The study of psychological well-being and related resilient outcomes is of increasing focus in cardiovascular research. Despite the critical importance of psychological well-being and related resilient outcomes in promoting optimal cardiac health, there have been very few psychological interventions directed towards children with heart disease. This paper describes the development and theoretical framework of the WE BEAT Wellbeing Education Program, a group-based psychoeducation and coping skills training intervention designed to improve psychological well-being and resilience in adolescents with paediatric heart disease.

Program development was informed by patient and family needs and input gathered via large, international survey methods as well as qualitative investigation, a theoretical framework, and related resilience intervention research.

An overview of the WE BEAT intervention components and structure of the programme is provided.

The WE BEAT Wellbeing Education Program was developed as one of the first resiliency-focused interventions in paediatric heart disease with an overall objective to foster positive psychological well-being and resilient outcomes through a health promotion and prevention lens in an accessible format while providing access to safe, peer-to-peer community building. Feasibility pilot results are forthcoming. Future directions include mobile app-based delivery and larger-scale efficacy and implementation trials.

There is little known about the spectrum of cardiac injury in acute COVID-19 infection in children.

A single-centre, retrospective chart analysis was performed. The protocol was deemed IRB exempt. All patients under the age of 21 years admitted from 20 March, 2020 to 22 June, 2021 for acute symptomatic COVID-19 infection or clinical suspicion of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 were included. Past medical history, lab findings, echocardiogram and electrocardiogram/telemetry findings, and clinical outcomes were reviewed.

Sixty-six patients with MIS-C and 178 with acute COVID-19 were reviewed. Patients with MIS-C had more cardiac testing than those with acute COVID-19. Inflammatory markers were more likely elevated, and function was more likely abnormal on echocardiogram in those with MIS-C with testing performed. Among patients with MIS-C, 17% had evidence of coronary dilation versus 0% in the acute COVID-19 group. One (0.6%) patient with acute COVID-19 had clinically significant electrocardiogram or telemetry findings, and this was in the setting of prior arrhythmias and CHD. Four (6%) patients with MIS-C had clinically significant findings on electrocardiogram or telemetry. Among patients with acute COVID-19, extracorporeal membrane oxygenation support was required in 0.6% of patients with acute COVID-19, and there was a 2.8% mortality. There were no deaths in the setting of MIS-C.

Patients with acute COVID-19 and clinical suspicion of cardiac injury had a lower incidence of abnormal laboratory findings, ventricular dysfunction, or significant arrhythmia than those with MIS-C.

This single-centre, retrospective cohort study was conducted to investigate the predictors of early peritoneal dialysis initiation in newborns and young infants undergoing cardiac surgery.

There were fifty-seven newborns and young infants. All subjects received peritoneal dialysis catheter after completion of the cardiopulmonary bypass. Worsening post-operative (post-op) positive fluid balance and oliguria (<1 ml/kg/hour) despite furosemide were the clinical indications to start early peritoneal dialysis (peritoneal dialysis +). Demographic, clinical, and laboratory data were collected from the pre-operative, intra-operative, and immediately post-operative periods.

Baseline demographic data were indifferent except that peritoneal dialysis + group had more newborns. Pre-operative serum creatinine was higher for peritoneal dialysis + group (p = 0.025). Peritoneal dialysis + group had longer cardiopulmonary bypass time (p = 0.044), longer aorta cross-clamp time (p = 0.044), and less urine output during post-op 24 hours (p = 0.008). In the univariate logistic regression model, pre-op serum creatinine was significantly associated with higher odds of being in peritoneal dialysis + (p = 0.021) and post-op systolic blood pressure (p = 0.018) and post-op mean arterial pressure (p=0.001) were significantly associated with reduced odds of being in peritoneal dialysis + (p = 0.018 and p = 0.001, respectively). Post-op mean arterial pressure showed a statistically significant association adjusted odds ratio = 0.89, 95% confidence interval [0.81, 0.96], p = 0.004) with peritoneal dialysis + in multivariate analysis after adjusting for age at surgery.

