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Congenital heart disease (CHD) is the most commonly reported birth defect in newborns. Neonates with CHD are more likely to be born prematurely, and a higher proportion of preterm neonates have CHD than their term counterparts. The implications of preterm birth on the cardiac and noncardiac organ systems are vast and require special management considerations. The feasibility of surgical interventions in preterm neonates is frequently limited by patient size and delicacy of immature cardiac tissues. Thus, special care must be taken when considering the appropriate timing and type of cardiac intervention. Despite improvements in neonatal cardiac surgical outcomes, preterm and early term gestational ages and low birthweight remain important risk factors for in-hospital mortality. Understanding the risks of early delivery of neonates with prenatally diagnosed CHD may help guide perioperative management in neonates who are born preterm. In this review, we will describe the risks and benefits of early delivery, postnatal cardiac and noncardiac evaluation and management, surgical considerations, overall outcomes, and future directions regarding optimization of perinatal evaluation and management of fetuses and preterm and early term neonates with CHD.

BACKGROUND: Neurodevelopmental impairment is a significant consequence for survivors of surgery for critical congenital heart disease. This study sought to determine if intraoperative methylprednisolone during neonatal cardiac surgery is associated with neurodevelopmental outcomes at 12 months of age and to identify early prognostic variables associated with neurodevelopmental outcomes. METHODS: A planned secondary analysis of a two-center, double-blind, randomized, placebo-controlled trial of intraoperative methylprednisolone in neonates undergoing cardiac surgery was performed. A brain injury biomarker was measured perioperatively. Bayley Scales of Infant and Toddler Development-III (BSID-III) were performed at 12 months of age. Two sample t-tests and generalized linear models were used. RESULTS: There were 129 participants (n=61 methylprednisolone, n=68 placebo). There were no significant differences in BSID-III scores and brain injury biomarker levels between the two treatment groups. Participants who underwent a palliative (vs. corrective) procedure had lower mean BSID-III cognitive (101±15 vs. 106±14, p=0.03) and motor scores (85±18 vs. 94±16, p

The majority of congenital cardiovascular disease including structural cardiac defects, abnormalities in cardiac function, and rhythm disturbances can be identified prenatally using screening obstetrical ultrasound with referral for fetal echocardiogram when indicated. Diagnosis of congenital heart disease in the fetus should prompt assessment for extracardiac abnormalities and associated genetic abnormalities once parental consent is obtained. Pediatric cardiologists, in conjunction with maternal-fetal medicine, neonatology, and cardiothoracic surgery subspecialists, should counsel families about the details of the congenital heart defect as well as prenatal and postnatal management. Prenatal diagnosis often leads to increased maternal depression and anxiety; however, it decreases morbidity and mortality for many congenital heart defects by allowing clinicians the opportunity to optimize prenatal care and plan delivery based on the specific lesion. Changes in prenatal care can include more frequent assessments through the remainder of the pregnancy, maternal medication administration, or, in selected cases, in utero cardiac catheter intervention or surgical procedures to optimize postnatal outcomes. Delivery planning may include changing the location, timing or mode of delivery to ensure that the neonate is delivered in the most appropriate hospital setting with the required level of hospital staff for immediate postnatal stabilization. Based on the specific congenital heart defect, prenatal echocardiogram assessment in late gestation can often aid in predicting the severity of postnatal instability and guide the medical or interventional level of care needed for immediate postnatal intervention to optimize the transition to postnatal circulation.

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 genetic data are complicated by the intrinsic severity of the CHD lesion and by interactions with conditionally dependent clinical variables. Here we apply Bayesian Networks, an explainable Artificial Intelligence solution, to describe the intertwined relationships between clinical variables, demography, and genetics in a cohort of children with single ventricle CHD. As isolated variables, a damaging genetic variant in a gene related to abnormal heart morphology and prolonged ventilator support following stage I palliative surgery increased 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 acted synergistically to further increase the probability of a low MDI score by 10-fold. Likewise, genetic information was predictive of a favorable neurodevelopmental outcome. For example, the absence of a damaging variant in a known syndromic CHD gene and a shorter post-operative ventilator support increased the probability of a normal MDI score 1.7- and 2.4-fold, respectively, but in combination increased the probability of a good outcome by 59-fold. Our analyses suggest a modest genetic contribution to neurodevelopmental and growth 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.

