated with educational institutions, and 46% with private establishments. Significant variability in PR practices was observed, primarily in urban areas. Key challenges included limited awareness, accessibility issues, inadequate interdisciplinary teamwork, and lack of standardization. Structural elements varied, with inconsistent team compositions and uneven resource distribution. Only 36% of centers conducted regular audit meetings, and many lacked essential emergency equipment., Conclusion: The survey highlights the need for standardized protocols and national guidelines to improve PR service consistency and quality in India. Emphasizing multidisciplinary teams, regular audits, and comprehensive data collection can enhance PR delivery and outcomes. Further research is needed to develop robust, evidence-based PR programs across diverse settings in India., (© Journal of the Association of Physicians of India 2024.)

Background: The interstage period after discharge from stage 1 palliation carries high morbidity and mortality. The impact of social determinants of health on interstage outcomes is not well characterized. We assessed the relationship between childhood opportunity and acute interstage outcomes., Methods: Infants discharged home after stage 1 palliation in the National Pediatric Quality Improvement Collaborative Phase II registry (2016-2022) were retrospectively reviewed. Zip code-level Childhood Opportunity Index (COI), a composite metric of 29 indicators across education, health and environment, and socioeconomic domains, was used to classify patients into 5 COI levels. Acute interstage outcomes included death or transplant listing, unplanned readmission, intensive care unit admission, unplanned catheterization, and reoperation. The association between COI level and acute interstage outcomes was assessed using logistic regression with sequential adjustment for potential confounders., Results: The analysis cohort included 1837 patients from 69 centers. Birth weight ( P <0.001) and proximity to a surgical center at birth ( P =0.02) increased with COI level. Stage 1 length of stay decreased ( P =0.001), and exclusive oral feeding rate at discharge increased ( P <0.001), with higher COI level. More than 98% of patients in all COI levels were enrolled in home monitoring. Death or transplant listing occurred in 101 (5%) patients with unplanned readmission in 987 (53%), intensive care unit admission in 448 (24%), catheterization in 345 (19%), and reoperation in 83 (5%). There was no difference in the incidence or time to occurrence of any acute interstage outcome among COI levels in unadjusted or adjusted analysis. There was no interaction between race and ethnicity and childhood opportunity in acute interstage outcomes., Conclusions: Zip code COI level is associated with differences in preoperative risk factors and stage 1 palliation hospitalization characteristics. Acute interstage outcomes, although common across the spectrum of childhood opportunity, are not associated with COI level in an era of highly prevalent home monitoring programs. The role of home monitoring in mitigating disparities during the interstage period merits further investigation., Competing Interests: None.

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., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

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., Competing Interests: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: American Heart Association’s Strategically Focused Research Network – Children’s grant (17SFRN33660465) to Dr. Fagerlin. Dr. Thorpe was supported by grant No. 51300302 from the American Heart Association Children’s Strategically Focused Research Network fellowship awarded to Dr. Fagerlin. Dr. Delaney’s effort was supported by the National Institutes of Health under Ruth L. Kirschstein National Research Service Award T32HL007576 from the National Heart, Lung, and Blood Institute. The American Heart Association had no role in the design or conduct of the study., (© The Author(s) 2023.)

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.

Competing Interests: Disclosures None.

Objective: Fetuses with congenital heart disease (CHD) are at increased risk of pregnancy loss compared with the general population. We aimed to assess the incidence, timing and risk factors of pregnancy loss in cases with major fetal CHD, overall and according to cardiac diagnosis., Methods: This was a retrospective, population-level cohort study of fetuses and infants diagnosed with major CHD between 1997 and 2018 identified by the Utah Birth Defect Network (UBDN), excluding cases with termination of pregnancy and minor cardiovascular diagnoses (e.g. isolated aortic/pulmonary pathology and isolated septal defects). The incidence and timing of pregnancy loss were recorded, overall and according to CHD diagnosis, with further stratification based on presence of isolated CHD vs additional fetal diagnosis (genetic diagnosis and/or extracardiac malformation). Adjusted risk of pregnancy loss was calculated and risk factors were assessed using multivariable models for the overall cohort and prenatal diagnosis subgroup., Results: Of 9351 UBDN cases with a cardiovascular code, 3251 cases with major CHD were identified, resulting in a study cohort of 3120 following exclusion of cases with pregnancy termination (n = 131). There were 2956 (94.7%) live births and 164 (5.3%) cases of pregnancy loss, which occurred at a median gestational age of 27.3 weeks. Of study cases, 1848 (59.2%) had isolated CHD and 1272 (40.8%) had an additional fetal diagnosis, including 736 (57.9%) with a genetic diagnosis and 536 (42.1%) with an extracardiac malformation. The observed incidence of pregnancy loss was highest in the presence of mitral stenosis (< 13.5%), hypoplastic left heart syndrome (HLHS) (10.7%), double-outlet right ventricle with normally related great vessels or not otherwise specified (10.5%) and Ebstein's anomaly (9.9%). The adjusted risk of pregnancy loss was 5.3% (95% CI, 3.7-7.6%) in the overall CHD population and 1.4% (95% CI, 0.9-2.3%) in cases with isolated CHD (adjusted risk ratio, 9.0 (95% CI, 6.0-13.0) and 2.0 (95% CI, 1.0-6.0), respectively, based on the general population risk of 0.6%). On multivariable analysis, variables associated with pregnancy loss in the overall CHD population included female fetal sex (adjusted odds ratio (aOR), 1.6 (95% CI, 1.1-2.3)), Hispanic ethnicity (aOR, 1.6 (95% CI, 1.0-2.5)), hydrops (aOR, 6.7 (95% CI, 4.3-10.5)) and additional fetal diagnosis (aOR, 6.3 (95% CI, 4.1-10)). On multivariable analysis of the prenatal diagnosis subgroup, years of maternal education (aOR, 1.2 (95% CI, 1.0-1.4)), presence of an additional fetal diagnosis (aOR, 2.7 (95% CI, 1.4-5.6)), atrioventricular valve regurgitation ≥ moderate (aOR, 3.6 (95% CI, 1.3-8.8)) and ventricular dysfunction (aOR, 3.8 (95% CI, 1.2-11.1)) were associated with pregnancy loss. Diagnostic groups associated with pregnancy loss were HLHS and variants (aOR, 3.0 (95% CI, 1.7-5.3)), other single ventricles (aOR, 2.4 (95% CI, 1.1-4.9)) and other (aOR, 0.1 (95% CI, 0-0.97)). Time-to-pregnancy-loss analysis demonstrated a steeper survival curve for cases with an additional fetal diagnosis, indicating a higher rate of pregnancy loss compared to cases with isolated CHD (P < 0.0001)., Conclusions: The risk of pregnancy loss is higher in cases with major fetal CHD compared with the general population and varies according to CHD type and presence of additional fetal diagnoses. Improved understanding of the incidence, risk factors and timing of pregnancy loss in CHD cases should inform patient counseling, antenatal surveillance and delivery planning. © 2023 International Society of Ultrasound in Obstetrics and Gynecology., (© 2023 International Society of Ultrasound in Obstetrics and Gynecology.)

