A standardised multi-site approach to manage paediatric post-operative chylothorax does not exist and leads to unnecessary practice variation. The Chylothorax Work Group utilised the Pediatric Critical Care Consortium infrastructure to address this gap.

Over 60 multi-disciplinary providers representing 22 centres convened virtually as a quality initiative to develop an algorithm to manage paediatric post-operative chylothorax. Agreement was objectively quantified for each recommendation in the algorithm by utilising an anonymous survey. “Consensus” was defined as ≥ 80% of responses as “agree” or “strongly agree” to a recommendation. In order to determine if the algorithm recommendations would be correctly interpreted in the clinical environment, we developed ex vivo simulations and surveyed patients who developed the algorithm and patients who did not.

The algorithm is intended for all children (<18 years of age) within 30 days of cardiac surgery. It contains rationale for 11 central chylothorax management recommendations; diagnostic criteria and evaluation, trial of fat-modified diet, stratification by volume of daily output, timing of first-line medical therapy for “low” and “high” volume patients, and timing and duration of fat-modified diet. All recommendations achieved “consensus” (agreement >80%) by the workgroup (range 81–100%). Ex vivo simulations demonstrated good understanding by developers (range 94–100%) and non-developers (73%–100%).

The quality improvement effort represents the first multi-site algorithm for the management of paediatric post-operative chylothorax. The algorithm includes transparent and objective measures of agreement and understanding. Agreement to the algorithm recommendations was >80%, and overall understanding was 94%.

Infants who require open heart surgery are at increased risk for developmental delays including gross motor impairments which may have implications for later adaptive skills and cognitive performance. We sought to evaluate the feasibility and efficacy of a tummy time intervention to improve motor skill development in infants after cardiac surgery.

Infants <4 months of age who underwent cardiac surgery were randomly assigned to tummy time with or without outpatient reinforcement or standard of care prior to hospital discharge. The Alberta Infant Motor Scale (AIMS) was administered to each infant prior to and 3 months after discharge. Groups were compared, and the association between parent-reported tummy time at home and change in motor scores at follow-up was examined.

Parents of infants (n = 64) who had cardiac surgery at a median age of 5 days were randomly assigned to tummy time instruction (n = 20), tummy time + outpatient reinforcement (n = 21) or standard of care (n = 23). Forty-nine (77%) returned for follow-up. At follow-up, reported daily tummy time was not significantly different between groups (p = 0.17). Fifteen infants had <15 minutes of tummy time daily. Infants who received >15 minutes of tummy time daily had a significantly greater improvement in motor scores than infants with <15 minutes of tummy time daily (p = 0.01).

In infants following cardiac surgery, <15 minutes of tummy time daily is associated with increased motor skill impairment. Further research is needed to elucidate the best strategies to optimise parental compliance with tummy time recommendations.

Children with congenital heart disease (CHD) are at risk for psychological challenges, including internalising (e.g., depression, anxiety) and externalising (e.g., aggression, inattention) problems. The present study aimed to investigate the development of psychological concerns in early childhood by identifying predictors of behavioural and emotional problems in toddlers with CHD.

Children with CHD who were seen for neurodevelopmental (ND) evaluation at 12 ± 3 months of age, who completed the Bayley Scales of Infant Development–III (BSID-III) and whose parents completed the Child Behavior Checklist (CBCL), a standardised measure of emotional/behavioural problems at age 24–36 months, were included in the study (n = 144). CBCL scores were compared to test norms and classified as normal or abnormal. A classification tree was used to assess the association between CBCL scores and demographic and clinical variables.

Multi-variable tree analyses revealed lower BSID-III language composite scores at age 9–15 months predicted clinical CBCL internalising (p < 0.001), externalising (p = 0.004) and total scores (p < 0.001) at age 24–36 months. Lower maternal education levels also predicted clinical CBCL internalising (p < 0.0001), externalising (p < 0.001) and total scores (p < 0.0001).

Lower language abilities and lower maternal education predict increased behavioural and emotional problems in toddlers with CHD. These risk factors should be considered during routine ND evaluations to allow for earlier identification of children with CHD and their families who may benefit from psychological support.

