Mechanical circulatory support, and specifically extra-corporeal membrane oxygenation, plays a critical role in managing paediatric cardiac patients with severe heart failure. Whilst these technologies are vital for bridging patients to recovery or heart transplantation, it is imperative to study the influence of extra-corporeal membrane oxygenation on neurodevelopmental outcomes.

This systematic review aimed to evaluate the influence of extra-corporeal membrane oxygenation on neurodevelopmental outcomes in paediatric cardiac patients, both post-cardiotomy and non-cardiotomy patients were included.

A comprehensive search was conducted using PubMed, EMBASE, and PsychInfo to identify primary research articles exploring the effects of extra-corporeal membrane oxygenation on neurodevelopmental outcomes in paediatric heart patients from inception to June 2024.

Our search yielded 5488 papers of which eight papers were included featuring 302 patients.

The discussion highlights the considerable variability in neurodevelopmental outcomes and how they are measured among extra-corporeal membrane oxygenation survivors. Outcomes vary by patient factors, with recovery potential influenced by the duration and timing of follow-up. We emphasised the importance of standardised assessment tools and extended follow-ups to gain a clearer understanding of these outcomes.

There is wide variation in institutional sedation strategies in paediatric cardiac ICU. Validated tools such as State Behavioral Scale and Richmond Agitation Sedation Scale were created to help standardise sedation practices.

This is a multi-phase, multicentre, prospective project with the goal of optimising safety and comfort for paediatric cardiac ICU patients. Phase one consisted of an educational intervention with a self-paced, web-based video module on optimal sedation practices using validated sedation screening tools. Participant knowledge was assessed via a de-identified, unmatched pre- and post-test survey. Survey scores were reported as an aggregate average score and compared using a t-test.

There were 259 pre-tests, and 142 post-tests collected during the video-assisted educational intervention. There was a significant increase in mean score on the post-test compared to the pre-test for both instruments: from 4 to 4.8/10 for State Behavioral Scale (p = 0.01) and from 4.5 to 4.9 for Richmond Agitation Sedation Scale (p = 0.04). 81% of respondents who completed the Richmond Agitation Sedation Scale post-test and 88.1% of those who completed the State Behavioral Scale post-test said their practice would change based on the new knowledge acquired.

We report that our newly developed learning module intervention was effective in increasing short-term knowledge about optimal sedation and sedation scoring. Ongoing phase two efforts include evaluation of long-term compliance of validated sedation screening tools and developing an objective score to measure individual cumulative opioid dosing in the cardiac critical care unit.

This study aimed to evaluate the association between serum magnesium levels and the risk of symptomatic patent ductus arteriosus in premature infants.

This retrospective single-centre cohort study analysed the medical records of patients. Neonates were categorised into two groups based on the presence or absence of symptomatic patent ductus arteriosus. Demographic factors including gender, gestational age, type of birth, birth weight, multiple pregnancies, medical treatment for duct closure or surgical ligation, length of hospital stay, and mortality and neonatal outcomes were compared between the groups. The relationship between serum magnesium levels at 24 hours of age and the risk of symptomatic patent ductus arteriosus and other neonatal morbidities was assessed.

This study found no significant relationship between symptomatic patent ductus arteriosus and serum magnesium levels. Additionally, no significant differences were observed between serum magnesium levels and ductal diameter, nor in the need for medical or surgical intervention for symptomatic patent ductus arteriosus. However, neonates with serum magnesium levels greater than 3 mg/dL exhibited a significantly higher incidence of respiratory distress syndrome. Conversely, the prevalence of bronchopulmonary dysplasia was significantly lower in this group, with both findings reaching statistical significance (p < 0.05)

These results suggest that while serum magnesium levels may not be a reliable marker for symptomatic patent ductus arteriosus, they could have clinical implications in the modulation of neonatal respiratory outcomes. Further research is warranted to explore the underlying mechanisms and assess the potential therapeutic role of magnesium in the management of neonatal morbidities.

International organisations recommend that patients with CHD undergo a structured transition process to prepare for lifelong cardiac care. However, there is a limited understanding of current transition practices in the United States (U.S.) from the perspective of adult CHD programs. The purpose of this study was to characterise adult CHD transition practices across the U.S.

We conducted a descriptive, cross-sectional survey of adult CHD programs in the U.S., inquiring about transition practices (preparation, transfer, and integration), resources, specialists, and barriers. We used descriptive statistics to analyse the data.

