To describe the epidemiology of severe bleeding in the immediate post-operative period in children who undergo cardiopulmonary bypass surgery using the Bleeding Assessment Scale for critically Ill Children (BASIC).

Retrospective cohort study in a paediatric ICU from 2015 to 2020.

356 children were enrolled; 59% were male with median (IQR) age 2.1 (0.5–8) years. Fifty-seven patients (16%) had severe bleeding in the first 24 hours post-operatively. Severe bleeding was observed more frequently in younger and smaller children with longer bypass and cross-clamp times (p-values <0.001), in addition to higher surgical complexity (p = 0.048). Those with severe bleeding received significantly more red blood cells, platelets, plasma, and cryoprecipitate in the paediatric ICU following surgery (all p-values <0.001). No laboratory values obtained on paediatric ICU admission were able to predict severe post-operative bleeding. Those with severe bleeding had significantly less paediatric ICU-free days (p = 0.010) and mechanical ventilation-free days (p = 0.013) as compared to those without severe bleeding.

Applying the BASIC definition to our cohort, severe bleeding occurred in 16% of children in the first day following cardiopulmonary bypass. Severe bleeding was associated with worse clinical outcomes. Standard laboratory assays do not predict bleeding warranting further study of available laboratory tests.

To study the impact of out-of-hours delivery on outcome for neonates with antenatally diagnosed transposition of the great arteries.

Tertiary paediatric cardiology centre (Yorkshire, United Kingdom), with co-located tertiary neonatal unit.

Neonates with antenatally diagnosed simple transposition of the great arteries delivered out-of-hours (Monday to Friday 17:00–08:00 and weekends) versus in-hours between 2015 and 2020.

The primary outcome was survival to hospital discharge. Secondary outcomes included neurological morbidity, length of stay, and time to balloon atrial septostomy.

Of 51 neonates, 38 (75%) were delivered out-of-hours. All neonates born in the tertiary centre survived to discharge. Time to balloon atrial septostomy was slightly longer for out-of-hours deliveries compared to in-hours (median 130 versus 93 mins, p = 0.33). Neurological morbidity occurred for nine (24%) patients in the out-of-hours group and one (8%) in-hours (OR 3.72, 95% CI: 0.42–32.71, p = 0.24). Length of stay was also similar (18.5 versus 17.3 days, p = 0.59). Antenatal diagnosis of a restrictive atrial septum was associated with a lower initial pH (7.03 versus 7.13; CI: 0.03–0.17, p = 0.01), longer length of stay (22.6 versus 17.3 days; CI: 0.37–10.17, p = 0.04), and increased neurological morbidity (44% versus 14%; OR 4.80, CI 1.00–23.15, p = 0.05). A further three neonates were delivered in surrounding hospitals, with a mortality of 67% (versus 0 in tertiary centre; OR 172, CI 5-5371, p = 0.003).

Neonates with antenatally diagnosed transposition of the great arteries have similar outcomes when delivered out-of-hours versus in-hours. Antenatal diagnosis of restrictive atrial septum is a significant predictor of worse outcomes. In our region, delivery outside the tertiary cardiac centre had a significantly higher risk of mortality.

Growth failure in infants born with CHD is a persistent problem, even in those provided with adequate nutrition.

To summarise the published data describing the change in urinary metabolites during metabolic maturation in infants with CHD and identify pathways amenable to therapeutic intervention

Scoping review.

Studies using qualitative or quantitative methods to describe urinary metabolites pre- and post-cardiac surgery and the relationship with growth in infants with CHD.

NICE Healthcare Databases website was used as a tool for multiple searches.

347 records were identified, of which 37 were duplicates. Following the removal of duplicate records, 310 record abstracts and titles were screened for inclusion. The full texts of eight articles were reviewed for eligibility, of which only two related to infants with CHD. The studies included in the scoping review described urinary metabolites in 42 infants. A content analysis identified two overarching themes of metabolic variation predictive of neurodevelopmental abnormalities associated with anaerobic metabolism and metabolic signature associated with the impact on gut microbiota, inflammation, energy, and lipid digestion.

The results of this scoping review suggest that there are considerable gaps in our knowledge relating to metabolic maturation of infants with CHD, especially with respect to growth. Surgery is a key early life feature for CHD infants and has an impact on the developing biochemical phenotype with implications for metabolic pathways involved in immunomodulation, energy, gut microbial, and lipid metabolism. These early life fingerprints may predict those individuals at risk for neurodevelopmental abnormalities.

Feeding difficulty is common in infants with CHD.

The purpose of this study was to describe the feeding skills and behaviours of infants with CHD at 6–12 months of age and explore relationships between feeding, gastrointestinal distress, and gastroesophageal reflux.

