This study explored the prospective use of the Ages and Stages Questionnaires-3 in follow-up after cardiac surgery.

For children undergoing cardiac surgery at 5 United Kingdom centres, the Ages and Stages Questionnaires-3 were administered 6 months and 2 years later, with an outcome based on pre-defined cut-points: Red = 1 or more domain scores >2 standard deviations below the normative mean, Amber = 1 or more domain scores 1–2 standard deviations below the normal range based on the manual, Green = scores within the normal range based on the manual.

From a cohort of 554 children <60 months old at surgery, 306 participated in the postoperative assessment: 117 (38.3%) were scored as Green, 57 (18.6%) as Amber, and 132 (43.1%) as Red. Children aged 6 months at first assessment (neonatal surgery) were likely to score Red (113/124, 85.6%) compared to older age groups (n = 32/182, 17.6%). Considering risk factors of congenital heart complexity, univentricular status, congenital comorbidity, and child age in a logistic regression model for the outcome of Ages and Stages score Red, only younger age was significant (p < 0.001). 87 children had surgery in infancy and were reassessed as toddlers. Of these, 43 (49.2%) improved, 30 (34.5%) stayed the same, and 13 (16.1%) worsened. Improved scores were predominantly in those who had a first assessment at 6 months old.

The Ages and Stages Questionnaires results are most challenging to interpret in young babies of 6 months old who are affected by complex CHD.

Extubation failure after neonatal cardiac surgery is associated with increased intensive care unit length of stay, morbidity, and mortality. We performed a quality improvement project to create and implement a peri-extubation bundle, including extubation readiness testing, spontaneous breathing trial, and high-risk criteria identification, using best practices at high-performing centers to decrease neonatal and infant extubation failure by 20% from a baseline of 15.7% to 12.6% over a 2-year period.

Utilising the transparency of the Pediatric Cardiac Critical Care Consortium database, five centres were identified as high performers, having better-than-expected neonatal extubation success rates with the balancing metric of as-expected or better-than-expected mechanical ventilation duration. Structured interviews were conducted with cardiac intensive care unit physician leadership at the identified centers to determine centre-specific extubation practices. Data from those interviews underwent qualitative content analysis which was used to develop a peri-extubation bundle. The bundle was implemented at a single-centre 17-bed cardiac intensive care unit. Extubation failure, defined as reintubation within 48 hours of extubation for anything other than a procedure, ventilator days and bundle compliance was tracked.

There was a 41.4% decrease in extubation failure following bundle implementation (12 failures of 76 extubations pre-implantation; 6 failures of 65 extubations post-implementation). Bundle compliance was 95.4%. There was no difference in ventilator days (p = 0.079) between groups.

Implementation of a peri-extubation bundle created from best practices at high-performing centres reduced extubation failure by 41.4% in neonates and infants undergoing congenital heart surgery.

Aortic arch obstruction and/or coarctation of aorta is uncommon (5–20%) in balanced atrioventricular septal defects. Although technically challenging, prenatal diagnosis of aortic arch obstruction in atrioventricular septal defect is critical for delivery planning and improves prenatal counselling regarding the timing of cardiac surgery. We sought to identify prenatal predictors of coarctation of aorta in atrioventricular septal defect.

Retrospective review of patients prenatally diagnosed with atrioventricular septal defect at two institutions.

Ninety-five fetuses with atrioventricular septal defect were identified and sufficient outcome data and diagnostic acoustic windows were available in 62. Six patients (10%) had coarctation of aorta after birth. Among the 38 patients with Trisomy 21, four (11%) had coarctation of aorta. On multivariable analysis, the proximal transverse aortic arch z score and ratio of left:right atrioventricular valve were independent predictors of coarctation of aorta with good interobserver reproducibility. Either proximal transverse aortic arch z score < −2, or ratio of left:right atrioventricular valve <0.7, predicted aortic arch obstruction with 100% sensitivity and 89% specificity.

Proximal transverse aortic arch z score and lower ratio of left:right atrioventricular valve diameter are independent predictors of postnatal coarctation of aorta in fetal patients with atrioventricular septal defect. The next step is the prospective application of these parameters to create an algorithm directing fetal counselling in terms of delivery location, and expected timing of surgical interventions.

To evaluate the influence of pre-existing maternal diabetes mellitus on fetal myocardial performance index and systolic-to-diastolic duration ratio.

Prospective cohort study included 179 pregnant women between 20 and 36w6d, divided into 3 groups: Group 1 (120, normal), Group 2 (31, type 1 diabetes mellitus), and Group 3 (28, type 2 diabetes mellitus). Systolic-to-diastolic duration ratio was calculated as the sum of isovolumic contraction time and ejection time divided by the sum of isovolumic relaxation time and ventricular filling time. Spectral Doppler was used to assess left ventricle systolic-to-diastolic duration ratio. Tissue Doppler was used to assess right ventricular filling time. Using spectral Doppler, left ventricle myocardial performance index was calculated as the sum of isovolumic contraction time and isovolumic relaxation time divided by ejection time.