We analysed responses from 38 adult CHD programs (37% response rate). Among these, 25 (66%) of adult CHD programs reported formal transition programs and 26 (68%) reported having a transfer process to receive patients from paediatric cardiology. Reported transition program specialists were interdisciplinary. Few programs reported having psychologists or psychiatrists on their teams or offered support for patients with intellectual disability. The main barriers affecting transition were insurance and health care costs.

Around two-thirds of respondent adult CHD programs reported the presence of formal transition programs. More resources may be needed within these programmes to support patient psychological well-being and those with intellectual disability and to address barriers related to insurance and health care costs.

Neurodevelopmental disorders occur in up to 50% of children with CHD. Small head circumference at birth has been associated with impaired neurodevelopment in patients with complex CHD. It is unknown if patients with simple CHD such as septal defects have smaller head circumferences. The objective of this study was to investigate the head circumference at birth in neonates with either an atrial or a ventricular septal defect.

This study is part of the Copenhagen Baby Heart Study; a prospective, population-based cohort study of more than 25,000 neonates. The neonates were examined with a comprehensive transthoracic echocardiography within the first 30 days of birth including assessment for atrial or ventricular septal defects. The head circumference at birth in term neonates with septal defects was compared to the head circumference in matched controls, term neonates without septal defects from the same birth cohort.

Neonates with septal defects (n = 1,030; 45.2% male; mean birthweight 3,534g ± 483g) had a mean head circumference of 34.8 cm (95% confidence interval 34.7–34.9 cm), compared to neonates without septal defects (n = 5,150; 45.6% male; mean birthweight 3,546g ± 476g) of 34.7 cm (95% confidence interval 34.7–34.8 cm); p-value 0.07. Mean calculated z-score of head circumferences was 0.05 for neonates with septal defects and –0.01 for neonates without septal defects, p = 0.07. Dividing cases into neonates with atrial septal defects, ventricular septal defects, and those without septal defects did not show differences between groups, p = 0.14.

The head circumference in term neonates with septal defects did not differ from matched controls without septal defects.

We explore the role of targeted echocardiography as a screening tool for bicuspid aortic valve and left ventricular hypertrophy, specifically assessing the risk of missing significant cardiac findings that would otherwise be identified by comprehensive echocardiograms.

Children < 18 years at initial echocardiogram for indications of “family history of bicuspid aortic valve” and “left ventricular hypertrophy on electrocardiogram” were queried. Cardiology clinic notes and complete echocardiogram reports were reviewed for additional background histories and incidental findings. Follow-up clinic visits, if any, and management for those with incidental findings were reviewed.

Bicuspid aortic valve group included 138 patients, 71 (51%) males and mean age at comprehensive echo was 8.4 ± 4.8 years. Bicuspid aortic valve was found in 3.6%, incidental findings were found in 15 (11%), and follow-up was recommended in 4 (2.8%). Left ventricular hypertrophy group included 70 patients, 58 (83%) males and mean age at echo 10.9 ± 4.7 years. Left ventricular hypertrophy was found in 2.8%, incidental findings were found in 9 (13%), and follow-up was recommended in 2 (2.8%).

None of the follow-up group developed symptoms or required cardiac medications, exercise restrictions, or catheter or surgical-based interventions, except for one case of mild aortic root dilation who was restricted from heavy weightlifting.

The risk of missing clinically important findings with targeted echocardiography that would have been identified with comprehensive echocardiography is extremely low for screening indications of isolated left ventricular hypertrophy on electrocardiogram or family history of bicuspid aortic valve, suggesting that targeted echocardiography could be an effective screening tool.

Passive leg raising is used to predict who will benefit from fluid therapy in critically ill patients, including children. Patients with a Fontan circulation may have a different haemodynamic response to a fluid challenge by passive leg raising.

The haemodynamic response of 31 paediatric patients with a Fontan circulation from the outpatient clinic (median age 14.0 years) and 35 healthy controls (median age 12.8 years) to passive leg raising was evaluated non-invasively by echocardiography for the assessment of, e.g., velocity time integral across the (neo)aortic valve, blood pressure measurements, and respiration. Participants were considered responders when the velocity time integral increased ≥ 10.0%.

Overall, patients and controls did not differ in the haemodynamic response. Twelve patients (38.7%) and 8 controls (22.9%) were responders, which was not statistically different (P = 0.22). Responders in the patient and control group also had a similar echo-estimated velocity time integral increase of + 18.9% and + 15.2%, respectively (P = 0.91). There was no difference in echo-estimated velocity time integral change between patient and control non-responders with a decrease of −1.4% and −6.4%, respectively (P = 0.70) and no difference in the amount of patients who were negatively affected by passive leg raising, ith a decrease of ≤−10.0% in 7 patients (22.6%) and 9 controls (25.7%)(P = 0.77).