In our single-centre cohort, pre-op serum creatinine, post-op systolic blood pressure, and mean arterial pressure demonstrated statistically significant association with peritoneal dialysis +. This finding may help to better risk stratify newborns and young infants for early peritoneal dialysis start following cardiac surgery.

The COVID-19 pandemic which has devastated the whole world for the past 3 years affects different patient groups differently. This study aims to evaluate the prevalence, symptoms, and severity of COVID-19 infection, vaccination status, and cardiac pathologies of children who exercise.

The records of the children and adolescents who applied to our paediatric cardiology outpatient clinic for preparticipation examinations between 01.01.22 and 31.12.2022 were scanned retrospectively, and information about their COVID-19 history, the severity of infection, symptoms during the infection, at the time of the examination, and vaccination status was obtained. The results were analysed using MS Excel 2016 software.

The study consisted of 240 children [82 (34.17%) girls and 158 (65.83%) boys] whose mean age was 12.64 ± 2.64 years, mean weight was 50.03 ± 15.53 kg, mean height was 157 ± 15.09 cm, and mean body mass index was 19.65 ± 3.59. 129 cases had a COVID-19 history, 74 cases had no COVID-19 history, and 37 only had contact but no polymerase chain reaction positivity. 84 cases were mild, 19 were moderate, and 12 were asymptomatic. The most common symptoms were fatigue, malaise, headache, sore throat, and fever. 51 cases (35.15%) were vaccinated against COVID-19. No significant cardiac pathologies were detected in electrocardiography or echocardiography

This study shows that COVID-19 infections in children who exercise are generally mild and self-limiting. Our findings suggest that exercise may have positive effects on immunity.

The safety of early post-operative cardiac catheterisation has been described following congenital heart surgery. Optimal timing of early post-operative cardiac catheterisation remains uncertain. The aim of this study was to describe the safety of early post-operative cardiac catheterisation and its impact on cardiac ICU and hospital length of stay, duration of mechanical ventilation, and extracorporeal support.

This single-centre retrospective cohort study compared clinical and outcome variables between “early” early post-operative cardiac catheterisation (less than 72 hours after surgery) and “late” early post-operative cardiac catheterisation (greater than 72 hours after surgery) groups using Chi-squared, Student’s t, and log-rank test (or appropriate nonparametric test).

In total, 132 patients were included, 22 (16.7%) “early” early post-operative cardiac catheterisation, and 110 (83.3%) “late” early post-operative cardiac catheterisation. Interventions were performed in 63 patients (51.5%), 7 (11.1%) early and 56 (88.9%) late. Complications of catheterisation occurred in seven (5.3%) patients, two early and five late. There were no major complications. Patients in the late group trended towards a longer stay in the cardiac ICU (19 days [7, 62] versus 11.5 days [7.2, 31.5], p = 0.6) and in the hospital (26 days [9.2, 68] versus 19 days [13.2, 41.8], p = 0.8) compared to the earlier group.

“Early” early post-operative cardiac catheterisation was associated with an overall low rate of complications. Earlier catheterisations trended towards shorter cardiac ICU and hospital length of stays. Earlier catheterisations may lead to earlier recovery for patients not following an expected post-operative course.

We have left antegrade pulmonary blood flow (APBF) at bidirectional cavopulmonary shunt (BCPS) only for high-risk patients. This study evaluates the indication and the outcomes of patients with APBF, compared to those without APBF.

Patients with APBF after BCPS were identified among patients who underwent BCPS between 1997 and 2022. Outcomes of patients with and without APBF after BCPS were compared.

