Cardiac Critical Care Fellowship Training in the United States and Canada: Pediatric Cardiac Intensive Care Society-Endorsed Subcompetencies to the 2022 Entrustable Professional Activities.

OBJECTIVES: We aimed to define and map subcompetencies required for pediatric cardiac critical care (PCCC) fellowship education and training under the auspices of the Pediatric Cardiac Intensive Care Society (PCICS). We used the 2022 frameworks for PCCC fellowship learning objectives by Tabbutt et al and for entrustable professional activities (EPAs) by Werho et al and integrated new subcompetencies to the EPAs. This complementary update serves to provide a foundation for standardized trainee assessment tools for PCCC. DESIGN: A volunteer panel of ten PCICS members who are fellowship education program directors in cardiac critical care used a modified Delphi method to develop the update and additions to the EPA-based curriculum. In this process, the experts rated information independently, and repetitively after feedback, before reaching consensus. The agreed new EPAs were later reviewed and unanimously accepted by all PCICS program directors in PCCC in the United States and Canada and were endorsed by the PCICS in 2023. PROCEDURE AND MAIN RESULTS: The procedure for defining new subcompetencies to the established EPAs comprised six consecutive steps: 1) literature search; 2) selection of key subcompetencies and curricular components; 3) written questionnaire; 4) consensus meeting and critical evaluation; 5) approval by curriculum developers; and 6) PCICS presentation and endorsement. Overall, 110 subcompetencies from six core-competency domains were mapped to nine EPAs with defined levels of entrustment and examples of simple and complex cases. To facilitate clarity and develop a future assessment tool, three EPAs were subcategorized with subcompetencies mapped to the appropriate subcategory. The latter covering common procedures in the cardiac ICU. CONCLUSIONS: This represents the 2023 update to the PCCC fellowship education and training EPAs with the defining and mapping of 110 subcompetencies to the nine established 2022 EPAs. This goal of this update is to serve as the next step in the integration of EPAs into a standardized competency-based assessment framework for trainees in PCCC.

Pre-operative Imaging of Critical Coarctation of the Aorta: Impact on Surgical Approach and Outcomes.

Computed tomographic angiography (CTA) has been increasingly used for the evaluation of infants with aortic arch hypoplasia and coarctation of the aorta. The goals of this study were to compare echocardiographic and CTA findings in critical coarctation of the aorta, to evaluate each modality's influence on surgical approach for repair and determine if pre-operative measurements or surgical approach are associated with residual lesions/re-interventions. This was a single-center retrospective cohort study that included 85 neonates and infants who underwent repair of coarctation/arch hypoplasia by three months of age. Two groups were compared: patients with pre-operative echocardiograms only and patients with both echocardiogram and CTA evaluations. 44 (52%) patients received an echocardiogram and CTA, and 41 (48%) patients received an echocardiogram only. Patients in the CTA + echo group had smaller mitral valve and ascending aorta measurements (p = 0.01). When comparing CTA to echocardiogram measurements, the aortic valve annulus, ascending aorta, proximal and distal transverse arch, and isthmus were smaller on echo (p < 0.01). A smaller aortic valve annulus and aortic root as well as thoracotomy approach were associated with residual gradients/re-intervention (p < 0.01). Our study found that patients who underwent CTA preoperatively had smaller left-sided structures. Aortic measurements were smaller on echocardiogram when compared to CTA. Smaller left-sided structures proximal to the aortic arch and thoracotomy predicted the development of residual lesions/re-intervention. CTA is useful in the surgical planning for neonates with arch hypoplasia/coarctation and may help risk stratify for residual lesions/re-intervention.

A Psychosocial Care Model for Families Affected by Congenital Heart Disease.