Vesicoureteral reflux is a prominent congenital anomaly of the kidney and the urinary tract. Further, renal scarring is known to be related to chronic inflammation. However, there have been limited studies to date regarding the cardiovascular consequences of vesicoureteral reflux.

The aim of this study is to evaluate the possible subclinical atherosclerosis and cardiovascular complications in children with vesicoureteral reflux.

Patients with vesicoureteral reflux and age matched healthy controls were prospectively included in this case-control study. Patients were divided into two groups concerning renal scarring status. To assess cardiac functions, carotid artery intima media, epicardial adipose tissue, and periaortic adipose tissue thicknesses were evaluated.

There were 50 patients with vesicoureteral reflux; 26 patients without renal scarring and 24 patients with renal scarring, as well as 40 healthy controls. Myocardial performance indexes (Tei indexes) measured by tissue Doppler echocardiography from septum and left ventricle were significantly increased in study group (for all, p < 0.001). Also, intima media, epicardial adipose tissue, and periaortic adipose tissue thicknesses of the study groups were significantly higher than the control group (for all, p < 0.001). However, no statistical difference was observed between renal scarring (−) and renal scarring (+) groups.

Results of our study showed early deterioration of cardiac systolic and diastolic functions in children with vesicoureteral reflux regardless of renal scarring. Also, diagnosis of vesicoureteral reflux is an important risk factor for subclinical atherosclerosis, independent of renal scarring, which should be considered in the follow-up of these patients.

The aim of the study was to revise our more recent experience about epicardial posterior-septal accessory pathways radiofrequency transcatheter ablation in children and young patients using a transvenous approach through the coronary sinus, to understand if new mapping and ablation technologies can increase success rate and safety.

Twenty children (mean age 13 ± 3 years) with epicardial posterior-septal accessory pathways (14 in coronary sinus and 6 in the middle cardiac vein) underwent radiofrequency transcatheter ablation with CARTO-3® system with help of the CARTO-Univu® module. Acute success rate was 73%. No patient was lost to follow-up (mean time 11.4 ± 9 months). The recurrence rate was 19%. Two patients underwent a successful redo-procedure; the overall long-term success rate was 65%. Navistar® catheter presented the highest acute success rate in the coronary sinus. Navistar SmartTouch® was the only catheter that did not present recurrences after the acute success, and it was successfully used in two patients previously unsuccessfully treated with a Navistar ThermoCool®. Acute success rate was 79% without image integration with angio-CT, while it was 63% after the introduction of CARTO-Merge®.

Epicardial posterior-septal accessory pathways can be definitively eliminated by transvenous radiofrequency transcatheter ablation in more than half of the cases in children. Acute success rate does not seem to depend on catheters used, but contact-force catheter seems to be useful in cases with recurrences. Image integration with cardiac-CT does not increase success rate, but it is useful to detect coronary sinus alterations to better guide ablation strategy.

Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure.

A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included.

Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation.

A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.

Paediatric obesity is a worldwide health burden, with growing evidence linking obesity to myocardial function impairments. The study aims to evaluate left ventricular functions among prepubertal obese children to obesity-related clinical and metabolic parameters.

Between June 2019 and March 2020, 40 prepubertal children with obesity were recruited and compared to 40 healthy controls. Patients were assessed for body mass index z scores, waist circumference, body adiposity by bioimpedance analysis, and obesity-related laboratory tests, for example, serum chemerin. Left ventricular functions were assessed using variable echocardiographic modalities, such as M-mode, tissue Doppler, and two-dimensional speckle tracking.

Mean patients’ age was 9.25 ± 1.05 years. Left ventricular mass index, E/E’, and myocardial performance index were significantly increased in obese children than controls. Although M-mode-derived ejection fraction was comparable in both groups, two-dimensional speckle tracking-derived ejection fraction, global longitudinal strain, and global circumferential strain were significantly lower in cases than controls. Left ventricular mass index displayed a positive correlation with body mass index z score (p = 0.003), fat mass index (p = 0.037), and trunk fat mass (p = 0.021). Global longitudinal strain was negatively correlated with body mass index z score (p = 0.015) and fat mass index (p = 0.016). Serum chemerin was positively correlated with myocardial performance index (p = 0.01).