APBF was open in 38 (8.2%) of 461 patients. Median age (7.7 versus 6.3 months, p = 0.55) and weight (5.6 versus 6.1 kg, p = 0.75) at BCPS were similar in both groups. The most frequent indication for APBF was high pulmonary artery pressure (PAP) in 14 patients, followed by hypoxaemia in 10, and hypoplastic left pulmonary artery in 8. The source of APBF was the pulmonary trunk in 10 patients and the aortopulmonary shunt in 28. Median hospital stay after BCPS was longer (22 versus 14 days, p = 0.018) and hospital mortality was higher (10.5 versus 2.1%, p = 0.003) in patients with APBF compared to those without APBF. However, 448 hospital survivors showed similar survival after discharge following BCPS (p = 0.224). Survival after total cavopulmonary connection (TCPC) was similar between the groups (p = 0.753), although patients with APBF were older at TCPC compared to those without (3.9 versus 2.2 years, p = 0.010).

APBF was left in 8% following BCPS in high-risk patients, mainly due to preoperative high PAP. Hospital survivors after BCPS demonstrated comparable survival in patients with and without APBF. Adding APBF at BCPS might be a useful option for high-risk patients.

Challenges to communication between families and care providers of paediatric patients in intensive care units (ICU) include variability of communication preferences, mismatched goals of care, and difficulties carrying forward family preferences from provider to provider. Our objectives were to develop and test an assessment tool that queries parents of children requiring cardiac intensive care about their communication preferences and to determine if this tool facilitates patient-centred care and improves families’ ICU experience.

In this quality improvement initiative, a novel tool was developed, the Parental Communication Assessment (PCA), which asked parents with children hospitalised in the cardiac ICU about their communication preferences. Participants were prospectively randomised to the intervention group, which received the PCA, or to standard care. All participants completed a follow-up survey evaluating satisfaction with communication.

One hundred thirteen participants enrolled and 56 were randomised to the intervention group. Participants who received the PCA preferred detail-oriented communication over big picture. Most parents understood the daily discussions on rounds (64%) and felt comfortable expressing concerns (68%). Eighty-six percent reported the PCA was worthwhile. Parents were generally satisfied with communication. However, an important proportion felt unprepared for difficult decisions or setbacks, inadequately included or supported in decision-making, and that they lacked control over their child’s care. There were no significant differences between the intervention and control groups in their communication satisfaction results.

Parents with children hospitalised in the paediatric ICU demonstrated diverse communication preferences. Most participants felt overall satisfied with communication, but individualising communication with patients’ families according to their preferences may improve their experience.

Catheterisation is the gold standard used to evaluate pulmonary blood flow in patients with a Blalock–Thomas–Taussig shunt. It involves risk and cannot be performed frequently. This study aimed to evaluate if echocardiographic measurements obtained in a clinical setting correlate with catheterisation-derived pulmonary blood flow in patients with a Blalock–Thomas–Taussig shunt as the sole source of pulmonary blood flow.

Chart review was performed retrospectively on consecutive patients referred to the catheterisation lab with a Blalock–Thomas–Taussig shunt. Echocardiographic parameters included peak, mean, and diastolic gradients across the Blalock–Thomas–Taussig shunt and forward and reverse velocity time integral across the distal transverse aorta. In addition to direct correlations, we tested a previously published formula for pulmonary blood flow calculated as velocity time integral across the shunt × heart rate × Blalock–Thomas–Taussig shunt area. Catheterisation parameters included pulmonary and systemic blood flow as calculated by the Fick principle.

18 patients were included. The echocardiography parameters and oxygen saturation did not correlate with catheterisation-derived pulmonary blood flow, systemic blood flow, or the ratio of pulmonary to systemic blood flow. As the ratio of reverse to forward velocity time integral across the transverse aorta increased, the probability of shunt stenosis decreased.

Echocardiographic measurements obtained outside the catheterisation lab do not correlate with catheterisation-derived pulmonary blood flow. The ratio of reverse to forward velocity time integral across the transverse aortic arch may be predictive of Blalock–Thomas–Taussig shunt narrowing; this finding should be investigated further.

The aim of this study is to evaluate the role of leukotriene B4, an inflammatory mediator, in the development of pulmonary hypertension in paediatric patients with CHD with left-right shunt.