OBJECTIVES: To develop a model of family-based psychosocial care for congenital heart disease (CHD). DESIGN: Qualitative study using crowdsourced data collected from parents of young children with CHD who received care across 42 hospitals. SETTING: Yammer, a social networking platform used to facilitate online crowdsourcing and qualitative data collection. SUBJECTS: Geographically diverse sample of 100 parents (72 mothers and 28 fathers) of young children with CHD. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Parents joined a private group on Yammer and responded to 37 open-ended study questions over a 6-month period. Qualitative data were coded and analyzed using an iterative process. Three broad themes corresponding to pillars of family-based psychosocial care were identified: pillar 1) parent partnership in family-integrated medical care, pillar 2) supportive interactions focused on parent and family wellbeing, and pillar 3) integrated psychosocial care and peer support for parents and families. Each pillar was supported by subthemes corresponding to specific intervention strategies. Most parents described the need for intervention strategies across multiple pillars, with almost half reporting needs across all three pillars of psychosocial care. Parents' preferences for psychosocial support changed over time with changes to their child's medical status and across care settings (e.g., hospital, outpatient clinic). CONCLUSIONS: Results support a model of family-based psychosocial care that is multidimensional and flexible to meet the needs of families affected by CHD. All members of the healthcare team play an important role in providing psychosocial support. Future research incorporating components of implementation science is needed to promote uptake of these findings, with the goal of optimizing family-based psychosocial support in the hospital setting and beyond.

Timing of surgical repair and resource utilisation in infants with complete atrioventricular septal defect.

INTRODUCTION: Variation exists in the timing of surgery for balanced complete atrioventricular septal defect repair. We sought to explore associations between timing of repair and resource utilisation and clinical outcomes in the first year of life. METHODS: In this retrospective single-centre cohort study, we included patients who underwent complete atrioventricular septal defect repair between 2005 and 2019. Patients with left or right ventricular outflow tract obstruction and major non-cardiac comorbidities (except trisomy 21) were excluded. The primary outcome was days alive and out of the hospital in the first year of life. RESULTS: Included were 79 infants, divided into tertiles based on age at surgery (1st = 46 to 137 days, 2nd = 140 - 176 days, 3rd = 178 - 316 days). There were no significant differences among age tertiles for days alive and out of the hospital in the first year of life by univariable analysis (tertile 1, median 351 days; tertile 2, 348 days; tertile 3, 354 days; p = 0.22). No patients died. Fewer post-operative ICU days were used in the oldest tertile relative to the youngest, but days of mechanical ventilation and hospitalisation were similar. Clinical outcomes after repair and resource utilisation in the first year of life were similar for unplanned cardiac reinterventions, outpatient cardiology clinic visits, and weight-for-age z-score at 1 year. CONCLUSIONS: Age at complete atrioventricular septal defect repair is not associated with important differences in clinical outcomes or resource utilisation in the first year of life.

Factors associated with caregiver adherence to mobile health interstage home monitoring in infants with single ventricle or biventricular shunt-dependent heart disease.

Mobile health technology is an emerging tool in interstage home monitoring for infants with single ventricle heart disease or biventricular shunt-dependent defects. This study sought to describe adherence to mobile health monitoring and identify factors and outcomes associated with adherence to mobile health monitoring. This was a retrospective, single-institution study of infants who were followed in a mobile health-based interstage home monitoring programme between February 2016 and October 2020. The analysis included 105 infants and subjects were grouped by frequency of adherence to mobile health monitoring. Within the study cohort, 16 (15.2%) had 0% adherence, 25 (23.8%) had <50% adherence, and 64 (61.0%) had >50% adherence. The adherent groups had a higher percentage of infants who were male (p = 0.02), white race (p < 0.01), non-Hispanic or non-Latinx ethnicity (p < 0.01) and had mothers with primary English fluency (p < 0.01), married marital status (p < 0.01), and a prenatal diagnosis of faetal cardiac disease (p = 0.03). Adherent groups also had a higher percentage of infants with non-Medicaid primary insurance (p < 0.01) and residence in a neighbourhood with a higher median household income (p < 0.04). Frequency of adherence was not associated with interstage mortality, unplanned cardiac reinterventions, or hospital readmissions. Impact of mobile health interstage home monitoring on caregiver stress as well as use of multi-language, low literacy, affordable mobile health options for interstage home monitoring warrant further investigation.

A late diagnosis of Pseudohypoaldosteronism type I in an infant with hypoplastic left heart syndrome presenting with failure to thrive.

Pseudohypoaldosteronism type I is caused by a peripheral resistance to aldosterone and can present with electrolyte abnormalities, poor growth, or dehydration. Although a rare disease, several case reports have been published regarding Pseudohypoaldosteronism type I in neonates and infants. We report a case of failure to thrive and hyponatremia in an infant with hypoplastic left heart syndrome who was subsequently found to have Pseudohypoaldosteronism type I.