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., Competing Interests: Declaration of Competing Interest The authors have indicated they have no financial relationships and no potential conflicts of interest relevant to this article to disclose., (Published by Elsevier Inc.)

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.

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 ( M age = 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.

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., Competing Interests: The SVR trial was funded by the Pediatric Heart Network/10.13039/100000050National Heart, Lung, and Blood Institute (HL068269, HL068270, HL068279, HL068281, HL068285, HL068288, HL068290, HL068292, and HL085057). Dr O'Byrne received support from 10.13039/100000050NHLBI (K23 HL130420-01). The project also received assistance from The Pediatric Heart Network’s Integrated CARDiac Data and Outcomes Collaborative (iCARD). The Pediatric Heart Network Ancillary Studies Committee and Single Ventricle Reconstruction Trial Committee reviewed the proposed research and manuscript for appropriateness but did not participate in the drafting of the paper. The views expressed are solely of the authors and do not reflect those of the funders and other supporting groups. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.PERSPECTIVESCOMPETENCY IN MEDICAL KNOWLEDGE: Costs (which are reflective of morbidity) overall did not differ between participants randomized to a Blalock-Taussig-Thomas shunt and those to a RVPAS. However, analysis of survivors suggests that the cost of living with a RVPAS is higher, consistent with higher reintervention rates, while higher mortality rates increased costs of the Blalock-Taussig-Thomas shunt strategy. Differences in cost were derived from differences in the first year of life where the majority of total costs were accrued. Efforts to improve care should focus on improving outcomes in this period. TRANSLATIONAL OUTLOOK: Although it did not reveal significant differences in costs between shunt types, this analysis of the SVR trial leads to a better understanding of the economic and medical impact of operative palliation for single ventricle heart disease., (© 2022 The Authors.)

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)., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

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., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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.

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.

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.

Post COVID-19 sequelae includes breathlessness, weakness, fatigue, decreased exercise tolerance and impaired quality of life. Physiotherapy based rehabilitation program is an essential component for post COVID-19 patients in facilitating maximum functional recovery. Expert consensus statements are available from the developed countries. There is a need for a guidelines to manage post COVID-19 sequelae in Indian context. The objective of this consensus statement is to provide evidence informed guidelines for post COVID-19 physiotherapy management as a component of pulmonary rehabilitation. This consensus statement was developed by expert panel across India. Published literatures were appraised and used to prepare the recommendations. This is the first of its kind of work providing preliminary guidelines for post COVID-19 physiotherapy., (© Journal of the Association of Physicians of India 2011.)

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., (© 2020 Elsevier B.V. All rights reserved.)

Background: Optimal methods to assess resource utilization in congenital heart surgery remain unclear. We compared traditional cost-to-charge ratio methods with newer standardized cost methods that aim to more directly assess resources consumed., Methods: Clinical data from The Society of Thoracic Surgeons Database were linked with resource use data from the Pediatric Health Information Systems Database (2010 to 2015). Standardized cost methods specific to the congenital heart surgery population were developed and compared with cost-to-charge ratio methods. Resource use in the overall population and variability across hospitals were described using hierarchical mixed effect models adjusting for case-mix., Results: Overall, 43 hospitals (65,331 patients) were included. There were minimal population-level differences in the distribution of resource use as estimated by the two methods. At the hospital level, there was less apparent variability in resource use across centers with the standardized cost vs cost-to-charge ratio method, overall (coefficient of variation 20% vs 25%) and across complexity (The Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery [STAT]) categories. When hospitals were categorized into tertiles by resource use, 33% changed classification depending on which resource use method was used (26% by one tertile and 7% by two tertiles)., Conclusions: In this first evaluation of standardized cost methodology in the congenital heart population, we found minimal differences vs traditional methods at the population level. At the hospital level, the magnitude of variation in resource use was less with standardized cost methods, and approximately one third of centers changed resource use categories depending on the methodology used. Because of these differences, care should be taken in future studies and in benchmarking and reporting efforts in selecting optimal methodology., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: We leveraged decomposition analysis, commonly used in labor economics, to understand determinants of cost differences related to location of admission in children undergoing neonatal congenital heart surgery., Design: A retrospective cohort study., Setting: Pediatric Health Information Systems database., Patients: Neonates (<30 d old) undergoing their index congenital heart surgery between 2004 and 2013., Measurements and Main Results: A decomposition analysis with bootstrapping determined characteristic (explainable by differing covariate levels) and structural effects (if covariates are held constant) related to cost differences. Covariates included center volume, age at admission, prematurity, sex, race, genetic or major noncardiac abnormality, Risk Adjustment for Congenital Heart Surgery-1 score, payor, admission year, cardiac arrest, infection, and delayed sternal closure.Of 19,984 infants included (10,491 [52%] to cardiac ICU/PICU and 9,493 [48%] to neonatal ICU), admission to the neonatal ICU had overall higher average costs ($24,959 ± $3,260; p < 0.001) versus cardiac ICU/PICU admission. Characteristic effects accounted for higher costs in the neonatal ICU ($28,958 ± $2,044; p < 0.001). Differing levels of prematurity, genetic syndromes, hospital volume, age at admission, and infection contributed to higher neonatal ICU costs, with infection rate providing the most significant contribution ($13,581; p < 0.001). Aggregate structural effects were not associated with cost differences for those admitted to the neonatal ICU versus cardiac ICU/PICU (p = 0.1). Individually, prematurity and age at admission were associated with higher costs due to structural effects for infants admitted to the neonatal ICU versus cardiac ICU/PICU., Conclusions: The difference in cost between neonatal ICU and cardiac ICU/PICU admissions is largely driven by differing prevalence of risk factors between these units. Infection rate was a modifiable factor that accounted for the largest difference in costs between admitting units.