Objectives In the vast majority of Children's Hospitals, the critically ill patient can be found in one of three locations: the PICU, the neonatal ICU, and the cardiac ICU. Training, certification, and maintenance of certification for neonatology and critical care medicine are over seen by the Accreditation Council for Graduate Medical Education and American Board of Pediatrics. There is no standardization of training or oversight of certification and maintenance of certification for pediatric cardiac critical care. Data sources The curricula from the twenty 4th year pediatric cardiac critical care training programs were collated, along with the learning objectives from the Pediatric Cardiac Intensive Care Society published "Curriculum for Pediatric Cardiac Critical Care Medicine." Study selection This initiative is endorsed by the Pediatric Cardiac Intensive Care Society as a first step toward Accreditation Council for Graduate Medical Education oversight of training and American Board of Pediatrics oversight of maintenance of certification. Data extraction A taskforce was established of cardiac intensivists, including the directors of all 4th year pediatric cardiac critical care training programs. Data synthesis Using modified Delphi methodology, learning objectives, rotational requirements, and institutional requirements for providing training were developed. Conclusions In the current era of increasing specialized care in pediatric cardiac critical care, standardized training for pediatric cardiac critical care is paramount to optimizing outcomes.

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.

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.

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. 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.

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.

Objective:To characterise the parenting priorities of mothers and fathers of infants hospitalised with CHD and generate recommendations to support parenting during infant hospitalisation.Study design:Through online crowdsourcing, an innovative research methodology to create an online community to serve as a research sample, 79 parents of young children with CHD responded to questions about parenting during hospitalisation via private social networking site. Responses were analysed using qualitative research methods.Results:Three broad themes were identified: (1) establishing a bond with my baby, (2) asserting the parental role, and (3) coping with fear and uncertainty. Parents value provider support in restoring normalcy to the parenting experience during infant hospitalisation.Conclusions:Care teams can support parenting during infant hospitalisation by promoting parents’ roles as primary caretakers and decision-makers and attending to the emotional impact of infant hospitalisation on the family.

Pediatric cardiac intensive care is an evolving and maturing field. There have been advances in education and training in recent years, specifically progress toward standardization of curricula, competencies, and certifications. International partnerships have fostered similar advancements in less resourced countries. For all disciplines and levels of expertise, simulation remains a versatile and effective modality in education. Although there is improved standardization for the training of physicians and nurses, the certification process remains undetermined.

Background The efficacy of intraoperative corticosteroids to improve outcomes following congenital cardiac operations remains controversial. Objectives The purpose of this study was to determine whether intraoperative methylprednisolone improves post-operative recovery in neonates undergoing cardiac surgery. Methods Neonates undergoing cardiac surgery with cardiopulmonary bypass at 2 centers were enrolled in a double-blind randomized controlled trial of methylprednisolone (30 mg/kg) or placebo after the induction of anesthesia. The primary outcome was a previously validated morbidity-mortality composite that included any of the following events following surgery before discharge: death, mechanical circulatory support, cardiac arrest, hepatic injury, renal injury, or rising lactate level (>5 mmol/l). Results Of the 190 subjects enrolled, 176 (n = 81 methylprednisolone, n = 95 placebo) were included in this analysis. A total of 27 (33%) subjects in the methylprednisolone group and 40 (42%) in the placebo group reached the primary study endpoint (odds ratio [OR]: 0.63; 95% confidence interval [CI]: 0.31 to 1.3; p = 0.21). Methylprednisolone was associated with reductions in vasoactive inotropic requirements and in the incidence of the composite endpoint in subjects undergoing palliative operations (OR: 0.38; 95% CI: 0.15 to 0.99; p = 0.048). There was a significant interaction between treatment effect and center. In this analysis, methylprednisolone was protective at 1 center, with an OR: 0.35 (95% CI: 0.15 to 0.84; p = 0.02), and not so at the other center, with OR: 5.13 (95% CI: 0.85 to 30.90; p = 0.07). Conclusions Intraoperative methylprednisolone failed to show an overall significant benefit on the incidence of the composite primary study endpoint. There was, however, a benefit in patients undergoing palliative procedures and a significant interaction between treatment effect and center, suggesting that there may be center or patient characteristics that make prophylactic methylprednisolone beneficial.