Improved outcomes after implementation of a specialized pediatric cardiac rapid response team.

INTRODUCTION: The efficacy of a specialized pediatric cardiac rapid response team is unknown. We hypothesized that a specialized cardiac rapid response team would facilitate team-wide communication between the cardiac stepdown unit and cardiac intensive care unit (ICU) teams and improve patient care. MATERIALS AND METHODS: A specialized pediatric cardiac rapid response team was implemented in June 2015. All pediatric cardiac rapid response team activations and outcomes from implementation through December 2018 were reviewed. Cardiac arrests and unplanned transfers to the cardiac ICU were indexed to 1000 patient-days to account for inpatient volume trends and evaluated over time. RESULTS: There were 202 cardiac rapid response team activations in 108 unique patients during the study period. After implementation of the pediatric cardiac rapid response team, unplanned transfers from the cardiac stepdown unit to the cardiac ICU decreased from 16.8 to 7.1 transfers per 1000 patient days (p = 0.012). The stepdown unit cardiac arrest rate decreased from 1.2 to 0.0 arrests per 1000 patient-days (p = 0.015). There was one death on the cardiac stepdown unit in the 5 years since the implementation of the cardiac rapid response team, compared to four deaths in the previous 5 years. CONCLUSIONS: A reduction in unplanned cardiac ICU transfers, cardiac arrests, and mortality on the cardiac stepdown unit has been observed since the implementation of a specialized pediatric cardiac rapid response team. A specialized cardiac rapid response team may improve communication and empower the interdisciplinary care team to escalate care for patients experiencing clinical decline.

Parent mental health and family functioning following diagnosis of CHD: a research agenda and recommendations from the Cardiac Neurodevelopmental Outcome Collaborative.

Diagnosis of CHD substantially affects parent mental health and family functioning, thereby influencing child neurodevelopmental and psychosocial outcomes. Recognition of the need to proactively support parent mental health and family functioning following cardiac diagnosis to promote psychosocial adaptation has increased substantially over recent years. However, significant gaps in knowledge remain and families continue to report critical unmet psychosocial needs. The Parent Mental Health and Family Functioning Working Group of the Cardiac Neurodevelopmental Outcome Collaborative was formed in 2018 through support from an R13 grant from the National Heart, Lung, and Blood Institute to identify significant knowledge gaps related to parent mental health and family functioning, as well as critical questions that must be answered to further knowledge, policy, care, and outcomes. Conceptually driven investigations are needed to identify parent mental health and family functioning factors with the strongest influence on child outcomes, to obtain a deeper understanding of the biomarkers associated with these factors, and to better understand how parent mental health and family functioning influence child outcomes over time. Investigations are also needed to develop, test, and implement sustainable models of mental health screening and assessment, as well as effective interventions to optimise parent mental health and family functioning to promote psychosocial adaptation. The critical questions and investigations outlined in this paper provide a roadmap for future research to close gaps in knowledge, improve care, and promote positive outcomes for families of children with CHD.

Factors Associated with Delayed Transition to Oral Feeding in Infants with Single Ventricle Physiology.

OBJECTIVES: To describe the duration of time to achieve exclusive oral feeding in infants with single ventricle physiology and to identify risk factors associated with prolonged gastrostomy tube dependence. STUDY DESIGN: Single center, retrospective study of infants with single ventricle physiology. The primary outcome was duration of time required to achieve oral feeding. Transition periods were defined as exclusive oral feeding by Glenn palliation (early), by 1 year of age (mid), or after 1 year of age (late). RESULTS: Seventy-eight infants were analyzed; 46 (59%) were discharged to home with a gastrostomy tube after the initial hospitalization. Overall, 39 infants (50%) achieved early transition, 14 (18%) mid, and 18 (23%) late. The group who achieved early transition had a higher percentage of preoperative oral feeding (P < .01), greater weight-for-age z score at initial discharge (P = .03), shorter initial intensive care unit duration (P < .01), shorter initial hospital length of stay (P < .01), and greater weight-for-age z score at Glenn admission (P = .02). No preoperative oral feeding (OR = 0.12, P = .02) and greater number of cardiac medications at initial discharge (OR = 3.8, P = .03) were associated with failure to achieve early transition. No preoperative oral feeding (OR = 0.09, P = .01) and longer initial intensive care unit duration (OR = 1.1, P = .03) were associated with failure to achieve mid transition. CONCLUSION: Preoperative oral feeding may potentially be a modifiable factor to help improve early transition to oral feeding.