The haemodynamic response of ambulatory paediatric patients with a Fontan circulation to passive leg raising is like that of healthy controls. Patients who did not respond were similarly affected as healthy controls. Whether the haemodynamic response is different in critically ill patients warrants further investigation.

The Netherlands National Trial Register (NTR), Trial: NL6415; date of registration 20-07-2017; Trial information: https://www.trialregister.nl/trial/6415.

To summarise the characteristics and postoperative outcomes in paediatric patients with coronary sinus septal defect.

This retrospective study recruited paediatric patients diagnosed with coronary sinus septal defect from the Guangdong Cardiovascular Institute between 2011 and 2023. Clinical characteristics, echocardiographic parameters, surgical procedures, and postoperative outcomes were collected from electronic health records.

Among the 68 patients, 50% were male, with a median age of 1.0 years. Four cases (5.9%) were diagnosed during the prenatal period. The proportions of patients with type I, II, III, and IV coronary sinus septal defect were 51.5%, 5.9%, 16.1%, and 26.5%, respectively. The most common coexisting cardiac anomalies were persistent left superior caval vein. Twenty-seven cases were either missed or misdiagnosed by echocardiogram, accounting for 39.7% of the overall cases, with type I being the most frequently missed diagnosis. Fifty-four patients underwent surgery, two patients received transcutaneous intervention, while the remaining patients did not undergo any surgery or intervention. At follow-up, two patients with type I coronary sinus septal defect died from multiorgan dysfunction, and one patient underwent reoperation due to narrowing of the extracardiac tunnel. The remaining patients did not experience any major events and recovered well.

Paediatric patients with coronary sinus septal defect often do not exhibit specific clinical manifestations. Enhancing our understanding of the anatomic and haemodynamic characteristics of coronary sinus septal defect can improve the diagnostic accuracy of echocardiography. If diagnosis is suspected, confirmation can be obtained by cardiac CT and cardiac magnetic resonance. Accurate preoperative and intraoperative diagnosis of coronary sinus septal defect contributes to high surgical success rates and favourable treatment outcomes.

Epicardial fat thickness also increases in insulin resistance and diabetes mellitus patients. In our study, we aimed to compare the epicardial fat thickness and interventricular septum diameter in infants of mothers with diabetes with infants of mothers without diabetes. Also, to determine the effect of birth weight on the epicardial fat thickness and the interventricular septum diameter in infants of mothers with diabetes.

Between November 2022 and June 2023, infants of mothers with diabetes and healthy infants were compared. According to birth weight, infants of mothers with diabetes were divided into two groups, macrosomic infants (≥ 4000 g) and non-macrosomic infants (2500–4000 g).

A total of 78 infants of mothers with diabetes and 56 infants of mothers without diabetes were evaluated. Epicardial fat thickness and interventricular septum diameter were found to be statistically significantly higher in infants of mothers with diabetes than in healthy infants. Epicardial fat thickness and interventricular septum diameter measurements were found to be statistically significantly higher in the macrosomic of infants than in the non-macrosomic of infants of mothers with diabetes. Asymmetric septal hypertrophy was found to be more common in macrosomic infants of diabetic mothers than in non-macrosomic infants, although not statistically significant. We also found a positive correlation between epicardial fat thickness and asymmetric septal hypertrophy.

Epicardial fat thickness is observed to increase along with neonatal macrosomia and heart mass in gestational diabetes mellitus. The lack of a significant increase in epicardial fat thickness and interventricular septum diameter in non-macrosomic infants of mothers with diabetes may be indicative of good glycaemic control in non-macrosomic infants of mothers with diabetes. Therefore, good glycaemic control during pregnancy and long-term follow-up of infants of mothers with diabetes after birth is important.

Medical and surgical advancements have enabled a 95% survival rate for children with CHD. However, these survivors are disproportionately affected by neurodevelopmental disabilities. In particular, they have behavioural problems in toddlerhood. Because there is a known relationship between behavioural problems and early language delay, we hypothesise that children with critical CHD have early detectable language deficits. To test our hypothesis, we performed a retrospective study on a cohort of children with critical CHD to visualise their early language developmental trajectories.

We identified a cohort of 27 children with two diagnoses: single ventricle physiology (19) and transposition of the great arteries (8). As part of their routine clinical care, all of these children had serial developmental evaluations with the language subsection of the Capute Scales. We visualised their developmental language trajectories as a function of chronologic age, and we used a univariate linear regression model to calculate diagnosis-specific expected developmental age equivalents.