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. TheParent Mental Health and Family Functioning Working Groupof 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.

This study aimed to identify barriers and facilitators to discussing parent mental health within child health care for parents of children with congenital heart disease (CHD). Seventy-nine parents of young children with CHD who received care across 40 hospitals in the United States responded to questions about barriers and facilitators to discussing their mental health with their child’s health care providers. Responses were analyzed using qualitative research methods. Parents described multiple barriers: (1) belief that parent mental health support was outside the care team’s scope of practice, (2) perceived expectation to “stay strong,” (3) fear of negative judgment or repercussion, (4) individual preferences for communication/support, (5) desire to maintain care resources on their child, (6) perceived need to compartmentalize emotions, and (7) negative reactions to past emotional disclosure. Parents also described several facilitators: (1) confidence in the care team’s ability to provide support, (2) intentional efforts by the care team to provide support, (3) naturally extroverted tendencies, and (4) developing personal connections with health care providers. It is important that health care providers normalize the impact of child illness on the family and create an environment in which parents feel comfortable discussing mental health challenges.

Background Deficits in swallowing physiology are a leading morbidity for infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliation. Despite the high prevalence of this condition, the underlying deficits that cause this post-operative impairment remain poorly understood. Objective Identify the physiologic correlates of dysphagia in infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliative surgery. Methods Postoperative fiberoptic laryngoscopies and videofluoroscopic swallow studies (VFSS) were conducted sequentially on infants with functional single ventricles following stage 1 palliative surgery. Infants were dichotomized as having normal or impaired laryngeal function based on laryngoscopy findings. VFSS were evaluated frame-by-frame using a scale that quantifies performance within 11 components of swallowing physiology. Physiologic attributes within each component were categorized as high functioning or low functioning based on their ability to support milk ingestion without bolus airway entry. Results Thirty-six infants (25 male) were included in the investigation. Twenty-four underwent the Norwood procedure and twelve underwent the Hybrid procedure. Low function physiologic patterns were observed within multiple swallowing components during the ingestion of thin barium as characterized by ≥4 sucks per swallow (36%), initiation of pharyngeal swallow below the level of the valleculae (83%), and incomplete late laryngeal vestibular closure (56%) at the height of the swallow. Swallowing deficits contributed to aspiration in 50% of infants. Although nectar thick liquids reduced the rate of aspiration (P = .006), aspiration rates remained high (27%). No differences in rates of penetration or aspiration were observed between infants with normal and impaired laryngeal function. Conclusions Deficits in swallowing physiology contribute to penetration and aspiration following stage 1 palliation among infants with normal and impaired laryngeal function. Although thickened liquids may improve airway protection for select infants, they may inhibit their ability to extract the bolus and meet nutritional needs.

For decades, physicians have administered corticosteroids in the perioperative period to infants undergoing heart surgery with cardiopulmonary bypass (CPB) to reduce the post-operative systemic inflammatory response to CPB. Some question this practice because steroid efficacy has not been conclusively demonstrated and because some studies indicate that steroids could have harmful effects. STRESS is a randomized, placebo-controlled, double blind, multi-center trial designed to evaluate safety and efficacy of perioperative steroids in infants (age < 1 year) undergoing heart surgery with CPB. Participants (planned enrollment = 1200) are randomized 1:1 to methylprednisolone (30mg/kg) administered into the CPB pump prime versus placebo. The trial is nested within the existing infrastructure of the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD). The primary outcome is a global rank score of mortality, major morbidities and hospital length of stay with components ranked commensurate with their clinical severity. Secondary outcomes include several measures of major post-operative morbidity, post-operative hospital length of stay and steroid-related safety outcomes including prevalence of hyperglycemia and post-operative infectious complications. STRESS will be one of the largest trials ever conducted in children with heart disease and will answer a decades old question related to safety and efficacy of perioperative steroids in infants undergoing heart surgery with CPB. The pragmatic “trial within a registry” design may provide a mechanism for conducting low cost, high efficiency trials in a heretofore-understudied patient population. Trial Registration Number