Utilisation of early intervention services in young children with hypoplastic left heart syndrome.

OBJECTIVE: Children with hypoplastic left heart syndrome are at a risk for neurodevelopmental delays. Current guidelines recommend systematic evaluation and management of neurodevelopmental outcomes with referral for early intervention services. The Single Ventricle Reconstruction Trial represents the largest cohort of children with hypoplastic left heart syndrome ever assembled. Data on life events and resource utilisation have been collected annually. We sought to determine the type and prevalence of early intervention services used from age 1 to 4 years and factors associated with utilisation of services. METHODS: Data from 14-month neurodevelopmental assessment and annual medical history forms were used. We assessed the impact of social risk and geographic differences. Fisher exact tests and logistic regression were used to evaluate associations. RESULTS: Annual medical history forms were available for 302 of 314 children. Greater than half of the children (52-69%) were not receiving services at any age assessed, whereas 20-32% were receiving two or more therapies each year. Utilisation was significantly lower in year 4 (31%) compared with years 1-3 (with a range from 40 to 48%) (p<0.001). Social risk factors were not associated with the use of services at any age but there were significant geographic differences. Significant delay was reported by parents in 18-43% of children at ages 3 and 4. CONCLUSION: Despite significant neurodevelopmental delays, early intervention service utilisation was low in this cohort. As survival has improved for children with hypoplastic left heart syndrome, attention must shift to strategies to optimise developmental outcomes, including enrolment in early intervention when merited.

Assessment of Growth 6 Years after the Norwood Procedure.

At 6 years of age, patients with hypoplastic left heart syndrome had mean age-adjusted z-scores for weight and height below the normative population, and body mass index was similar to the normative population. Males had the greatest increase in z-scores for body mass index. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00115934.

Counseling Practices for Fetal Hypoplastic Left Heart Syndrome.

While counseling parents of a fetus diagnosed with hypoplastic left heart syndrome (HLHS), pediatric cardiologists play a critical role in shaping a family's expectations for the months and years to come. However, techniques for the most effective counseling practices have not been studied, and significant variation among physicians is likely present. Web-based survey of pediatric cardiologists that perform fetal echocardiography using snowball sampling. 201 physicians responded (61% male, 81% from academic centers, and 95% from the U.S.), with an average experience of 12 years. The majority of respondents (73%) typically received initial referrals for HLHS between 20 and 24 weeks of gestation. Most physicians counsel families alone (54%), while others counsel with a nurse (35%), social worker (12%), and/or maternal-fetal medicine colleague (15%). Termination of pregnancy was discussed by 79% of respondents, although 15% did not know their state's legal limit for termination. While initial counseling sessions routinely described the typical earlier ramifications of HLHS, many long-term sequelae of the disease were not commonly discussed. Content of counseling was affected by region of the country, but not by practice setting, experience, or fetal volume. Respondents identified multiple barriers that limited their counseling practices. Our data suggest that current counseling practices often fail to cover important information. Perceived barriers to a full discourse on long-term sequelae of HLHS are common and may lead to a disconnect between reality and a family's understanding of the natural history of palliated HLHS. Opportunities to improve counseling practices exist, and there may be benefits to gain from more formal training.

Growth Asymmetry, Head Circumference, and Neurodevelopmental Outcomes in Infants with Single Ventricles.