Introduction: This study aimed to assess whether ultrasonic activation (UA) or the EasyClean (EC; BassiEndo, Belo Horizonte, BH, Brazil) or EDDY (ED; VDW, Munich, Germany) systems used to promote agitation of the irrigating solutions during the final irrigation step can lead to smear layer formation in the apical third of the root canal., Methods: Thirteen premolars were instrumented with the Reciproc R40 file (VDW) and embedded in silicone, forming a closed irrigation/aspiration system. The teeth were cleaved, and 4 indentations were made on the inner buccal wall of the canal to standardize the observation sites. All the specimens were cleaned in an ultrasonic bath and evaluated under environmental scanning electron microscopy, thus constituting the control group. The same specimens were reassembled, submitted to final irrigation using UA or the ED or EC systems, and classified using a 4-level scoring system. The data were analyzed using the kappa, Pearson, and Kruskal-Wallis tests (P < .05)., Results: Smear layer formation occurred in all of the experimental groups and at all apical levels. At 3 and 4 mm, all of the experimental groups had significantly higher levels of smear layer formation than the control group. At 2 mm, the level of smear layer formation in the UA group was significantly higher than that of the control group, and there were no significant differences among the EC, ED, and control groups. At 1 mm, there were no significant differences between the ED and control groups, and the levels of smear layer formation in the EC and UA groups were significantly higher than that of the control group. There were no significant differences between the ED and EC groups at any of the apical levels., Conclusions: The smear layer formation occurred in all of the specimens submitted to final irrigation, irrespective of the technique used., (Copyright © 2020 American Association of Endodontists. Published by Elsevier Inc. All rights reserved.)

Background Significant variability in morbidity and mortality persists for children with functionally single ventricle congenital heart disease (SV-CHD) despite standardization in medical and surgical care. We hypothesized that maternal health factors may be associated with an increased risk of poor outcomes in children with SV-CHD. Methods and Results This retrospective, observational, cohort study included term maternal-infant pairs with a diagnosis of SV-CHD who underwent surgical palliation from 2006 to 2015 at Primary Children's Hospital. Pairs lacking maternal variables of interest or infant follow-up data were excluded. The association of maternal risk factors of abnormal pre-pregnancy body mass index, abnormal gestational weight gain (<7 or >20 kg), hypertensive disorders, and gestational diabetes mellitus with death/transplant and hemodynamics were analyzed using regression models. Of 190 infants, 135 (71%) maternal-infant dyads had complete data for inclusion. Death or transplant occurred in 48 infants (36%) during an average follow-up of 2.2 years (0.1-11.7 years). Abnormal gestational weight gain was associated with an increased risk of death and/or transplant in logistic regression (odds ratio, 3.22; 95% CI, 1.32-7.86; P =0.01), but not Cox regression (hazard ratio, 1.9; 95% CI, 1.0-3.7; P =0.055). Mean pulmonary artery pressures were higher in the setting of abnormal gestational weight gain (16.5±2.9 versus 14.7±3.0 mm Hg; P <0.001), and abnormal pre-pregnancy body mass index (15.7±3.5 versus 14.2±2.1 mm Hg; P <0.001) in the systemic right ventricle group. Conclusions Abnormal gestational weight gain (excessive or inadequate) is a novel risk factor for worse outcomes in SV-CHD. The fetoplacental environment may alter the trajectory of vascular development to impact outcomes in infants with SV-CHD.

Objectives: Prenatal detection of congenital heart disease with obstetric screening remains at less than 50% in most population studies, far from what is thought to be achievable. We sought to identify barriers/facilitators for screening from the perspective of interpreting physicians and to understand how these barriers/facilitators may be associated with interpretation of screening images., Methods: Our mixed-methods studies included 4 focus groups in centers across the United States with obstetric, maternal-fetal medicine, and radiology providers who interpreted obstetric ultrasound studies. Themes around barriers/facilitators for fetal heart screening were coded from transcripts. A national Web-based survey was then conducted, which quantitatively measured reported barriers/facilitators and measured physicians' ability to interpret fetal heart-screening images. Multivariable generalized linear random-effect models assessed the association between barriers/facilitators and the accuracy of image interpretation at the image level., Results: Three main themes were identified in the focus groups: intrinsic barriers (ie, comfort with screening), external barriers (ie, lack of feedback), and organizational barriers (ie, study volumes). Among 190 physician respondents, 104 interpreted ultrasound studies. Perceptions of barriers varied by practice setting, with nontertiary providers having lower self-efficacy and perceived usefulness of cardiac screening. Facilitators associated with the odds of accurate interpretation of screening images were knowledge (odds ratio, 2.54; P = .002) and the volume of scans per week (odds ratio, 1.01 for every additional scan; P = .04)., Conclusions: Some of the main barriers to cardiac screening identified and prioritized by physicians across the United States were knowledge of screening and minimal volumes of scans. Targeting these barriers will aid in improving prenatal detection of congenital heart disease., (© 2019 by the American Institute of Ultrasound in Medicine.)