Alterations of left ventricular myocardial functions in prepubertal obese children could be detected using different echocardiographic modalities. Chemerin, body mass index z score, fat mass index, and trunk fat mass were correlated with subclinical left ventricular myocardial dysfunction parameters before puberty. Our results reinforce early and strict management of childhood obesity upon detection of changes in anthropometric and body adiposity indices.

In this retrospective single centre cohort study, hybrid PDA stenting was attempted in 11 patients with “flip technique”, between January 2020 and February 2021, and was successful in 10. Median age was 29 days (interquartile range 17.5–87) and mean weight 3.37 ± 1.23 kg. Mean fluoroscopy time was 13.58 ± 5.35 minutes, mean procedure time was 48.50 ± 22.5 minutes, and mean radiation dose was 1719.5 ± 1217.6 mGycm2. Mean time for cutdown was 9.9 ± 2.4 minutes and for haemostasis and suturing was 25.3 ± 11.0 minutes. Median duration of ventilation post-stenting was 26 hours (interquartile range 21–43.75). The median ICU stay post-procedure was 5 days (interquartile range 4–7.25) and mean hospital stay was 12 ± 6.3 days. On early follow-up, carotid patency was confirmed in all patients with colour Doppler, with no intravascular thrombi, narrowing, haematomas, or aneurysms noted. There were no complications secondary to vascular access. There was one early mortality, 27 days post-stenting, which was unrelated to the procedure.

This study adds to the limited literature on ductal stenting with carotid access and the flip technique. In our early experience, the hybrid carotid approach is an attractive alternative to percutaneous carotid puncture and has simplified a complex and challenging intervention, with good outcomes.

To describe outcomes of acute coronavirus disease 2019 in paediatric and young adult patients with underlying cardiac disease and evaluate the association between cardiac risk factors and hospitalisation.

We conducted a retrospective single-institution review of patients with known cardiac disease and positive severe acute respiratory syndrome coronavirus 2 RT-PCR from 1 March, 2020 to 30 November, 2020. Extracardiac comorbidities and cardiac risk factors were compared between those admitted for coronavirus disease 2019 illness and the rest of the cohort using univariate analysis.

Forty-two patients with a mean age of 7.7 ± 6.7 years were identified. Six were 18 years of age or more with the oldest being 22 years of age. Seventy-six percent were Hispanic. The most common cardiac diagnoses were repaired cyanotic (n = 7, 16.6%) and palliated single ventricle (n = 7, 16.6%) congenital heart disease. Fourteen patients (33.3%) had underlying syndromes or chromosomal anomalies, nine (21%) had chronic pulmonary disease and eight (19%) were immunosuppressed. Nineteen patients (47.6%) reported no symptoms. Sixteen (38.1%) reported only mild symptoms. Six patients (14.3%) were admitted to the hospital for acute coronavirus disease 2019 illness. Noncardiac comorbidities were associated with an increased risk of hospitalisation (p = 0.02), particularly chronic pulmonary disease (p = 0.01) and baseline supplemental oxygen requirement (p = 0.007). None of the single ventricle patients who tested positive required admission.

Hospitalisations for coronavirus disease 2019 were rare among children and young adults with underlying cardiac disease. Extracardiac comorbidities like pulmonary disease were associated with increased risk of hospitalisation while cardiac risk factors were not.

Women carrying a fetus diagnosed with congenital heart disease often experience significant distress because of their medical diagnosis. Given the well-documented impact associated with elevated prenatal stress and critical importance of developing targeted interventions, this study aims to examine stressors, coping and resilience resources, and mental health treatment preferences in pregnant women receiving a congenital heart disease diagnosis to inform the development of a psychological intervention to reduce maternal distress prenatally.

Three groups of participants were included consisting of two pregnant women carrying a fetus with congenital heart disease, five women of children (4−16 months) with congenital heart disease, and five paediatric cardiology medical providers. Responses were gathered via semi-structured interviews and analysed using qualitative thematic analysis.