OBJECTIVE: To assess the variability in asymmetric growth and its association with neurodevelopment in infants with single ventricle (SV). STUDY DESIGN: We analyzed weight-for-age z-score minus head circumference-for-age z-score (HCAZ), relative head growth (cm/kg), along with individual growth variables in subjects prospectively enrolled in the Infant Single Ventricle Trial. Associations between growth indices and scores on the Psychomotor Developmental Index (PDI) and Mental Developmental Index (MDI) of the Bayley Scales of Infant Development-II (BSID-II) at 14 months were assessed. RESULTS: Of the 230 subjects enrolled in the Infant Single Ventricle trial, complete growth data and BSID-II scores were available in 168 (73%). Across the cohort, indices of asymmetric growth varied widely at enrollment and before superior cavopulmonary connection (SCPC) surgery. BSID-II scores were not associated with these asymmetry indices. In bivariate analyses, greater pre-SCPC HCAZ correlated with higher MDI (r = 0.21; P = .006) and PDI (r = 0.38; P < .001) and a greater HCAZ increase from enrollment to pre-SCPC with higher PDI (r = 0.15; P = .049). In multivariable modeling, pre-SCPC HCAZ was an independent predictor of PDI (P = .03), but not MDI. CONCLUSION: In infants with SV, growth asymmetry was not associated with neurodevelopment at 14 months, but pre-SCPC HCAZ was associated with PDI. Asymmetric growth, important in other high-risk infants, is not a brain-sparing adaptation in infants with SV. TRIAL REGISTRATION: Clinicaltrials.gov: NCT00113087.

Intraoperative Steroid Use and Outcomes Following the Norwood Procedure: An Analysis of the Pediatric Heart Network's Public Database.

OBJECTIVE: Data supporting the use of perioperative steroids during cardiac surgery are conflicting, and most pediatric studies have been limited by small sample sizes and/or diverse cardiac diagnoses. The objective of this study was to determine if intraoperative steroid administration improved outcomes following the Norwood procedure. DESIGN: A retrospective analysis was performed on the 549 neonates who underwent a Norwood procedure in the publicly available datasets from the Pediatric Heart Network's Single Ventricle Reconstruction trial. Groups were compared to determine if outcomes differed between intraoperative steroid recipients (n = 498, 91%) and nonrecipients (n = 51, 9%). SETTING: Fifteen North American centers. SUBJECTS: Infants enrolled in the Single Ventricle Reconstruction trial. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Baseline characteristics and intraoperative variables were similar between groups with the exception of a shorter duration of cross clamp and cardiopulmonary bypass time in the group that received steroids. Subjects who did not receive intraoperative steroids had improved hospital survival (94% vs 83%, p = 0.03) but longer ICU stays (16 d; interquartile range, 12-33 vs 14 d; interquartile range, 9-28; p = 0.04) and hospital stays (29 d; interquartile range, 21-50 vs 23 d; interquartile range, 15-40; p = 0.01) than steroid recipients. In multivariate analysis, lengths of stay associations were no longer significant, but hospital survival trended toward favoring the nonsteroid group with an odds ratio of 3.52 (95% CI, 0.98-12.64; p = 0.054). CONCLUSIONS: In the large multicentered Single Ventricle Reconstruction trial, there was widespread use of intraoperative steroids. Intraoperative steroid administration was not associated with an improvement in outcomes and may be associated with a reduction in hospital survival in neonates undergoing the Norwood procedure. This study highlights the need for a randomized control trial.

Feasibility and Efficacy of Defatted Human Milk in the Treatment for Chylothorax After Cardiac Surgery in Infants.

Chylothorax is a well-described complication after cardiothoracic surgery in children. Medical nutritional therapy for chylothorax includes medium-chain triglyceride (MCT) formulas and reduction in enteral long-chain triglyceride intake to reduce chyle production. Human milk is usually eliminated from the diet of infants with chylothorax because of its high long-chain triglyceride content. However, given the immunologic properties of human milk, young infants with chylothorax may benefit from using human milk over human milk substitutes. We performed a retrospective cohort study to describe the feasibility and efficacy of defatted human milk (DHM) for the treatment for chylothorax in infants after cardiac surgery and to compare growth outcomes between infants treated with DHM (n = 14) versus MCT formula (n = 21). There were no differences in mortality or length of hospital stay between the DHM and MCT formula treatment groups. The DHM treatment group had a significantly higher weight-for-age z-score at hospital discharge compared to the MCT formula group with median z-scores of -1 (-2 to 0.5) and -1.5 (-2 to 0), respectively (p = 0.02). In infants with chylothorax after cardiac surgery, DHM is a safe and feasible medical nutritional treatment and may have potential benefits for improved nutrition and growth.

Randomized Clinical Trial of Preoperative Feeding to Evaluate Intestinal Barrier Function in Neonates Requiring Cardiac Surgery.