Objective: We surveyed obstetric sonographers, who are at the forefront of the screening process to determine how barriers to prenatal cardiac screening impacted screening abilities., Methods: We performed a cross-sectional national survey of obstetric sonographers in the United States using a sampling frame from American Registry of Diagnostic Medical Sonography mailing lists. The web survey measured the ability to obtain and interpret fetal heart images. Several cognitive, sociodemographic, and system-level factors were measured, including intention to perform cardiac imaging. Regression and mediation analyses determined factors associated with intention to perform and ability to obtain and interpret cardiac images. Subgroup analyses of sonographers in tertiary versus nontertiary centers were also performed., Results: Survey response rate either due to noncontact or nonresponse was 40%. Of 480 eligible sonographers, ~30% practiced in tertiary settings. Sonographers had lower intention to perform outflow views compared to 4 chambers. Higher self-efficacy and professional expectations predicted higher odds of intention to perform outflow views (OR 2.8, 95% CI 1.9-4.2 and 1.9, 95% CI 1.1-3.0, respectively). Overall accuracy of image interpretation was 65% (±14%). For the overall cohort and nontertiary subgroup, higher intention to perform outflows was associated with increased accuracy in overall image interpretation. For the tertiary subgroup, self-efficacy and feedback were strongly associated with accuracy., Conclusions: We identified several modifiable (some heretofore unrecognized) targets to improve prenatal cardiac screening. Priorities identified by sonographers that are associated with screening success should guide future interventions., (© 2019 S. Karger AG, Basel.)

Tympanoplasty which is the repair of the tympanic membrane using temporalis fascia, has been done worldwide and has stood the test of time. However in cases of reperforation or large/subtotal perforations, we are often left in need of some sturdy material for grafting. To compare the graft uptake and hearing improvement in patients undergoing type I tympanoplasty using temporalis fascia alone and temporalis fascia along with conchal cartilage. The current research is a prospective study of 60 patients with chronic suppurative otitis media (Tubo tympanic type), undergoing type I tympanoplasty, using temporalis fascia alone and temporalis fascia along with conchal cartilage. The graft uptake and hearing improvement was much better using temporalis fascia along with conchal cartilage graft as compared to cartilage alone. The use of temporalis fascia along with conchal cartilage graft is beneficial for patients with chronic suppurative otitis media (tubotympanic type) undergoing type I tympanoplasty than using temporalis fascia alone., (© Association of Otolaryngologists of India 2018.)

Recent years have seen an exponential increase in the variety of healthcare data captured across numerous sources. However, mechanisms to leverage these data sources to support scientific investigation have remained limited. In 2013 the Pediatric Heart Network (PHN), funded by the National Heart, Lung, and Blood Institute, developed the Integrated CARdiac Data and Outcomes (iCARD) Collaborative with the goals of leveraging available data sources to aid in efficiently planning and conducting PHN studies; supporting integration of PHN data with other sources to foster novel research otherwise not possible; and mentoring young investigators in these areas. This review describes lessons learned through the development of iCARD, initial efforts and scientific output, challenges, and future directions. This information can aid in the use and optimisation of data integration methodologies across other research networks and organisations.

Objective: To assess longitudinal estimates of inpatient costs through early childhood in patients with critical congenital heart defects (CCHDs), for whom reliable estimates are scarce, using a population-based cohort of clinically validated CCHD cases., Study Design: Longitudinal retrospective cohort of infants with CCHDs live born from 1997 to 2012 in Utah. Cases identified from birth defect registry data were linked to inpatient discharge abstracts and vital records to track inpatient days and costs through age 10 years. Costs were adjusted for inflation and discounted by 3% per year to generate present value estimates. Multivariable models identified infant and maternal factors potentially associated with higher resource utilization and were used to calculate adjusted costs by defect type., Results: The final statewide cohort included 1439 CCHD cases among 803 509 livebirths (1.8/1000). The average cost per affected child through age 10 years was $136 682 with a median of $74 924 because of a small number of extremely high cost children; costs were highest for pulmonary atresia with ventricular septal defect and hypoplastic left heart syndrome. Inpatient costs increased by 1.6% per year during the study period. A single birth year cohort (~50 000 births/year) had estimated expenditures of $11 902 899 through age 10 years. Extrapolating to the US population, inpatient costs for a single birth year cohort through age 10 years were ~$1 billion., Conclusions: Inpatient costs for CCHDs throughout childhood are high and rising. These revised estimates will contribute to comparative effectiveness research aimed at improving the value of care on a patient and population level., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Aim: To describe neurobehavioral patterns in neonates with congenital heart disease (CHD)., Method: A cohort study describing neurobehavioral performance of neonates with CHD requiring cardiac surgery. The neonates were evaluated preoperatively and postoperatively with the Neonatal Intensive Care Unit Network Neurobehavioral Scale (NNNS) and scores were compared with published normative values. Clinical factors were obtained by chart review to assess their association with behavior. The CHD NNNS score pattern was compared with previously reported profiles in other high-risk populations., Results: NNNS evaluations were completed on 67 neonates with CHD, resulting in 97 evaluations (50 preoperative, 47 postoperative). Compared with normative values, the cohort with CHD demonstrated decreased attention, regulation, asymmetry, stress, arousal, and excitability, along with increased non-optimal reflexes, lethargy, and need for handling (p<0.05 for all). Additional clinical factors had a minimal effect on the neurobehavioral pattern. Compared with previously published patterns in high-risk neonates without CHD, the cohort with CHD demonstrated a unique pattern of behavior., Interpretation: Neonates with CHD demonstrate different neurobehavioral performance compared with typically developing neonates born at term as well as other high-risk neonates. Our experience suggests there is a unique neonatal neurobehavioral pattern in the hospitalized population with CHD. Targeted neonatal neurobehavioral evaluations may be useful in developing specific therapies to improve neurodevelopmental outcomes in neonates with CHD., What This Paper Adds: Neonates with congenital heart disease demonstrate different neurobehavioral performance than typically developing neonates. Evaluation of neonatal neurobehavioral performance provides an opportunity to identify neurodevelopmental variability early. Identification of neurobehavioral performance variability allows targeted interactions and therapy., (© 2018 Mac Keith Press.)