Information regarding four broad areas were analysed of emotional distress during pregnancy; experience of initial diagnosis; coping and resilience; and perspectives on a mental health intervention in pregnancy. Anxiety regarding baby’s future, guilt following diagnosis, and various coping strategies emerged as primary themes among the participant sample. Medical staff corroborated mothers’ heightened anxiety and viewed a psychotherapeutic intervention during the prenatal period as essential and complimentary to standard of care.

We identified salient themes and preferred components for a future psychological intervention delivered prenatally.

Patients’ and providers’ perspectives regarding the nature of maternal distress, resilience and treatment preferences can inform the development of interventions to support the emotional well-being of pregnant women carrying a fetus with congenital heart disease to optimise care and potentially improve outcomes for fetal brain development.

Low socio-economic status is associated with poorer quality of life among children with congenital heart disease (CHD), but this finding is based on disparities among children remaining under cardiology follow-up. We used a population-based health survey data set to analyse the impact of socio-economic status on health and functional status among children with CHD.

We used 2007–2018 National Health Interview Survey data, selecting children 2–17 years of age who had been diagnosed with CHD. Outcomes included caregiver-rated general health, presence of functional limitations, number of missed school days, need for special education, and need for special equipment related to the child’s health conditions. Socio-economic status measures included maternal educational attainment, food stamp programme participation, poverty status, and insurance coverage.

Based on a sample of 233 children with CHD, 10% had fair or poor health, 38% reported having any health-related limitation on their usual activities, 11% needed special equipment, and 27% received special education services. On multivariable analysis, lower maternal educational attainment was correlated with worse caregiver-rated health, and children without insurance were especially likely to experience functional limitations. Black children with CHD had significantly worse caregiver-rated health compared to White children (ordered logit odds ratio: 0.19; 95% confidence interval: 0.08, 0.45; p < 0.001).

In a population-based survey of children with CHD, race and several measures of socio-economic status disadvantage were associated with worse health outcomes. Further evaluation of social determinants of health during cardiology follow-up may help improve outcomes for children with CHD in socio-economically disadvantaged families.

Cardiopulmonary exercise test (CPET) allows quantification of functional capacity of patients with Fontan. The objective of this study was to determine the role of CPET parameters in predicting a higher maximum oxygen consumption (VO2 max) and to analyse the role of CPET parameters in predicting an unfavourable outcome.

A retrospective, cross-sectional, descriptive study was carried out on 57 patients with Fontan, who had undergone incremental CPET with cycloergometer between 2010 and 2020. Determinants of VO2 max and determinants of clinical deterioration were analysed.

In the univariate analysis, the variables significantly related to VO2 max were: age, sex, body mass index (BMI), years of Fontan evolution, intracardiac Fontan, oxygen consumption at anaerobic threshold (VO2AT), CO2 equivalents at anaerobic threshold (VE/VCO2) and chronotropic insufficiency. The multiple linear regression model that best fitted the relationship between VO2 max and independent variables (correlation coefficient 0.73) included sex (correlation index 3.35; p = 0.02), BMI (−0.27; p = 0.02), chronotropic failure (−2.79; p = 0.01) and VO2AT (0.92; p < 0.0001). In the univariate analysis of the prognostic CPET variables related to an unfavourable clinical situation, significance was only obtained with chronotropic insufficiency (p = 0.003). In multivariate analysis, chronotropic insufficiency maintains its association [p= 0.017, OR = 4.65 (1.3–16.5)].

In conclusion, together with the anthropometric parameters universally related to VO2 max, chronotropic insufficiency and VO2AT are the main determinants of functional capacity in patients with Fontan. Moreover, chronotropic insufficiency is closely related to unfavourable clinical evolution. Our data would support the intensive treatment of chronotropic insufficiency in order to improve the quality of life and the clinical situation of patients with Fontan.