Pre-existing maternal diabetes mellitus had a significant influence on fasting glucose levels (p < 0.001), left ventricle isovolumic contraction time (p < 0.001), left ventricle ejection time (p = 0.025), and left ventricle myocardial performance index (p < 0.001). Group 2 had higher left ventricle isovolumic contraction time (0.036 vs. 0.031 sec, p = 0.001) and left ventricle myocardial performance index (0.487 vs. 0.453, p = 0.003) compared with Group 1. Group 3 showed higher left ventricle myocardial performance index (0.492 vs. 0.449, p = 0.006) and lower left ventricle ejection time (0.161 vs. 0.169 sec, p = 0.038) than Group 1. Left ventricle systolic-to-diastolic duration (p = 0.704), right ventricle systolic-to-diastolic duration ratio’ (p = 0.757), left ventricle isovolumic contraction time (p = 0.163), left ventricle ejection time (p = 0.093), and left ventricle myocardial performance index (p = 0.087) were not useful parameters in predicting composite neonatal outcomes.

Pre-existing maternal diabetes mellitus had significant influence on fetal left ventricle myocardial performance index, but no effect on systolic-to-diastolic duration ratio. Systolic-to-diastolic duration ratio was not useful in predicting adverse perinatal outcomes.

Radiation exposure and the effect of anti-scatter grids are not well studied in premature infants during transcatheter patent ductus arteriosus closure.

This study aimed to investigate whether the use of anti-scatter grids altered the level of radiation exposure to premature infants undergoing transcatheter patent ductus arteriosus closure.

Demographic and radiation exposure data for premature infants who underwent transcatheter patent ductus arteriosus closure at the Stead Family Children’s Hospital from 10/2019 to 10/2021 were retrospectively reviewed and analyzed. Twenty-four patients (41%) underwent transcatheter patent ductus arteriosus closure with anti-scatter grids, while 34 (59%) underwent the procedure without using anti-scatter grids. At the time of catheterization, the median age, corrected age, and weight were 4.3 weeks (3.4–6), 29 weeks (28.1–30.9), and 1200g (1000-1600), respectively. Total radiation exposures for the dose area product and air kerma were 2.73 µGy.m2 (1.65–4.16), and 1.63 mGy (1.15–2.58), respectively. Radiation doses were higher in the group in whom the anti-scatter grids were utilized with dose area product of 3.33 µGy.m2 (2.39–5.43) and air kerma of 2.27 mGy (1.41–3.06) versus 1.86 µGy.m2 (1.46–3.60) and air kerma of 1.40 mGy (1.08–1.92). When radiation doses were adjusted to the radiation time, no difference in radiation exposure was noticed between the groups.

Transcatheter patent ductus arteriosus closure in premature infants can be safely performed with minimal radiation exposure. In the authors’ laboratory, the use of anti-scatter grids does not impact radiation exposure in premature infants.

Alternate electrocardiogram acquisition with fewer leads lacks systematic evaluation in children. This study aims to determine if electrocardiograms with fewer leads maintain diagnostic accuracy in paediatrics.

This is a single-centre review of 200 randomly selected standard 12-lead electrocardiograms from our hospital database (2017–2020) for patients aged 2 weeks to 21 years. An overlay technique generated 8-lead (limb + V1/V6) and 6-lead (limb only) variations of the 12-lead tracings, resulting in a total of 600 electrocardiograms, which were then interpreted by two independent paediatric electrophysiologists.

In total, 18% (35/200) of the baseline electrocardiograms were abnormal. Intervals were measured in lead II for all electrocardiograms. Comparing 12-lead to 6- and 8-lead electrocardiograms, there was almost perfect agreement for specific rhythm identification (97.5–100%, κ 0.85-1). The 8-lead showed substantial agreement with 12-lead electrocardiograms when identifying specific electrocardiogram patterns (97.5–100%, κ 0.66–1). A similar degree of agreement was not demonstrated with the 6-lead variant. Utilising the 12-lead electrocardiogram as the gold standard, sensitivity and specificity of the 8- and 6-lead electrocardiogram were > 89% for specific rhythm identification. Specificity for specific pattern recognition was > 99% while sensitivity was < 90% for certain variables for both 6- and 8-lead electrocardiogram, likely due to smaller sample size and fewer abnormal electrocardiograms. There was high percent reader agreement (92.5–100%).

8-lead electrocardiograms provide comparable diagnostic accuracy to 12-lead electrocardiograms for children. This information holds potential for future technological advancements in electrocardiogram acquisition tailored specifically for paediatrics. Additional studies are required to further refine conventional electrocardiogram acquisition.