The efficacy of intraoperative corticosteroids to improve outcomes following congenital cardiac operations remains controversial.The purpose of this study was to determine whether intraoperative methylprednisolone improves post-operative recovery in neonates undergoing cardiac surgery.Neonates undergoing cardiac surgery with cardiopulmonary bypass at 2 centers were enrolled in a double-blind randomized controlled trial of methylprednisolone (30 mg/kg) or placebo after the induction of anesthesia. The primary outcome was a previously validated morbidity-mortality composite that included any of the following events following surgery before discharge: death, mechanical circulatory support, cardiac arrest, hepatic injury, renal injury, or rising lactate level (5 mmol/l).Of the 190 subjects enrolled, 176 (n = 81 methylprednisolone, n = 95 placebo) were included in this analysis. A total of 27 (33%) subjects in the methylprednisolone group and 40 (42%) in the placebo group reached the primary study endpoint (odds ratio [OR]: 0.63; 95% confidence interval [CI]: 0.31 to 1.3; p = 0.21). Methylprednisolone was associated with reductions in vasoactive inotropic requirements and in the incidence of the composite endpoint in subjects undergoing palliative operations (OR: 0.38; 95% CI: 0.15 to 0.99; p = 0.048). There was a significant interaction between treatment effect and center. In this analysis, methylprednisolone was protective at 1 center, with an OR: 0.35 (95% CI: 0.15 to 0.84; p = 0.02), and not so at the other center, with OR: 5.13 (95% CI: 0.85 to 30.90; p = 0.07).Intraoperative methylprednisolone failed to show an overall significant benefit on the incidence of the composite primary study endpoint. There was, however, a benefit in patients undergoing palliative procedures and a significant interaction between treatment effect and center, suggesting that there may be center or patient characteristics that make prophylactic methylprednisolone beneficial.

Background: The potential for necrotizing enterocolitis (NEC) in neonates requiring cardiac surgery has contributed largely to wide feeding practice variations and a hesitation to initiate enteral feeding during the preoperative period, specifically those patients with hypoplastic left heart syndrome. Methods: A retrospective chart review of neonates undergoing cardiac surgery at a single institution between July 2011 and July 2013 was performed. The primary objective of this study was to determine if preoperative feeding was associated with NEC in neonates requiring cardiac surgery. Univariable and multivariable analyses were performed to evaluate the relationship between preoperative feeding and NEC. Secondary outcomes including growth failure, total ventilator days, total length of stay, and tube-assisted feeds at discharge were analyzed. Results: One hundred thirty consecutive neonates who required cardiac surgery were included in the analysis. Preoperative feeding occurred in 61% (n = 79). The overall prevalence of NEC was 9% (12/130), including three neonates with surgical NEC. There was no difference in the prevalence of NEC between the preoperative feeding and nil per os (NPO) groups. Preoperative NPO status was associated with longer ventilator-dependent days ( P = .01) but was not associated with worsened growth failure, longer length of stay, or increased prevalence of tube-assisted feeds at discharge. Conclusion: In this study cohort, preoperative feeding was associated with a low prevalence of NEC. Larger prospective studies evaluating the safety and benefits of preoperative feeding in cardiac neonates are warranted.

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.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).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.Preoperative oral feeding may potentially be a modifiable factor to help improve early transition to oral feeding.

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.

Growth failure is a widespread health problem in infants with congenital heart disease, specifically infants with single ventricle heart lesions, including hypoplastic left heart syndrome. Optimizing nutrition delivery for neonates undergoing cardiac surgery is challenging given environmental and physiologic barriers. Concomitant with nutrition delivery challenges, wide practice variations exist among clinicians. Malnutrition and poor growth are associated with poor wound healing, increased infection risk, prolonged hospitalizations, and long-term neurodevelopmental disability, including worse school performance. The cause of early postnatal growth failure is multifactorial and related to the delay in onset of enteral feedings, increased metabolic stress of cardiac surgery, cyanosis, low cardiac output, increased energy expenditure, altered splanchnic perfusion, and gastrointestinal morbidities. In addition, cardiac infants are susceptible to unique complications, including chylothorax, vocal cord dysmotility, and swallowing dysfunction.

ObjectiveChildren 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.MethodsData 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.ResultsAnnual 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%) (pConclusionDespite 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.

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.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 level20 ng/mL.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).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.

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.