Truncus arteriosus is a rare CHD. Neonatal and early infancy repair is recommended though some cases may present late. The aim of our study is to investigate the current results of truncus arteriosus repair and to analyse the differences in outcome and reintervention need between early versus late truncus arteriosus surgical repair.

In this cohort study, we reviewed all children who underwent truncus arteriosus repair from 2001 till 2021. We divided patients into two groups; early repair group including patients repaired at age less than 3 months and late repair group including patients who had repair at 3 months of age and later. We compared both groups for outcome variables.

Sixty-four children had truncus arteriosus repair including 48(75%) patients in early repair and 16(25%) patients in late repair groups. Peri-operative course was comparable between both groups. Post-surgery, we observed pulmonary hypertension in 6(12%) patients in early repair group comparing with 11(69%) patients in late repair group (p = 0.0001). In the last follow-up visit, pulmonary hypertension resolved in all early repair group patients while 6(37.5%) patients in late repair group continued to have pulmonary hypertension (p = 0.0001). Twenty-three(36%) patients required reintervention including 22(48%) in early repair group versus 1(6%) in late repair group (p = 0.007).

In general, the outcome of early truncus arteriosus repair is excellent with resolution of pulmonary hypertension following early repair. Late repair caries higher risk of persistent pulmonary hypertension (37.5%). About one-third of the patients who had truncus arteriosus repair will require re-intervention within 38±38.4 months after initial surgery.

Systemic ventricular end-diastolic pressure is an important haemodynamic variable in adult patients with Fontan circulation. Risk factors associated with elevated end-diastolic pressure have not been clearly identified in this population.

All patients > 18 years with Fontan circulation who underwent cardiac catheterisation at our centre between 1/08 and 3/19 were included. Relevant patient variables were extracted. Univariate and multivariate general linear models were analysed to identify variables associated with end-diastolic pressure.

Forty-two patients were included. Median age was 24.0 years (20.9–29.0) with a body mass index of 23.7 kg/m2 (21.5–29.7). 10 (23.8%) patients had a systemic right ventricle. The median (Interquartile range) and mean pulmonary artery pressure were 11.0 mmHg (9.0–12.0) and 16.0 mmHg (13.0–18.0), respectively. On univariate analysis, end-diastolic pressure was positively associated with body mass index (p < 0.01), age > 25 years (p = 0.04), symptoms of heart failure (p < 0.01), systemic ventricular systolic pressure (p = 0.03), pulmonary artery mean pressure (p < 0.01), and taking diuretics (p < 0.01) or sildenafil (p < 0.01). End-diastolic pressure was negatively associated with aortic saturation (p < 0.01). On multivariate analysis, end-diastolic pressure was positively associated with age ≥ 25 years (p < 0.01), and body mass index (p = 0.04).

In a cohort of adult patients with Fontan circulation undergoing catheterisation, end-diastolic pressure was positively associated with age ≥ 25 years and body mass index on multivariate analysis. Maintaining a healthy body mass index may offer haemodynamic benefit in adults with Fontan physiology.

Iron deficiency is associated with worse outcomes in children and adults with systolic heart failure. While oral iron replacement has been shown to be ineffective in adults with heart failure, its efficacy in children with heart failure is unknown. We hypothesised that oral iron would be ineffective in replenishing iron stores in ≥50% of children with heart failure.

We performed a single-centre retrospective cohort study of patients aged ≤21 years with systolic heart failure and iron deficiency who received oral iron between 01/2013 and 04/2019. Iron deficiency was defined as ≥2 of the following: serum iron <50 mcg/dL, serum ferritin <20 ng/mL, transferrin >300 ng/mL, transferrin saturation <15%. Iron studies and haematologic indices pre- and post-iron therapy were compared using paired-samples Wilcoxon test.

Fifty-one children with systolic heart failure and iron deficiency (median age 11 years, 49% female) met inclusion criteria. Heart failure aetiologies included cardiomyopathy (51%), congenital heart disease (37%), and history of heart transplantation with graft dysfunction (12%). Median dose of oral iron therapy was 2.9 mg/kg/day of elemental iron, prescribed for a median duration of 96 days. Follow-up iron testing was available for 20 patients, of whom 55% (11/20) remained iron deficient despite oral iron therapy.