The study included forty patients with CHD with left-right shunts. Based on haemodynamic data obtained from cardiac diagnostic catheterisation, 25 patients who met the criteria for pulmonary arterial hypertension were included in the patient group. The control group comprised 15 patients who did not meet the criteria. The standard cardiac haemodynamic study was conducted. Leukotriene B4 levels were assessed in blood samples taken from both pulmonary arteries and peripheral veins.

The median age of patients with pulmonary arterial hypertension was 10 months (range: 3–168), while the median age of the control group was 50 months (range: 3–194). In the pulmonary hypertension group, the median pulmonary artery systolic/diastolic/mean pressures were 38/18/24 mmHg, compared to 26/10/18 mmHg in the control group. Leukotriene B4 levels in pulmonary artery blood samples were significantly higher in the pulmonary arterial hypertension group compared to the controls (p < 0.05). Peripheral leukotriene B4 levels were also elevated in the pulmonary arterial hypertension group in comparison to the control group, though the difference was not statistically significant.

The discovery of elevated leukotriene B4 levels in pulmonary artery samples from paediatric patients with pulmonary arterial hypertension secondary to CHD with left-to-right shunt suggests that local inflammation may have a pathological role in the development of pulmonary arterial hypertension.

Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital heart surgery database worldwide but does not provide information beyond primary episode of care. Linkage to hospital electronic health records would capture complications and comorbidities along with long-term outcomes for patients with CHD surgeries. The current study explores linkage success between Society of Thoracic Surgeons Congenital Heart Surgery Database and electronic health record data in North Carolina and Georgia.

The Society of Thoracic Surgeons Congenital Heart Surgery Database was linked to hospital electronic health records from four North Carolina congenital heart surgery using indirect identifiers like date of birth, sex, admission, and discharge dates, from 2008 to 2013. Indirect linkage was performed at the admissions level and compared to two other linkages using a “direct identifier,” medical record number: (1) linkage between Society of Thoracic Surgeons Congenital Heart Surgery Database and electronic health records from a subset of patients from one North Carolina institution and (2) linkage between Society of Thoracic Surgeons data from two Georgia facilities and Georgia’s CHD repository, which also uses direct identifiers for linkage.

Indirect identifiers successfully linked 79% (3692/4685) of Society of Thoracic Surgeons Congenital Heart Surgery Database admissions across four North Carolina hospitals. Direct linkage techniques successfully matched Society of Thoracic Surgeons Congenital Heart Surgery Database to 90.2% of electronic health records from the North Carolina subsample. Linkage between Society of Thoracic Surgeons and Georgia’s CHD repository was 99.5% (7,544/7,585).

Linkage methodology was successfully demonstrated between surgical data and hospital-based electronic health records in North Carolina and Georgia, uniting granular procedural details with clinical, developmental, and economic data. Indirect identifiers linked most patients, consistent with similar linkages in adult populations. Future directions include applying these linkage techniques with other data sources and exploring long-term outcomes in linked populations.

The left ventricular outflow tract is an important source of ventricular arrhythmias. Up to one-third of all idiopathic ventricular arrhythmias in patients with structurally normal hearts may arise from this region. We would like to share the results of our left ventricular outflow tract ablation using three-dimensional mapping and limited fluoroscopy.

This is a single-centre retrospective cohort study. Forty-six consecutive patients who underwent left ventricular outflow tract ablation procedures between January 2015 and June 2023 were included in the study. The EnSite Precision System (Abbott, St. Paul, MN, USA) was used to facilitate mapping and to reduce or eliminate the need for fluoroscopy.

The study group comprised 29 males and 17 females, with a mean age of 13.4 ± 4.5 years. The most common location for arrhythmias was the left coronary cusp (n : 21). Other locations, in sequence, included the junction of the right and left coronary commissure (n : 10), right coronary cusp (n : 10), left ventricular outflow tract endocardium (n:4), aorto-mitral junction (n : 1), and great cardiac vein (n : 1). Nine of these patients had previously undergone unsuccessful right ventricular outflow tract ablation at another centre. Cryoablation was performed in three patients, irrigated radiofrequency ablation in three patients, and conventional radiofrequency ablation in the remaining patients. The acute success rate was 100%, and no recurrences were observed. The mean follow-up period was 49.6 ± 24.4 months. All patients were asymptomatic and were being followed without antiarrhythmic medication.