Objective A heightened inflammatory response occurs after cardiac surgery. The perioperative use of glucocorticoids has been advocated as a method to improve postoperative outcomes. Randomized prospective studies to quantify the effect of methylprednisolone on perioperative outcomes in neonatal cardiac surgery have not been performed. We sought to determine whether preoperative methylprednisolone would improve postoperative recovery in neonates requiring cardiac surgery. Methods Neonates scheduled for cardiac surgery were randomly assigned to receive either 2-dose (8 hours preoperatively and operatively, n = 39) or single-dose (operatively, n = 37) methylprednisolone (30 mg/kg per dose) in a prospective double-blind trial. The primary outcome was the incidence of low cardiac output syndrome (standardized score) or death 36 hours postoperatively. Secondary outcomes were death at 30 days, interleukin-6 levels, inotropic score, fluid balance, serum creatinine, and intensive care unit and hospital stay. Results Preoperative plasma levels of the inflammatory cytokine interleukin-6 were reduced by 2-fold ( P .001) in the 2-dose methylprednisolone group, consistent with the anti-inflammatory effects of methylprednisolone. However, the incidence of low cardiac output syndrome was 46% (17/37) in the single-dose and 38% (15/39) in the 2-dose methylprednisolone groups ( P = .51). Two-dose methylprednisolone was associated with a higher serum creatinine (0.61 ± 0.18 mg/dL vs 0.53 ± 0.12 mg/dL, P = .03) and poorer postoperative diuresis (−96 ± 49 mL, P = .05). Inotropic requirement, duration of mechanical ventilation, intensive care unit, and hospital stay did not differ between the 2 groups. Conclusions Combined preoperative and intraoperative use of glucocorticoids in neonatal cardiac surgery does not favorably affect early clinical outcomes and may exacerbate perioperative renal dysfunction.

Purpose There has been reluctance to use intraoperative transesophageal echocardiography (TEE) in small infants. We assessed the utility and safety of a new miniaturized multiplane micro-TEE probe in small infants undergoing cardiac operations. Description Hemodynamic and ventilation variables were prospectively recorded before and after micro-TEE insertion and removal in infants weighing 5 kg or less undergoing cardiac operations. Evaluation The study included 42 patients with a mean weight of 3.6 ± 0.9 kg (range, 1.7 to 5 kg). All probe insertions were successful. There were no complications or clinically significant changes in hemodynamic or ventilation variables. Information provided by TEE resulted in surgical revision in 6 of the 42 patients. Conclusions The micro-TEE provides high quality, useful diagnostic images without hemodynamic or ventilation compromise in small infants undergoing cardiac operations. This advance is important with the growing trend towards complete repair of complex structural heart disease in small infants.

Extracardiac or genetic abnormalities (EGA) represent a factor in the morbidity of patients with congenital heart disease. We evaluated the way neonates with CHD are screened at our institution and determined the yield for the screening tests. We reviewed the charts of 223 neonates with structural CHD. Subjects were categorized into 6 groups: univentricular, left-sided obstructive lesions, right-sided obstructive lesions, septal defects, conotruncal defects (CTD), and other. We reviewed which patients underwent cranial ultrasonogram (CUS), abdominal ultrasonogram (AUS), and/or genetic studies (GS) as well as their results. There was a high prevalence of EGA in each group by CUS (32% to 42%), AUS (32% to 69%), and GS (10% to 60%). There was considerable variability in the proportion within each group that underwent screening tests, and the consistency of screening often was not congruent with the likelihood of abnormal results. Approximately 50% of our patients had ≥1 EGA identified, resulting in a cost-yield ratio of $4,508/patient with EGA. Screening for EGA at our institution is not uniform and is often at odds with the prevalence of such patients. Given the high prevalence of EGA, we advocate for a universal screening program for neonates with CHD using cranial/abdominal ultrasonography and genetic testing.

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.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%).Fifteen North American centers.Infants enrolled in the Single Ventricle Reconstruction trial.None.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).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.

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.

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.