Deletion of 22q11.2 (del22q11) is associated with adverse outcomes in patients with tetralogy of Fallot (TOF). We sought to investigate its contribution to perioperative outcome in patients with a severe form of TOF characterized by pulmonary atresia (PA) or severe pulmonary stenosis (PS) and major aortopulmonary collateral arteries (MAPCAS). We conducted a retrospective review of patients with TOF/MAPCAS who underwent staged surgical reconstruction between 1995 and 2006. Groups were compared according to 22q11.2 deletion status using t-tests or the Wilcoxon Rank sum test. We included 26 subjects, 24 of whom survived the initial operation. Of those, 21 subjects had known deletion status and constitute the group for this analysis [15 with no deletion present (ND) and 6 del22q11 subjects]. There was no difference with respect to occurrence of palliative procedure prior to initial operation, or to timing of closure of the ventricular septal defect (VSD). Other than higher prevalence of prematurity (50%) in the del22q11 group versus no prematurity in the ND, the groups were comparable in terms of pre-operative characteristics. The intra- and post-operative course outcomes (length of cardiopulmonary bypass, use of vasopressors, duration of intensive care and length of hospital stay, tube-feeding) were also comparable. Although the del22q11 had longer mechanical ventilation than the ND, this difference was not significant [68 h (range 4-251) vs. 45 h (range 3-1005), p = 0.81]. In this detailed comparison of a small patient cohort, 22q11.2 deletion syndrome was not associated with adverse perioperative outcomes in patients with TOF, PA, and MAPCAS when compared to those without 22q11.2 deletion syndrome. These results are relevant to prenatal and neonatal pre-operative counseling and planning.

Objective: Neonates undergoing congenital heart surgery require highly specialized, resource-intensive care. Location of care and degree of specialization can vary between and within institutions. Using a multi-institutional cohort, we sought to determine whether location of admission is associated with an increase in health care costs, resource use and mortality., Methods: We retrospectively analyzed admission for neonates (<30 days) undergoing congenital heart surgery between 2004 and 2013 by using the Pediatric Health Information Systems database (44 children's hospitals). Multivariate generalized estimating equations adjusted for center- and patient-specific risk factors and stratified by age at admission were performed to examine the association of admission intensive care unit (ICU) with total hospital costs, mortality, and length of stay., Results: Of 19,984 neonates (60% male) identified, 39% were initially admitted to a cardiac ICU (CICU), 48% to a neonatal ICU (NICU), and 13% to a pediatric ICU. In adjusted models, admission to a CICU versus NICU was associated with a $20,440 reduction in total hospital cost for infants aged 2 to 7 days at admission (P = .007) and a $23,700 reduction in total cost for infants aged 8 to 14 days at admission (P = .01). Initial admission to a CICU or pediatric ICU versus NICU at <15 days of age was associated with shorter hospital and ICU length of stay and fewer days of mechanical ventilation. There was no difference in adjusted mortality by admission location., Conclusions: Admission to an ICU specializing in cardiac care is associated with significantly decreased hospital costs and more efficient resource use for neonates requiring cardiac surgery., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Disease progression of an isolated bicuspid aortic valve (BAV) in children is poorly understood and adult management guidelines may not be applicable. Thus, we sought to evaluate disease progression of pediatric isolated BAV and its relationship to current management practices., Methods: Children with a BAV and ≤mild aortic stenosis (AS) and/or aortic regurgitation (AR) at the time of initial evaluation were included in this retrospective cohort study (1/2005-12/2014). Outcomes included change in z-scores for aortic root and ascending aorta diameters, cardiac interventions, adverse outcomes, recommended follow-up interval, and frequency of cardiac imaging studies at each follow up evaluation, as well as AS/AR severity at final evaluation. Outcomes were analyzed using generalized mixed-effect models with subject and provider clustering., Results: BAV disease progression was evaluated in 294 subjects over 4.1 ± 2.4 (range 0.2-9.5) years. Ascending aorta z-scores increased by 0.1/year (P < .001) but aortic root diameter z-scores were unchanged. AS and/or AR progressed to >mild in 9 (3%), 1 subject underwent cardiac intervention, and none had a major complication. Management was evaluated in 454 subjects (1343 encounters) with 27 different cardiologists. The average recommended follow-up interval was 1.5 ± 0.9 years. Younger age at diagnosis, greater aortic root or ascending aorta z-score at diagnosis, ≥mild AS/AR at follow-up, and earlier diagnosis era were associated with shorter recommended follow-up interval (P < .001 for all). Imaging was obtained at 87% of follow-up encounters and was associated with age at encounter with children ≥12 years most frequently imaged (P < .001). Provider accounted for 14% of variability in recommended follow-up interval and 24% of imaging variability (P < .001 for both)., Conclusions: We found little to no evidence of disease progression in children with an isolated BAV. Given the low risk, close follow-up and frequent cardiac imaging for BAV surveillance may not be warranted for children., (© 2018 Wiley Periodicals, Inc.)