Parents of 30 infants with CHD completed online surveys when their infant was 6, 8, 10, and 12 months old. Surveys included parent-report measures of feeding skills (Child Oral and Motor Proficiency Scale), feeding behaviours (Pediatric Eating Assessment Tool), symptoms of gastrointestinal distress (Infant Gastrointestinal Symptoms Questionnaire), and gastroesophageal reflux (Infant Gastroesophageal Reflux Questionnaire-Revised).

We found that 95, 32, 67, and 30% of infants were delayed in their feeding skill development at 6, 8, 10, and 12 months, respectively. Symptoms of problematic feeding behaviours were similarly high, with 90, 62, 29, and 38% of infants meeting criteria for problematic feeding at 6, 8, 10, and 12 months, respectively. Feeding skills and behaviours were related but unique contributors. Feeding behaviours were related to both gastrointestinal distress and gastroesophageal reflux, but feeding skills were rarely related to either.

Delayed acquisition of feeding skills and problematic feeding were common in infants with CHD. Infants with more gastrointestinal and gastroesophageal reflux symptoms had more symptoms of problematic feeding behaviours.

Comprehensive assessment, targeted interventions, and early involvement of feeding specialists are important to support feeding in infants with CHD through at least the first year of life.

Right ventricular outflow tract intervention spans transcatheter, surgical, or hybrid pulmonary valve replacement methodologies. Standardised pre-procedure workup includes cardiac MRI to identify an intended valve site (landing zone). Our institutional practice includes measurement of the right ventricular outflow tract perimeter (circumference) of this site in end-systole. Our primary aim was to compare patients by their perimeter values to the palliative interventions performed (transcatheter versus surgical/hybrid methodologies).

Retrospective review of patients undergoing pulmonary valve replacement from January 2017 to 2021. We performed perimeter measurements at the intended valve site on advanced imaging; the outcomes of interventions were outlined via descriptive and statistical analyses.

A total of 37 patients underwent pulmonary valve replacement that met study criteria – 21 transcatheter, 7 surgical, and 9 hybrid. Median age at intervention was 26 years (range 8–70). The mean end-systolic perimeter of the transcatheter cohort was 88.9 ± 8.7 mm and in the surgical/hybrid cohort measured 106.6 ± 7.5 mm. For the transcatheter cohort, the median “circularised” diameter derived from the perimeter measurement (divided by π) was 27.7 mm (range 24.3–32.4). Notably, this correlated (r = 0.93, p < 0.01) with the median diameter of the narrowest region during actual transcatheter right ventricular outflow tract balloon sizing (lateral imaging) of 27.1 mm (range 23.2–30.1).

Right ventricular outflow tract perimeter measurement to determine circularised diameter is useful in planning pulmonary valve replacement in terms of candidacy of transcatheter versus the need for a surgical/hybrid approach. The circularised diameter correlates with transcatheter right ventricular outflow tract balloon sizing.

The goal of this meta-analysis is to evaluate the effects of exercise training on long-term health and cardiorespiratory fitness in participants with CHD after surgery and to investigate the optimal type of exercise training for post-operative patients and how to improve adherence to it.

We searched the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, and Web of Science from the date of the inception of the database through August 2021.

Altogether, 1424 records were identified in the literature search. Studies evaluating outcomes between exercise training and usual care among post-operative patients with CHD were included. The assessed outcomes were quality of life and cardiorespiratory fitness. We analysed heterogeneity by using the I2 statistic and evaluated the evidence quality according to the recommendation by the Cochrane Collaboration. Nine randomised controlled trials were included. The evidence showed that exercise interventions increased peak oxygen consumption (mean difference = 2.29 [95% CI 0.43, 4.15]; p = 0.02, I2 = 0%). However, no differences in scores of health-related quality of life and pulmonary function were observed between the experimental and control groups.

In conclusion, participation in a physical exercise training programme was safe and improved fitness in patients after surgery for CHD. We recommend that post-operative patients with CHD participate in physical exercise training. Additional research is needed to study the various forms of exercise training and their impact on quality of life.

There is a paucity of information reported regarding the use of milrinone in patients with hypoplastic left heart syndrome prior to the Norwood procedure. At our institution, milrinone is initiated in the pre-operative setting when over-circulation and elevated serum lactate levels develop. We aimed to review the responses associated with the administration of milrinone in the pre-operative hypoplastic left heart syndrome patient. Second, we compared patients who received high- versus low-dose milrinone prior to Norwood procedure.

Single-centre retrospective study of patients diagnosed with hypoplastic left heart syndrome between January 2000 and December 2019 who underwent Norwood procedure. Patient characteristics and outcomes were compared.