Background We sought to characterize growth between birth and age 3 years in infants with hypoplastic left heart syndrome who underwent the Norwood procedure. Methods and Results We performed a secondary analysis using the Single Ventricle Reconstruction Trial database after excluding patients z score ( LAZ ) and weight‐for‐age z score ( WAZ ) at birth and age 3 years and change in WAZ over 4 clinically relevant time periods. We identified correlates of change in WAZ and LAZ using multivariable linear regression with bootstrapping. Mean WAZ and LAZ were below average relative to the general population at birth ( P P =0.05, respectively) and age 3 years ( P WAZ occurred between birth and Norwood discharge; the greatest gain occurred between stage II and 14 months. At age 3 years, WAZ and LAZ were WAZ differed among time periods. Shunt type was associated with change in WAZ only in the Norwood discharge to stage II period; subjects with a Blalock‐Taussig shunt had a greater decline in WAZ than those with a right ventricle‐pulmonary artery shunt ( P =0.002). Conclusions WAZ changed over time and the predictors of change in WAZ varied among time periods. By age 3 years, subjects remained small and three times as many children were short as were underweight (>2 SD below normal). Failure to find consistent risk factors supports the strategy of tailoring nutritional therapies to patient‐ and stage‐specific targets. Clinical Trial Registration URL: http://clinicaltrials.gov/ . Unique identifier: NCT00115934.

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.

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.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.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).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.

Growth faltering is common in children with hypoplastic left heart syndrome (HLHS) after the Norwood operation. We previously demonstrated that transplant-free survivors remained small at 3 years of age with mean weight-for-age z-scores (WAZ) and height-for-age z-scores (HAZ) below average. We

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 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.

Despite the emerging relevance of high-density lipoprotein (HDL) in the inflammatory cascade and vascular barrier integrity, HDL levels in children undergoing cardiac surgery are unexplored. As a measure of HDL levels, the HDL-cholesterol (HDL-C) in single-ventricle patients was quantified before and after the Fontan operation, and it was determined whether relationships existed between the duration and the type of postoperative pleural effusions. The study prospectively enrolled 12 children undergoing the Fontan operation. Plasma HDL-C levels were measured before and after cardiopulmonary bypass. The outcome variables of interest were the duration and type of chest tube drainage (chylous vs. nonchylous). The Kendall rank correlation coefficient and the Wilcoxon rank sum test were used. There were 11 complete observations. The median preoperative HDL-C level for all the subjects was 30 mg/dl (range, 24–53 mg/dl), and the median postcardiopulmonary bypass level was 21 mg/dl (range, 14–46 mg/dl) (p = 0.004). There was a tendency toward a moderate inverse correlation (–0.42) between the postcardiopulmonary bypass HDL-C level and the duration of chest tube drainage, but the result was not statistically significant (p = 0.07). In the chylous effusion group, the median postcardiopulmonary bypass HDL-C tended to be lower (16 vs. 23 mg/dl; p = 0.09). After the Fontan operation, the plasma HDL-C levels in children are significantly reduced. It is reasonable to conclude that the reduction in HDL-C reflects reduced plasma levels of HDL particles, which may have pertinent implications in postoperative pleural effusions given the antiinflammatory and endothelial barrier functions of HDL.

Low cardiac output syndrome (LCOS) and maximum vasoactive inotropic score (VIS) have been used as surrogate markers for early postoperative outcomes in pediatric cardiac surgery. The objective of this study was to determine the associations between LCOS and maximum VIS with clinical outcomes in neonatal cardiac surgery. This was a secondary retrospective analysis of a prospective randomized trial, and the setting was a pediatric cardiac intensive care unit in a tertiary care children’s hospital. Neonates (n = 76) undergoing corrective or palliative cardiac operations requiring cardiopulmonary bypass were prospectively enrolled. LCOS was defined by a standardized clinical criteria. VIS values were calculated by a standard formula during the first 36 postoperative hours, and the maximum score was recorded. Postoperative outcomes included hospital mortality, duration of mechanical ventilation, intensive care unit (ICU) and hospital lengths of stay (LOS), as well as total hospital charges. At surgery, the median age was 7 days and weight was 3.2 kg. LCOS occurred in 32 of 76 (42%) subjects. Median maximum VIS was 15 (range 5–33). LCOS was not associated with duration of mechanical ventilation, ICU LOS, hospital LOS, and hospital charges. Greater VIS was moderately associated with a longer duration of mechanical ventilation (p = 0.001, r = 0.36), longer ICU LOS (p = 0.02, r = 0.27), and greater total hospital costs (p = 0.05, r = 0.22) but not hospital LOS (p = 0.52). LCOS was not associated with early postoperative outcomes. Maximum VIS has only modest correlation with duration of mechanical ventilation, ICU LOS, and total hospital charges.