Pediatric heart transplantation (HT) is resource intensive. Event-driven pediatric databases do not capture data on resource use. The objective of this study was to evaluate resource utilization and identify associated factors during initial hospitalization for pediatric HT. This multicenter retrospective cohort study utilized the Pediatric Health Information Systems database (43 children's hospitals in the United States) of children ≤19 years of age who underwent transplant between January 2007 and July 2013. Demographic variables including site, payer, distance and time to center, clinical pre- and post-transplant variables, mortality, cost, and charge were the data collected. Total length of stay (LOS) and charge for the initial hospitalization were used as surrogates for resource use. Charges were inflation adjusted to 2013 dollars. Of 1,629 subjects, 54% were male, and the median age at HT was 5 years (IQR [interquartile range] 0 to 13). The median total and intensive care unit LOS were 51 (IQR 23 to 98) and 23 (IQR 9 to 58) days, respectively. Total charge and cost for hospitalization were $852,713 ($464,900 to $1,609,300) and $383,600 ($214,900 to $681,000) respectively. Younger age, lower volume center, southern region, and co-morbidities before transplant were associated with higher resource use. In later years, charges increased despite shorter LOS. In conclusion, this large multicenter study provides novel insight into factors associated with resource use in pediatric patients having HT. Peritransplant morbidities are associated with increased cost and LOS. Reducing costs in line with LOS will improve health-care value. Regional and center volume differences need further investigation for optimizing value-based care and efficient use of scarce resources., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: This study evaluates the morbidity, mortality, and cost differences between patients who underwent either a simple or a complex arterial switch operation., Methods: A retrospective study of patients undergoing an arterial switch operation at a single institution was performed. Simple cases were defined as patients with d-transposition of the great arteries with usual coronary anatomy or circumflex artery originating from the right with either intact ventricular septum or ventricular septal defect. Complex cases included all other forms of coronary anatomy, aortic coarctation or arch hypoplasia, and Taussig-Bing anomalies. Costs were acquired using an institutional activity-based accounting system., Results: A total of 98 patients were identified, 68 patients in the simple group and 30 in the complex group. The mortality rate was 2% for the simple and 7% for the complex group, p=0.23. Major morbidities including cardiac arrest, extracorporeal membrane oxygenation, a major coronary event, surgical or catheter-based re-intervention, stroke, or permanent pacemaker placement, non-cardiac surgical procedures, mediastinitis, and sepsis did not differ between the simple and complex groups (16 versus 27%, p=0.16). The complex group had increased bleeding requiring re-exploration (0 versus 10%, p=0.04). Hospital and ICU length of stay did not differ. Complex patients had higher overall hospital costs (simple $80,749 versus complex $97,387, p=0.01) and higher postoperative costs (simple $60,192 versus complex $70,132, p=0.02). The operating room and supplies accounted for the majority of the cost difference., Conclusion: Complex arterial switches can be safely performed with low rates of morbidity and mortality but at an increased cost.

Background: The impact of prenatal diagnosis of d-transposition of the great arteries (dTGA) on health-care usage is largely unknown. We evaluated a population-based cohort to assess costs, mortality and inpatient encounters by whether dTGA was prenatally diagnosed or not., Methods: The dTGA cases (born 1997-2011) identified at the Utah Birth Defect Network, which includes data on timing of diagnosis, were linked to statewide inpatient discharge data. We excluded preterm cases or cases with additional major heart defects. We evaluated hospitalizations and costs for infants (first year of life) and mothers (10 months before birth) using multivariable models adjusted for demographic and clinical risk factors., Results: Of 119 cases, 14 (12%) were prenatally diagnosed. Birth weight, surgical complexity and extracardiac defects/syndromes were similar between groups. Of 7 deaths (6%), two occurred pre-intervention in postnatally diagnosed infants. Prenatal diagnosis was associated with more in-hospital days (estimate 13 additional days, p = 0.03) and higher mean costs for mothers ($4,141 vs $12,148) and infants (90,419 vs $49,576). Prenatal diagnosis independently predicted higher adjusted costs for the overall cohort ($22,570, p = 0.045). After excluding deaths, total costs were no longer significantly different., Conclusion: Mothers of prenatally diagnosed infants with dTGA had higher inpatient costs compared with those postnatally diagnosed. Costs trended higher for their infants, although were not significantly different. Linkage of population-based surveillance systems and outcome databases can be a powerful tool to further explore the complex relationship of prenatal diagnosis to costs and outcomes in other types of congenital heart diseases. Birth Defects Research 109:262-270, 2017. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

Rozenek, R, Salassi III, JW, Pinto, NM, and Fleming, JD. Acute cardiopulmonary and metabolic responses to high-intensity interval training protocols using 60 s of work and 60 s recovery. J Strength Cond Res 30(11): 3014-3023, 2016-Low-volume, high-intensity interval training (HIIT) consisting of 60 s work and 60 s recovery (60 s/60 s) repeated for 10 times has previously been found to produce beneficial cardiopulmonary, cellular, and metabolic adaptations in healthy and at-risk populations. There is currently relatively little information pertaining to the acute changes that take place during individual training sessions. The purpose of this study was to examine the acute physiological responses to 60 s/60 s × 10 HIIT protocols using several combinations of work and recovery intensities. Eleven healthy adults (mean age ± SD = 26.0 ± 5.3 years) performed 4 HIIT trials on separate days at varying percentages of peak power output that consisted of the following work/recovery intensities: (a) 80% PPO/0% PPO (80/0); (b) 80% PPO/50% PPO (80/50); (c) 100% PPO/0% PPO (100/0); and (d) 100% PPO/50% PPO (100/50). Compared with the other protocols, 100/50 produced higher (p ≤ 0.05) peak, average, and nadir %V[Combining Dot Above]O2peak. Other than the nadir values resulting from the 80/0 trial, all trials produced average, peak, and nadir %V[Combining Dot Above]O2peak and %HRpeak values that were within exercise intensity ranges (≈45-90% V[Combining Dot Above]O2max; ≈65-90% HRmax) recommended by the American College of Sports Medicine for improvement of cardiopulmonary function. Similar average HR and peak HR, RPE, blood lactate, and %V[Combining Dot Above]O2peak values were produced by 80/50 and 100/0 protocols. However, the average %V[Combining Dot Above]O2peak was significantly higher (∼9.3% absolute) in 80/50. It appeared that use of the 80/0, 80/50, and 100/0 protocols would be appropriate for individuals who are at the low to moderate end of the cardiopulmonary fitness spectrum.