Several organizations including the Environmental Protection Agency, World Health Organization and American Academy of Pediatrics recommend that hospital sound levels not exceed 45 decibels. Yet, several studies across multiple age groups have observed higher than recommended levels in the intensive care setting. Elevated sound levels in hospitals have been associated with disturbances in sleep, patient discomfort, delayed recovery, and delirium.

We measured sound levels in a pediatric cardiac intensive care unit and collected vital signs data, sedation dosing and delirium scores. During a 5-week study period, sound levels for 68 patients in 22 private and 4 semi-private rooms were monitored.

Sound levels were consistently above stated recommendations with an average daytime level of 50.6 decibels (maximum, 76.9 decibels) and an average nighttime level of 49.5 decibels (maximum, 69.6 decibels). An increase in average and maximum sound levels increased the probability of sedation administration the following hour (p-value < 0.001 and 0.01, respectively) and was predictive of an increase in heart rate and blood pressure (p-value < 0.001).

Sound levels in the CICU were consistently higher than recommended. An increase in heart rate, blood pressure and sedation utilization may suggest a stress response to persistent and sudden loud sounds. Given known negative impacts of excessive noise on stress, sleep, and brain development, as well as the similar adverse effects from the related use of sedative medications, reducing excessive and sudden noise may provide an opportunity to improve short- and long-term hemodynamic and neurodevelopmental outcomes in the pediatric cardiac intensive care unit.

Paediatric patients with heart failure requiring ventricular assist devices are at heightened risk of neurologic injury and psychosocial adjustment challenges, resulting in a need for neurodevelopmental and psychosocial support following device placement. Through a descriptive survey developed in collaboration by the Advanced Cardiac Therapies Improving Outcomes Network and the Cardiac Neurodevelopmental Outcome Collaborative, the present study aimed to characterise current neurodevelopmental and psychosocial care practices for paediatric patients with ventricular assist devices.

Members of both learning networks developed a 25-item electronic survey assessing neurodevelopmental and psychosocial care practices specific to paediatric ventricular assist device patients. The survey was sent to Advanced Cardiac Therapies Improving Outcomes Network site primary investigators and co-primary investigators via email.

Of the 63 eligible sites contacted, responses were received from 24 unique North and South American cardiology centres. Access to neurodevelopmental providers, referral practices, and family neurodevelopmental education varied across sites. Inpatient neurodevelopmental care consults were available at many centres, as were inpatient family support services. Over half of heart centres had outpatient neurodevelopmental testing and individual psychotherapy services available to patients with ventricular assist devices, though few centres had outpatient group psychotherapy (12.5%) or parent support groups (16.7%) available. Barriers to inpatient and outpatient neurodevelopmental care included limited access to neurodevelopmental providers and parent/provider focus on the child’s medical status.

Paediatric patients with ventricular assist devices often have access to neurodevelopmental providers in the inpatient setting, though supports vary by centre. Strengthening family neurodevelopmental education, referral processes, and family-centred psychosocial services may improve current neurodevelopmental/psychosocial care for paediatric ventricular assist device patients.

To examine feasibility, acceptability, and preliminary effectiveness of a novel group-based telemedicine psychoeducation programme aimed at supporting psychological well-being among adolescents with Fontan-palliated CHD.

A 5-week telemedicine psychoeducation group-based programme (WE BEAT) was developed for adolescents (N = 20; 13–18 years) with Fontan-palliated CHD aimed at improving resiliency and psychological well-being. Outcome measures included surveys of resilience (Connor–Davidson Resilience Scale), benefit finding (Benefit/Burden Scale for Children), depression, anxiety, peer relationships, and life satisfaction (National Institutes of Health Patient-Reported Outcomes Measurement Information System scales). Within-subject changes in these outcomes were compared pre- to post-intervention using Cohen’s d effect size. In addition, acceptability in the form of satisfaction measures and qualitative feedback was assessed.

Among eligible patients reached, 68% expressed interest in study participation. Of those consented, 77% have been scheduled for a group programme to date with 87% programme completion. Twenty adolescents (mean age 16.1 ± SD 1.6 years) participated across five WE BEAT group cohorts (range: 3–6 participants per group). The majority (80%) attended 4–5 sessions in the 5-session programme, and the median programme rating was a 9 out of 10 (10 = most favourable rating). Following WE BEAT participation, resiliency (d = 0.44) and perceptions of purpose in life increased (d = 0.26), while depressive symptoms reduced (d = 0.36). No other changes in assessed outcome measures were noted.

These findings provide preliminary support that a group-based, telemedicine delivered psychoeducation programme to support psychological well-being among adolescents with CHD is feasible, acceptable, and effective. Future directions include examining intervention effects across diverse centres, populations, and implementation methods.