This is the first report on the efficacy of oral iron therapy in children with heart failure. Over half of the children with heart failure did not respond to oral iron and remained iron deficient.

Children with CHD are at increased risk for neurodevelopmental impairments. There is little information on long-term motor function and its association with behaviour.

To assess motor function and behaviour in a cohort of 10-year-old children with CHD after cardiopulmonary bypass surgery.

Motor performance and movement quality were examined in 129 children with CHD using the Zurich Neuromotor Assessment providing four timed and one qualitative component, and a total timed motor score was created based on the four timed components. The Beery Test of Visual–Motor Integration and the Strengths and Difficulties Questionnaire were administered.

All Zurich Neuromotor Assessment motor tasks were below normative values (all p ≤ 0.001), and the prevalence of poor motor performance (≤10th percentile) ranged from 22.2% to 61.3% in the different components. Visuomotor integration and motor coordination were poorer compared to norms (all p ≤ 0.001). 14% of all analysed children had motor therapy at the age of 10 years. Children with a total motor score ≤10th percentile showed more internalising (p = 0.002) and externalising (p = 0.028) behavioural problems.

School-aged children with CHD show impairments in a variety of motor domains which are related to behavioural problems. Our findings emphasise that motor problems can persist into school-age and require detailed assessment and support.

We carried out a prospective pilot observational experimental study in peri-operative cardiac infants with feeding intolerance between 2019 and 2021. Feeding tube insertion depends on a combination of ultrasound and gastric insufflation with air-saline mixture. Insertion was confirmed by bedside abdominal X-ray.

Out of 500 peri-operative cardiac infants, 15 needed post-pyloric feeding tube insertion in median 15 postoperative day. All were under 6 months of age with average weight of 3 ± 0.2 kg. Median Risk Adjustment for Congenital Heart Surgery Categories was 4. Median insertion time was 15 minutes. No complications have been reported. First pass success rate was 87%, while a second successful insertion attempt was needed in 2 cases (13%). Target daily calorie intake was achieved within average of 3.5 ± 0.4 days. Mean post-pyloric feeding tube stay was 20 ± 3 days. Out of 15 infants, 3 patients died, 1 patient needed gastrostomy tube, and 11 patients were discharged home on oral feeds.

Ultrasound-guided post-pyloric feeding tube insertion using gastric insufflation with air-saline mixture in peri-operative cardiac infants with feeding intolerance is a useful and practical bedside tool, and it can be performed by a trained paediatric cardiac ICU intensivist. It may have potential positive effects on morbidity and outcome.

This study aimed to investigate whether the third trimester fetal cardiac diastolic function measured by selected conventional Doppler indices is affected in appropriate-for-gestational-age or macrosomic fetuses of gestational diabetic mothers with poor glycaemic control.

This cross-sectional study included 93 pregnant women divided into two groups. Group 1 included 45 appropriate-for-gestational-age or macrosomic fetuses from gestational diabetic mothers with poor glycaemic control (study group). Group 2 included 48 appropriate-for-gestational-age fetuses from gestational age-matched healthy mothers (control group). Functional fetal cardiac parameters and fetoplacental Doppler parameters were measured. Data were compared between the two groups.

Maternal characteristics did not differ significantly between the study and the control group. There were no significant differences in the early and late velocity, early/late velocity ratio of both mitral and tricuspid valves, the fetal pulmonary vein pulsatility index, and the ductus venosus pulsatility index between the study and the control group. Moreover, the rate of abnormal Doppler findings in pulmonary vein (pulmonary vein pulsatility index >95th centile), ductus venosus (ductus venosus pulsatility index >95th centile), and peripheral vessels (umbilical artery pulsatility index >95th centile, middle cerebral artery pulsatility index <5th centile, cerebra-placental index >95th centile) were comparable in both groups.

The third trimester fetal diastolic functions measured by selected conventional Doppler techniques do not seem to be altered in appropriate-for-gestational-age or macrosomic fetuses of gestational diabetic mothers who have poor glycaemic control.