OBJECTIVES: To evaluate intestinal barrier function in neonates undergoing cardiac surgery using lactulose/mannitol (L/M) ratio measurements, and to determine correlations with early breast milk feeding. STUDY DESIGN: This was a single-center, prospective, randomized pilot study of 27 term-born neonates (≥ 37 weeks gestation) requiring cardiac surgery who were randomized to 1 of 2 preoperative feeding groups: nil per os (NPO) or trophic (10 mL/kg/day) breast milk feeds. At 3 time points (preoperative [preop], postoperative [postop] day 7, and postop day 14), subjects were administered an oral L/M solution, after which urine L/M ratios were measured using gas chromatography, with higher ratios indicative of increased intestinal permeability. Trends over time in the mean urine L/M ratios for each group were estimated using a general linear mixed model. RESULTS: There were no adverse events related to preoperative trophic feeding. In the NPO group (n = 13), the mean urine L/M ratio was 0.06 at preop, 0.12 at postop day 7, and 0.17 at postop day 14. In the trophic breast milk feeds group (n = 14), the mean urine L/M ratio was 0.09 at preop, 0.19 at postop day 7, and 0.15 at postop day 14. In both groups, L/M ratios were significantly higher at postop day 7 and postop day 14 compared with preop (P < .05). CONCLUSION: Neonates have increased intestinal permeability after cardiac surgery extending to at least postop day 14. This pilot study was not powered to detect differences in benefit or adverse events comparing the NPO and trophic breast milk feeds groups. Further studies to identify mechanisms of intestinal injury and therapeutic interventions are warranted. TRIAL REGISTRATION: Registered with ClinicalTrials.gov: NCT01475357.

Variation in feeding practices following the Norwood procedure.

OBJECTIVES: To assess variation in feeding practice at hospital discharge after the Norwood procedure, factors associated with tube feeding, and associations among site, feeding mode, and growth before stage II. STUDY DESIGN: From May 2005 to July 2008, 555 subjects from 15 centers were enrolled in the Pediatric Heart Network Single Ventricle Reconstruction Trial; 432 survivors with feeding data at hospital discharge after the Norwood procedure were analyzed. RESULTS: Demographic and clinical variables were compared among 4 feeding modes: oral only (n = 140), oral/tube (n = 195), nasogastric tube (N-tube) only (n = 40), and gastrostomy tube (G-tube) only (n = 57). There was significant variation in feeding mode among sites (oral only 0%-81% and G-tube only 0%-56%, P < .01). After adjusting for site, multivariable modeling showed G-tube feeding at discharge was associated with longer hospitalization, and N-tube feeding was associated with greater number of discharge medications (R(2) = 0.65, P < .01). After adjusting for site, mean pre-stage II weight-for-age z-score was significantly higher in the oral-only group (-1.4) vs the N-tube-only (-2.2) and G-tube-only (-2.1) groups (P = .04 and .02, respectively). CONCLUSIONS: Feeding mode at hospital discharge after the Norwood procedure varied among sites. Prolonged hospitalization and greater number of medications at the time of discharge were associated with tube feeding. Infants exclusively fed orally had a higher weight-for-age z score pre-stage II than those fed exclusively by tube. Exploring strategies to prevent morbidities and promote oral feeding in this highest risk population is warranted.

Vitamin D status in neonates undergoing cardiac operations: relationship to cardiopulmonary bypass and association with outcomes.

OBJECTIVES: To determine the incidence of vitamin D deficiency in neonates with congenital heart disease and whether differences exist by race. In addition, we determined the effect of cardiopulmonary bypass on vitamin D levels and explored associations between 25-hydroxyvitamin D [25(OH)D] levels and postoperative outcomes. STUDY DESIGN: We performed a secondary analysis of a prospective randomized controlled trial in 70 neonates undergoing cardiac surgery. The neonates' 25(OH)D levels were measured in the operating room before skin incision (baseline), at the cessation of cardiopulmonary bypass, and at 24 hours postoperatively. Associations between these levels and clinical outcomes were explored. Vitamin D deficiency was defined as a 25(OH)D level <20 ng/mL. RESULTS: Vitamin D deficiency was present in 84% (59/70); concentrations in African Americans (n = 20) were significantly lower than those of Caucasian/other race/ethnicity (n = 50) (10.2 ± 4.2 vs 16.0 ± 5.6 ng/mL, P < .0001). The 24-hour postoperative 25(OH)D levels were not different from baseline and correlated with a reduced postoperative inotropic requirement (r = -0.316, P = .008). CONCLUSIONS: Vitamin D deficiency is prevalent in neonates with congenital cardiac defects, and lower postoperative 25(OH)D levels are associated with the need for increased inotropic support in neonates undergoing cardiac operations. These findings support that vitamin D deficiency may play a role in myocardial injury and postoperative recovery and warrants further investigation.