Background A modification to the Norwood procedure involving a right ventricle-to-pulmonary artery (RV-PA) shunt may improve early postoperative outcomes. Concerns remain about the effect of the right ventriculotomy required with this shunt on long-term ventricular function. Methods Between January 2000 and April 2005, 76 patients underwent the Norwood procedure, 35 with a modified Blalock-Taussig shunt (mBTS) and 41 with a RV-PA shunt. Patients were monitored until death or September 1, 2009, with an average follow-up of 6.8 years. Cardiac catheterization, echocardiograms, perioperative Fontan courses, and need for cardiac transplantation were compared between groups. Results Cumulative survival was 63% (22 of 35) in the mBTS group vs 78% (32 of 41) in the RV-PA group (p = 0.14). Pre-Fontan echocardiography revealed poorer ventricular function in RV-PA patients (p = 0.03). Cardiac transplantation was required in 6 of 32 (19%) patients with a prior RV-PA shunt vs 1 of 23 (4%) in the mBTS group (p = 0.06). This results in an almost identical cumulative transplant-free survival between groups; 60% (21 of 35) in the mBTS group and 63% (26 of 41) in the RV-PA group (p = 0.95). Conclusions Neither shunt offers a clear survival advantage through an average follow-up of 6.8 years. The RV-PA shunt results in impaired late ventricular function that may result in an increased need for cardiac transplantation.

This study aimed to identify the variables that influence parental treatment decisions after a prenatal diagnosis of congenital heart disease (CHD). The authors reviewed all cases of prenatally diagnosed structural CHD from August 1998 to December 2006 at their center. The following variables were studied as potential predictors of parental intent to treat: maternal age, race, insurance status, obstetric history, fetal gender, univentricular versus biventricular cardiac physiology, and fetal chromosomal abnormality. Uni- and multivariable logistic regression analyses were performed. In the review, 252 consecutive cases of prenatally diagnosed CHD were identified. Of these, 204 women pursued full medical treatment, whereas 25 women sought termination of pregnancy or comfort care. Parental intent to treat was unknown for 23 cases. Multivariable logistic regression analysis identified fetal chromosomal abnormality as the only variable that influenced parental intent to treat (odds ratio [OR], 14.33; 95% confidence interval [CI], 3.28–62.66; p = 0.0006). An associated chromosomal abnormality rather than the severity of the heart defect influences the decision to choose termination of pregnancy or comfort care for a fetus with prenatally diagnosed CHD. Women were 14 times more likely to terminate a pregnancy or seek comfort care for a fetus with CHD if a chromosomal abnormality was present.

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 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 .

Controversy still exists in the categorization of holes between the ventricles, although they are the most common congenital cardiac malformation. Advanced imaging techniques such as three-dimensional echocardiography and computed tomographic angiography offer superb anatomical details of these defects. In this review, we have sought to collate the features highlighted in different categorizations and identify their similarities, but also emphasize their differences. We hope that an analysis of this type, now achievable during life, using advanced imaging, might lead to the appearance of a unified system for diagnosis and description of holes between the ventricles.

ObjectiveThe objective of this study was to determine whether a composite outcome, derived of objective signs of inadequate cardiac output, would be associated with other important measures of outcomes and therefore be an appropriate end point for clinical trials in neonatal cardiac surgery.MethodsNeonates (n = 76) undergoing cardiac operations requiring cardiopulmonary bypass were prospectively enrolled. Patients were defined to have met the composite outcome if they had any of the following events before hospital discharge: death, the use of mechanical circulatory support, cardiac arrest requiring chest compressions, hepatic injury (2 times the upper limit of normal for aspartate aminotransferase or alanine aminotransferase), renal injury (creatinine >1.5 mg/dL), or lactic acidosis (an increasing lactate >5 mmol/L in the postoperative period). Associations between the composite outcome and the duration of mechanical ventilation, intensive care unit stay, hospital stay, and total hospital charges were determined.ResultsThe median age at the time of surgery was 7 days, and the median weight was 3.2 kg. The composite outcome was met in 39% of patients (30/76). Patients who met the composite outcome compared with those who did not had a longer duration of mechanical ventilation (4.9 vs 2.9 days, P

Emerging evidence suggests that altered vitamin D metabolism has a role in multiple disease processes including ischemia-reperfusion and inflammation. Vitamin D status and its association with outcomes have been unexplored in children with congenital heart disease or cardiac surgery and