In North America, less than 30% of children with complex CHD receive recommended follow-up for neurodevelopmental and psychosocial care. While rates of follow-up care at surgical centres have been described, little is known about similar services outside of surgical centres.

This cohort study used Maine Health Data Organization’s All Payer Claims Data from 2015 to 2019 to identify developmental and psychosocial-related encounters received by children 0–18 years of age with complex CHD. Encounters were classified as developmental, psychological, and neuropsychological testing, mental health assessment interventions, and health and behaviour assessments and interventions. We analysed the association of demographic and clinical characteristics of children and the receipt of any encounter.

Of 799 unique children with complex CHD (57% male, 56% Medicaid, and 64% rural), 185 (23%) had at least one developmental or psychosocial encounter. Only 13 children (1.6%) received such care at a surgical centre. Developmental testing took place at a mix of community clinics/private practices (39%), state-based programmes (31%), and hospital-affiliated clinics (28%) with most encounters billing Medicaid (86%). Health and behavioural assessments occurred exclusively at hospital-affiliated clinics, predominately with Medicaid claims (82%). Encounters for mental health interventions, however, occurred in mostly community clinics/private practices (80%) with the majority of encounters billing commercial insurance (64%).

Children with complex CHD in Maine access developmental and psychosocial services in locations beyond surgical centres. To better support the neurodevelopmental outcomes of their patients, CHD centres should build partnerships with these external providers.

Advances in surgical and perioperative care have reduced the need for prolonged mechanical ventilation in children following cardiac surgery.

To evaluate the feasibility and assess the clinical outcomes of an early extubation strategy in the operating room for children undergoing congenital heart surgery, including neonates (age < 28 days).

This is a retrospective analysis including congenital open-heart surgery cases. We excluded patients who remained open chest postoperatively or patients with severe hemodynamic instability and high inotropic support from the study. Study variables include age, gender, weight, preoperative cardiac diagnosis, preoperative diagnosis of genetic or chromosomal abnormalities, prematurity, preoperative mechanical ventilation (invasive or non-invasive), cardiopulmonary bypass time, circulatory arrest time, postoperative use of inotropes, duration of mechanical ventilation, postoperative respiratory complication, sepsis, bleeding or other complications, paediatric cardiac ICU stay, total hospital stay, incidence of failed extubation, and operative or postoperative mortality.

This study included 163 patients who underwent congenital open cardiac surgery, out of these studied patients, 118 (72.4%) were extubated in the operating room. In total, 19.6% of studied patients had Down syndrome. Other genetic or chromosomal disorders were present among 8.6% of studied patients. There was a statistically significant difference between operating room extubation group and non-operating room extubation group regarding Risk Adjustment for Congenital Heart Surgery Score classification, postoperative paediatric ICU duration, postoperative hospital length of stay, vasoactive-inotrope score, duration of inotrope, open chest, and mortality.

Extubation in the operating room after congenital open-heart surgery was successful in most of our patients, even following complex procedures.

Early recognition of cardiac involvement and prediction of disease prognosis are essential for the management of inflammatory diseases such as multisystem inflammatory syndrome. This study aimed to investigate the role of Pentraxin-3 levels in identifying cardiac involvement and evaluating disease severity in patients with multisystem inflammatory syndrome.

The study included 56 multisystem inflammatory syndrome patients and 26 healthy children as a control group. The multisystem inflammatory syndrome group was divided into those with cardiac involvement (n = 34) and those without (n = 22), as well as those with clinically mild-moderate (n = 30) and severe (n = 26) multisystem inflammatory syndrome. Blood samples for measurement of Pentraxin-3 levels were obtained from all patients before treatment and from the healthy controls.

In the patient group, the mean age was 8.2 ± 4 years (range: 2–17 years), and the male-to-female ratio was 1.8. In the control group, these values were 9.5 ± 3.7 years (range: 2–16 years) and 1.9, respectively (p > 0.05). Plasma Pentraxin-3 levels were significantly higher in multisystem inflammatory syndrome patients compared to controls (7.1 ± 5 ng/mL vs. 2.9 ± 2.1 ng/mL, p = 0.001). Patients with cardiac involvement had a significantly higher median Pentraxin-3 level than those without (5.8 ng/mL vs. 4.1 ng/mL, p = 0.004). Severe disease was also associated with a higher median Pentraxin-3 level compared to mild-moderate disease (6.1 ng/mL vs. 4.4 ng/mL, p = 0.001). Pentraxin-3 level was negatively correlated with left ventricular ejection fraction and positively correlated with B-type natriuretic peptide, troponin.

Elevated Pentraxin-3 levels in multisystem inflammatory syndrome patients may help predict the clinical course of the disease and cardiac involvement. However, larger-scale prospective studies are needed to further elucidate this.