Although left ventricular outflow tract ablations pose a risk for coronary artery and heart valve complications, they can be performed successfully and safely with the guidance of three-dimensional mapping.

The aim of this study was to review our institution’s experience with truncus arteriosus from prenatal diagnosis to clinical outcome.

and results: We conducted a single-centre retrospective cohort study for the years 2005–2020. Truncus arteriosus antenatal echocardiographic diagnostic accuracy within our institution was 92.3%. After antenatal diagnosis, five parents (31%) decided to terminate the pregnancy. After inclusion from referring hospitals, 16 patients were offered surgery and were available for follow-up. Right ventricle-to-pulmonary artery continuity was preferably established without the use of a valve (direct connection), which was possible in 14 patients (88%). There was no early or late mortality. Reinterventions were performed in half of the patients at latest follow-up (median follow-up of 5.4 years). At a median age of 5.5 years, 13 out of 14 patients were still without right ventricle-to-pulmonary artery valve, which was well tolerated without signs of right heart failure. The right ventricle demonstrated preserved systolic function as expressed by tricuspid annular plane systolic excursion z-score (−1.4 ± 1.7) and fractional area change (44 ± 12%). The dimensions and function of the left ventricle were normal at latest follow-up (ejection fraction 64.4 ± 6.2%, fractional shortening 34.3 ± 4.3%).

This study demonstrates good prenatal diagnostic accuracy of truncus arteriosus. There was no mortality and favourable clinical outcomes at mid-term follow-up, with little interventions on the right ventricle-to-pulmonary artery connection and no right ventricle deterioration. This supports the notion that current perspectives of patients with truncus arteriosus are good, in contrast to the poor historic outcome series. This insight can be used in counselling and surgical decision-making.

Haemodynamic changes in caval venous flow distribution occurring during bidirectional cavopulmonary anastomosis operation are still largely unknown.

Transit time flow measurements were performed in 15 cavopulmonary anastomosis operations. Superior and inferior caval vein flows were measured before and after the cavopulmonary anastomosis. Ratio of superior caval vein to overall caval veins flow was calculated.

Mean superior caval vein flow ratio before cavopulmonary anastomosis was higher than previously reported for healthy children. Superior caval vein flow ratio decreased in 14/15 patients after cavopulmonary anastomosis: mean 0.63 ± 0.12 before versus 0.43 ± 0.14 after. No linear correlation between intraoperative superior caval vein pressure and superior caval vein flow after cavopulmonary anastomosis was found. Neither Nakata index nor pulmonary vascular resistance measured at preoperative cardiac catheterisation correlated with intraoperative flows. None of patients died or required a take down.

The higher mean superior caval vein flow ratio before cavopulmonary anastomosis compared to healthy children suggests flow redistribution in univentricular physiology to protect brain and neurodevelopment. The decrease of superior caval vein flow ratio after cavopulmonary anastomosis may reflect the flow redistribution related to trans-pulmonary gradient. The lack of correlation between superior caval vein pressure and superior caval vein flow could be explained by limited sample size and multifactorial determinants of caval veins flow, although pressure remain essential. Larger sample of measurements are needed to find flow range potentially predictive for clinical failure. To authors’ knowledge, this is the first intraoperative flow measurement of both caval veins during cavopulmonary operations.

Postural orthostatic tachycardia syndrome is a debilitating disorder. We compared paediatric patients with this dysautonomia presenting with and without peak upright heart rate > 100 beats per minute.

Subjects were drawn from the Postural Orthostatic Tachycardia Syndrome Program database of the Children’s Hospital of Philadelphia diagnosed between 2007 and 2018. Subjects were aged 12–18 years at diagnosis with demographic data, supine and peak heart rate from 10-minute stand, symptoms, and family history. Patients were divided into “low heart rate” (peak less than 100 beats/minute) and “high heart rate” (peak at least 100 beats/minute) groups.