During the study period, 375 patients were identified; 79 (21%) received milrinone prior to the Norwood procedure with median lactate 2.55 mmol/l, and SpO2 93%. Patients who received milrinone were older at the time of Norwood procedure (6 vs. 5 days) and were more likely to be intubated and sedated. In a subset analysis stratifying patients to low- versus high-dose milrinone, median lactate decreased from time of initiation (2.39 vs 2.75 to 1.6 vs 1.8 mmol/l) at 12 hours post-initiation, respectively. Repeated measures analysis showed a significant decrease in lactate levels by 4 hours following initiation of milrinone, that persisted over time, with no significant difference in mean arterial pressure.

The use of milrinone in the pre-operative over-circulated hypoplastic left heart syndrome patient is well tolerated, is associated with decreased lactate levels, and was not associated with significant hypotension or worsening of excess pulmonary blood flow.

Chylothorax after congenital cardiac surgery is associated with increased risk of malnutrition. Nutritional management following chylothorax diagnosis varies across sites and patient populations, and a standardised approach has not been disseminated. The aim of this review article is to provide contemporary recommendations related to nutritional management of chylothorax to minimise risk of malnutrition.

The management guidelines were developed by consensus across four dietitians, one nurse practitioner, and two physicians with a cumulative 52 years of experience caring for children with CHD. A PubMed database search for relevant literature included the terms chylothorax, paediatric, postoperative, CHD, chylothorax management, growth failure, and malnutrition.

Fat-modified diets and nil per os therapies for all paediatric patients (<18 years of age) following cardiac surgery are highlighted in this review. Specific emphasis on strategies for treatment, duration of therapies, optimisation of nutrition including nutrition-focused lab monitoring, and supplementation strategies are provided.

Our deliverable is a clinically useful guide for the nutritional management of chylothorax following paediatric cardiac surgery.

Paediatricians play an integral role in the lifelong care of children with CHD, many of whom will undergo cardiac surgery. There is a paucity of literature for the paediatrician regarding the post-operative care of such patients.

The aim of this manuscript is to summarise essential principles and pertinent lesion-specific context for the care of patients who have undergone surgery or intervention resulting in a biventricular circulation.

Familiarity with common issues following cardiac surgery or intervention, as well as key details regarding specific lesions and surgeries, will aid the paediatrician in providing optimal care for these patients.

Single ventricle CHD affects about 5 out of 100,000 newborns, resulting in complex anatomy often requiring multiple, staged palliative surgeries. Paediatricians are an essential part of the team that cares for children with single ventricle CHD. These patients often encounter their paediatrician first when a complication arises, so it is critical to ensure the paediatrician is knowledgeable of these issues to provide optimal care.

We reviewed the subtypes of single ventricle heart disease and the various palliative surgeries these patients undergo. We then searched the literature to detail the general paediatrician’s approach to single ventricle patients at different stages of surgical palliation.

Single ventricle patients undergo staged palliation that drastically changes physiology after each intervention. Coordinated care between their paediatrician and cardiologist is requisite to provide excellent care. This review highlights what to expect when these patients are seen by their paediatrician for either well child visits or additional visits for parental or patient concern.

Systemic-to-pulmonary collateral flow is a well-recognised phenomenon in patients with single ventricle physiology, but remains difficult to quantify. The aim was to compare the reported formula’s that have been used for calculation of systemic-to-pulmonary-collateral flow to assess their consistency and to quantify systemic-to-pulmonary collateral flow in patients with a Glenn and/or Fontan circulation using four-dimensional flow MRI (4D flow MR).

Retrospective case–control study of Glenn and Fontan patients who had a 4D flow MR study. Flows were measured at the ascending aorta, left and right pulmonary arteries, left and right pulmonary veins, and both caval veins. Systemic-to-pulmonary collateral flow was calculated using two formulas: 1) pulmonary veins – pulmonary arteries and 2) ascending aorta – caval veins. Anatomical identification of collaterals was performed using the 4D MR image set.

Fourteen patients (n = 11 Fontan, n = 3 Glenn) were included (age 26 [22–30] years). Systemic-to-pulmonary collateral flow was significantly higher in the patients than the controls (n = 10, age 31.2 [15.1–38.4] years) with both formulas: 0.28 [0.09–0.5] versus 0.04 [−0.66–0.21] l/min/m2 (p = 0.036, formula 1) and 0.67 [0.24–0.88] versus -0.07 [−0.16–0.08] l/min/m2 (p < 0.001, formula 2). In patients, systemic-to-pulmonary collateral flow differed significantly between formulas 1 and 2 (13% versus 26% of aortic flow, p = 0.038). In seven patients, veno-venous collaterals were detected and no aortopulmonary collaterals were visualised.

4D flow MR is able to detect increased systemic-to-pulmonary collateral flow and visualise collaterals vessels in Glenn and Fontan patients. However, the amount of systemic-to-pulmonary collateral flow varies with the formula employed. Therefore, further research is necessary before it could be applied in clinical care.

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.

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.

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.

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.