Association of Digoxin Use With Transplant-Free Interstage Survival in Infants Palliated With a Stage 1 Hybrid Procedure.

Background Digoxin prescription in patients with single-ventricle physiology after stage 1 palliation is associated with reduced interstage death. Prior literature has primarily included patients having undergone the Norwood procedure. We sought to determine if digoxin prescription at discharge in infants following hybrid stage 1 palliation was associated with improved transplant-free interstage survival. Methods and Results A retrospective multicenter cohort analysis was conducted using data from the National Pediatric Cardiology Quality Improvement Collaborative registry data from 2008 to 2021. Infants with functional single ventricles and aortic arch obstruction discharged home after the hybrid stage 1 palliation hospitalization were included. Patients were excluded if they had supraventricular tachycardia or conversion to Norwood operation. The primary outcome was transplant-free survival. Multivariable logistic regression analysis including a propensity score for digoxin use identified associations between digoxin use and interstage death or transplant. Of 259 included infants from 45 sites, 158 (61%) had hypoplastic left heart syndrome. Forty-nine percent had a gestational age ≤38 weeks, 18% had a birth weight <2.5 kg, and 58% had a preoperative risk factor. Of the 259 subjects, 129 (50%) were discharged on digoxin. Interstage death or transplant occurred in 30 (23%) patients in the no-digoxin group compared with 18 (14%) in the digoxin group (P=0.06). With multivariate analysis, discharge digoxin prescription was associated with a lower risk of interstage death or transplant (adjusted odds ratio, 0.48 [95% CI, 0.24-0.93]; P=0.03). Conclusions In infants with single-ventricle physiology who underwent hybrid stage 1 palliation, digoxin prescription at hospital discharge was associated with improved interstage transplant-free survival.

Genetic and clinical variables act synergistically to impact neurodevelopmental outcomes in children with single ventricle heart disease.

BACKGROUND: Recent large-scale sequencing efforts have shed light on the genetic contribution to the etiology of congenital heart defects (CHD); however, the relative impact of genetics on clinical outcomes remains less understood. Outcomes analyses using genetics are complicated by the intrinsic severity of the CHD lesion and interactions with conditionally dependent clinical variables. METHODS: Bayesian Networks were applied to describe the intertwined relationships between clinical variables, demography, and genetics in a cohort of children with single ventricle CHD. RESULTS: As isolated variables, a damaging genetic variant in a gene related to abnormal heart morphology and prolonged ventilator support following stage I palliative surgery increase the probability of having a low Mental Developmental Index (MDI) score at 14 months of age by 1.9- and 5.8-fold, respectively. However, in combination, these variables act synergistically to further increase the probability of a low MDI score by 10-fold. The absence of a damaging variant in a known syndromic CHD gene and a shorter post-operative ventilator support increase the probability of a normal MDI score 1.7- and 2.4-fold, respectively, but in combination increase the probability of a good outcome by 59-fold. CONCLUSIONS: Our analyses suggest a modest genetic contribution to neurodevelopmental outcomes as isolated variables, similar to known clinical predictors. By contrast, genetic, demographic, and clinical variables interact synergistically to markedly impact clinical outcomes. These findings underscore the importance of capturing and quantifying the impact of damaging genomic variants in the context of multiple, conditionally dependent variables, such as pre- and post-operative factors, and demography.

Single ventricle congenital heart disease is a birth defect. In these children, the heart has only one effective blood-pumping chamber instead of two. Surgery can reroute the blood to use only one chamber, but multiple risk factors influence how well a child develops afterwards. Studying these risk factors can be challenging because they are interconnected, i.e. children with a genetic birth defect may be more likely to have a lower birthweight, and hence more likely to spend longer in hospital after surgery. Here, we used a statistical approach not commonly applied to study congenital heart disease and describe that whether a genetic variant (a small difference in a child's DNA) is important for how a child with single ventricle heart disease develops and grows after surgery depends on the presence of other risk factors.