In total, 729 subjects were included (low heart rate group: 131 patients, high heart rate group: 598 patients). The low heart rate group had later age at diagnosis (16.1 versus 15.7, p = 0.0027). Median heart rate increase was 32 beats/minute in the low heart rate group versus 40 beats/minute in the high heart rate group (p < 0.00001). Excluding palpitations and tachypalpitations, there were no differences in symptom type or frequency between groups.

Paediatric patients meeting heart rate criteria for postural orthostatic tachycardia syndrome but without peak heart rate > 100 demonstrate no difference in symptom type or frequency versus those who meet both criteria. Differences observed reached statistical significance due to population size but are not clinically meaningful. This suggests that increased heart rate, but not necessarily tachycardia, is seen in these patients, supporting previous findings suggesting maximal heart rate is not a major determinant of symptom prevalence in paediatric postural orthostatic tachycardia syndrome.

In this study, we describe our experience utilising Advisor™ High Density (HD) Grid mapping catheter in transcatheter ablation of intraatrial re-entrant and focal atrial tachycardias with or without CHD.

Forty-five consecutive patients with intraatrial re-entrant and focal atrial tachycardia who underwent a transcatheter ablation procedure by using Advisor™ HD Grid mapping catheter and high-density mapping system in our hospital from January 2017 to January 2023 were included into the study.

The mean age of the patients was 14.2 ± 7.3 years (6–32 years), and the mean weight was 48.3 ± 16.2 kg (22–83 kg). Of the total 45 patients, 21 were intraatrial re-entrant tachycardia and 25 were focal atrial tachycardia. Of the 21 re-entrant circuits, 15 were classified as cavotricuspid isthmus-dependent and 5 were non-cavotricuspid isthmus-dependent. In one patient, two re-entrant circuits were identified. A transbaffle ablation was successfully performed from the left atrium in one patient. Of the 25 focal atrial tachycardia, 19 were from right atrium and 6 were from left atrium. A cryoablation was performed in only one patient and radiofrequency ablation in others. The mean procedure time was 180 ± 64 minutes. The mean follow-up period was 69.3 ± 35.3 months. Acute success was 95.5%. Recurrence was noted in two patients (4.4%).

Advisor™ HD Grid mapping catheter was found to be safe and achieved an acceptable success in transcatheter ablation of patients with intraatrial re-entrant tachycardia and focal atrial tachycardias.

Monitoring cerebral and renal near-infrared spectroscopy for regional venous oxygenation is a common practice in the postoperative care of neonates recovering from surgery for CHD. In this study, we aimed to test the feasibility of using this technology for monitoring changes in splanchnic perfusion during feeds in infants recovering from cardiac surgery.

We monitored renal and splanchnic near-infrared spectroscopy in 29 neonates once recovered from the critical postoperative state and tolerating full enteral nutrition. Infants were tested over 3 feeds for splanchnic regional oxygenation (rO2), arterial to splanchnic saturation difference and splanchnic to renal regional oxygenation ratio.

Splanchnic regional oxygenation data were obtained with no failure or interruptions. Interclass correlation for agreement between measurements suggested good repeatability: 0.84 at baseline and 0.82 at end of feed. Infants with physiologic repair (n = 19) showed a trend towards increased splanchnic regional oxygenation at the end of feeds and were more likely to achieve regional oxygenation > 50% compared to infants with shunt-dependent circulation (n = 10, p = 0.02). Calculating AVO2 and regional oxygenation index did not result in improved test sensitivity.

Monitoring splanchnic regional oxygenation during feeds for infants recovering from congenital heart surgery is feasible and reliable. These results suggest that near-infrared spectroscopy could be further studied as a tool for bedside monitoring to assist in feeding management and prevention of necrotising enterocolitis in this sensitive patient population.