Current guidelines recommend that all neonates with Down syndrome (DS) be screened for congenital heart disease (CHD) with an echocardiogram. We sought to determine the effectiveness of a more accessible and less expensive screening strategy consisting of physical examination, electrocardiogram (ECG), and chest X-ray. The Intermountain Healthcare Enterprise Data Warehouse was used to identify infants with a positive karyotype for DS who were born between January 1, 2000, and June 30, 2012. Infants with the results of an echocardiogram, physical examination, ECG, and chest X-ray documented at age ≤6 months were included. Infants with an abnormality on physical examination, ECG, or chest X-ray were considered to have a positive screen. Echocardiography was the gold standard for calculating sensitivity, specificity, positive and negative predictive values for major CHD, defined as any heart defect that would typically require intervention during early childhood. Of 408 eligible infants, 240 (59 %) had major CHD, of whom 228 (95 %) had a positive screen. Screening missed eight infants with moderate/large patent ductus arteriosus and four infants with a moderate/large atrial septal defect. In 11 of these infants, the defect resolved spontaneously by age ≤4 months. One infant had a moderate atrial septal defect persisting at 2-year follow-up. Sensitivity and specificity of the screening for detecting CHD were 95 % (CI 92-98 %) and 41 % (CI 32-47 %); positive and negative predictive values were 69 % (CI 63-73 %) and 85 % (CI 75-92 %). Screening with physical examination, ECG, and chest X-ray is an effective method of identifying which infants with DS should have an echocardiogram. This method would have resulted in 69 (17 %) fewer echocardiograms without missing infants with major CHD.

Objective: Prenatal diagnosis of congenital heart disease (CHD) reportedly increases parental stress compared with postnatal diagnosis. We investigated the association of timing of diagnosis with parental stress and modifiers of this relationship., Methods: We enrolled parents with a fetus/infant diagnosed prenatally (Group 1) or postnatally (Group 2) with CHD requiring intervention prior to newborn discharge. Parents completed a Basic Symptom Inventory (BSI) - at diagnosis, birth, and follow-up. Adjusted mixed effects regression models compared scores., Results: The BSI was completed by 105 families (Group 1, n = 60 and Group 2, n = 45). On regression modeling, anxiety and global stress were lower in Group 1 (effect size -0.19 to -0.62) at diagnosis and birth though not at follow-up. When stratified by gender, Group 1 scores for anxiety and stress were primarily lower in fathers. Within Group 1, mothers scored higher in all domains and later gestational age at diagnosis was associated with higher anxiety and stress., Conclusions: Contrary to prior reports, parents of prenatally diagnosed infants with CHD had lower anxiety and stress than those diagnosed postnatally after adjusting for severity. Identifying those most vulnerable and modifiable risk factors will allow us to appropriately target psychosocial services for families with a CHD diagnosis.

Newborn screening for critical congenital heart defects (CCHD) was added to the US Recommended Uniform Screening Panel in 2011. Within 4 years, 46 states and the District of Columbia had adopted it into their newborn screening program, leading to CCHD screening being nearly universal in the United States. This rapid adoption occurred while there were still questions about the effectiveness of the recommended screening protocol and barriers to follow-up for infants with a positive screen. In response, the Centers for Disease Control and Prevention partnered with the American Academy of Pediatrics to convene an expert panel between January and September 2015 representing a broad array of primary care, neonatology, pediatric cardiology, nursing, midwifery, public health, and advocacy communities. The panel's goal was to review current practices in newborn screening for CCHD and to identify opportunities for improvement. In this article, we describe the experience of CCHD screening in the United States with regard to: (1) identifying the target lesions for CCHD screening; (2) optimizing the algorithm for screening; (3) determining state-level challenges to implementation and surveillance of CCHD; (4) educating all stakeholders; (5) performing screening using the proper equipment and in a cost-effective manner; and (6) implementing screening in special settings such as the NICU, out-of-hospital settings, and areas of high altitude., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2016 by the American Academy of Pediatrics.)

Objective: Bleaching agents may affect the properties of dental materials. The aim of this study was to evaluate the effect of different polishing techniques on the surface roughness of composite resins submitted to the at-home and in-office bleaching treatment., Materials and Methods: Disc-shaped specimens were carried out of nanofilled and microhybrid composites (n = 10). Finishing step was performed after light curing (L1) and polishing after 24 h with two systems (L2). Then, specimens were submitted to the home or in-office bleaching procedures, and roughness was re-evaluated (L3). The surface roughness (Ra) readings were measured at L1, L2, and L3 times using a profilometer. Data were statistically analyzed by multiple-way analysis of variance and Tukey test (α = 0.05)., Results: The polishing procedures decreased Ra for both composites compared to baseline values (L1). The roughness of specimens polished with jiffy did not present significant difference after polishing step (L2) and bleaching treatment (L3). However, the groups polished with Sof-Lex discs had increase on the Ra values after bleaching., Conclusion: The polishing is an important procedure to reduce the roughness of dental restorations and composite surface polished with jiffy system improved the degradation resistance to the bleaching agents compared to Sof-Lex discs.

Newborns with hypoplastic left heart syndrome and other single right ventricular variants require substantial health care resources. Weekend acute care has been associated with worse outcomes and increased resource use in other populations but has not been studied in patients with single ventricle. Subjects of the Single Ventricle Reconstruction trial were classified by whether they had a weekend admission and by day of the week of Norwood procedure. The primary outcome was hospital length of stay (LOS); secondary outcomes included transplant-free survival, intensive care unit (ICU) LOS, and days of mechanical ventilation. The Student's t test with log transformation and the Wilcoxon rank-sum test were used to analyze associations. Admission day was categorized for 533 of 549 subjects (13% weekend). The day of the Norwood was Thursday/Friday in 39%. There was no difference in median hospital LOS, transplant-free survival, ICU LOS, or days ventilated for weekend versus non-weekend admissions. Day of the Norwood procedure was not associated with a difference in hospital LOS, transplant-free survival, ICU LOS, or days ventilated. Prenatally diagnosed infants born on the weekend had lower mean birth weight, younger gestational age, and were more likely to be intubated but did not have a difference in measured outcomes. In conclusion, in this cohort of patients with single right ventricle, neither weekend admission nor end-of-the-week Norwood procedure was associated with increased use of hospital resources or poorer outcomes. We speculate that the complex postoperative course following the Norwood procedure outweighs any impact that day of admission or operation may have on these outcomes., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: There is an increasing focus on optimizing health care quality and reducing costs. The care of children undergoing heart surgery requires significant investment of resources, and it remains unclear how costs of care relate to quality. We evaluated this relationship across a multicenter cohort., Methods: Clinical data from The Society of Thoracic Surgeons Database were merged with cost data from the Pediatric Health Information Systems Database for children undergoing heart surgery (2006 to 2010). Hospital-level costs were modeled using Bayesian hierarchical methods adjusting for case-mix, and hospitals were categorized into cost tertiles. The primary quality metric evaluated was in-hospital mortality., Results: Overall, 27 hospitals (30,670 patients) were included. Median adjusted cost per case was $82,360 and varied fivefold across hospitals, while median adjusted mortality was 3.4% and ranged from 2.4% to 5.0% across hospitals. Overall, hospitals in the lowest cost tertile had significantly lower adjusted mortality rates compared with the middle and high cost tertiles (2.5% vs 3.8% and 3.5%, respectively, both p < 0.001). When assessed at the individual hospital level, most (75%) but not all hospitals in the lowest cost tertile were also in the lowest mortality tertile. Similar relationships were seen across the spectrum of surgical complexity. Lower cost hospitals also had shorter length of stay and trends toward fewer major complications., Conclusions: Lowest cost hospitals generally deliver the highest quality care for children undergoing heart surgery, although there is some variation in this relationship. This information is important in the design of initiatives aiming to optimize health care value in this population., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Based on outcome data, surgery is recommended for asymptomatic adults with chronic mitral regurgitation (MR) and systolic dysfunction, marked left ventricular (LV) dilation, pulmonary hypertension, atrial fibrillation, or high likelihood of successful repair; but indications for children are poorly defined. We sought to determine predictors of postoperative LV dysfunction in asymptomatic children with chronic MR. The surgical database was searched for all children who underwent mitral valve surgery for chronic MR (2000-2012). Exclusion criteria were preoperative symptoms, acute MR, cardiomyopathy, or other defects affecting LV size. Preoperative and latest follow-up clinical and echocardiographic data were obtained. LV dysfunction was defined as ejection fraction (EF) ≤55% or shortening fraction (SF) ≤28%. Associations between preoperative factors and late LV dysfunction were determined using univariate Poisson regression. For the 25 children who met criteria, preoperative median LV end systolic Z score (LVESZ) was 5.3, EF was 65%, and SF was 34%. At follow-up (median 3.9 years), nine patients (36%) had LV dysfunction. Lower preoperative SF (OR 0.6, p < 0.001) and higher LVESZ (OR 1.7, p < 0.01) were associated with late LV dysfunction. LVESZ ≥ 5 combined with SF ≤ 33% had a sensitivity of 89%, specificity of 88%, and negative predictive value of 93% for late LV dysfunction. Only 1/14 patients with preoperative SF > 33% had late LV dysfunction. For asymptomatic children with chronic MR, surgery should be considered before LVESZ exceeds five and SF falls below 33%. Patients with SF > 33% may be followed with serial echocardiographic measurements.

Hospital volume has been associated with improved outcomes in congenital cardiac surgery. However, the relationship between hospital volume and hospitalization cost remains unclear. This study examines the relationship between hospital surgical volume and hospitalization costs, while accounting for measures of quality, in children undergoing congenital heart surgery. A retrospective, repeated cross-sectional analysis was performed, using discharges from the 2006 and 2009 Kids' Inpatient Database. All pediatric admissions (<18 years) with a Risk Adjustment for Congenital Heart Surgery procedure and hospitalization cost/charge data were included. Multivariate, linear mixed regression models were run on hospitalization costs, with and without adjustment for indicators of quality (hospital mortality rate and complication rate). Both medium and high-volume hospitals (200-400 cases/year and >400 cases/year, respectively) were associated with lower odds of mortality but not occurrence of a complication. Hospital mortality was associated with the largest increase in hospitalization costs. High-volume hospitals (>400 cases/year) were associated with the lowest hospitalization costs per discharge ($37,775, p < 0.01) when compared to low-($43,270) and medium($41,085)-volume hospitals, prior to adjusting for quality indicators. However, when adjusting for hospital mortality rate, high-volume hospitals no longer demonstrated significant cost savings. When adjusting for hospital complication rate, high-volume hospitals continued to have the lowest hospitalization costs. High-volume hospitals are associated with a reduction in hospitalization costs that appear to be mediated through improvements in quality.