In each group, language development is age-appropriate in infancy. Deviation from age-appropriate development is apparent by 18 months. This results in borderline-mild language delay by 30 months.

Using the Capute Scales, our team quantified early language development in infants and toddlers with critical CHD. Our identification of deceleration in skill acquisition reinforces the call for ongoing neurodevelopmental surveillance in these children. Understanding early language development will help clinicians provide informed anticipatory guidance to families of children with critical CHD.

Children with single ventricle physiology and transposition of the great arteries have measurable early language delays.

Kawasaki disease, an acute systemic small- and medium-vessel vasculitis, is mostly detected in children under 5 years old.

We aimed to explore the predictive value of long non-coding ribonucleic acid small nucleolar RNA host gene 5 (SNHG5) and microRNA (miRNA)-27a for the effect of standard intravenous immunoglobulintherapy on children with Kawasaki disease.

The study included 182 children undergoing standard intravenous immunoglobulin therapy for Kawasaki disease and another 182 healthy children receiving physical examinations as a control group. LncRNA SNHG5 and miRNA-27a expression levels were determined at admission.

The ineffective group had higher levels of interleukin-6, C-reactive protein, procalcitonin, lncRNA SNHG5, and miRNA-27a and Kobayashi score than those of the effective group (P < 0.05). Multivariate regression analysis showed that Kobayashi score, interleukin-6, C-reactive protein, procalcitonin, lncRNA SNHG5, and miRNA-27a were associated with the treatment outcomes (P < 0.05). LncRNA SNHG5 and miRNA-27a levels were positively correlated with Kobayashi score, interleukin-6, receiver operating characteristic and procalcitonin levels (r > 0, P < 0.05). High Kobayashi score and levels of interleukin-6, c-reactive roe, procalcitonin, lncRNA SNHG5, and miRNA-27a were influencing factors for treatment failure (odds ratio > 1, P < 0.05). The areas under the curves of lncRNA SNHG5, miRNA-27a, and their combination were 0.757, 0.766, and 0.831, respectively.

LncRNA SNHG5 and miRNA-27a are highly expressed in children with Kawasaki disease, and their levels are closely correlated with the efficacy of standard immunoglobulin therapy.

Tetralogy of Fallot is the most common cyanotic congenital malformation of the heart. The right ventricular outflow tract is of great interest in this setting, but most of the focus on this feature has been on the size of the so-called pulmonary valvar “annulus”. We aimed to characterise other aspects of the morphology of the pulmonary root in heart specimens with tetralogy of Fallot.

We reviewed archived hearts with tetralogy of Fallot from four registries. The pulmonary root was examined with specific attention to the number of sinuses, the number of leaflets, presence of any fusion of leaflets, and the direction of the zone of apposition between the leaflets. Cluster analyses were then conducted to see if the features permitted segregation into groups.

We examined a total of 155 hearts. The pulmonary valve had two leaflets in 62%, three leaflets in 34%, and one leaflet in 3%. Irrespective of leaflet morphology, most hearts had two sinuses. Cluster analysis permitted segregation into three groups, with the direction of the zone of apposition being the most important feature for segregation.

In two-thirds of our hearts with tetralogy of Fallot, the pulmonary valve had two leaflets. Most frequently there were three sinuses. In the setting of a valve with two sinuses, the zone of apposition between the leaflets pointing towards the aorta. Cluster analysis permitted statistically sound segregation of the heart and highlights the importance of delineating these features, specifically the leaflet and sinus morphology, with clinical imaging.

Aortic coarctation can occur isolated or associated with ventricular septal defect. This study evaluated aortic stiffness in normotensive patients surgically treated for aortic coarctation and ventricular septal defect and in those who underwent simple aortic coarctation repair. Both groups were compared with healthy controls. Again, the two pathological groups were compared with each other regarding aortic stiffness and left ventricular diastolic function. A possible relationship between aortic stiffness and left ventricular diastolic function was investigated.

Twenty-two isolated aortic coarctation patients and 17 aortic coarctation and ventricular septal defect patients were enrolled. Aortic root distensibility and aortic stiffness index were calculated from echocardiography and blood pressure. E wave to A wave (E/A) ratio was measured from mitral valve inflow profile.

Aortic root distensibility and aortic stiffness index in simple aortic coarctation vs healthy controls: both p < 0.0001. Aortic root distensibility and aortic stiffness index in aortic coarctation/ventricular septal defect vs healthy controls: both p < 0.0001. Aortic root distensibility and aortic stiffness index were similar in the two pathological groups (both p = ns). No statistically significant difference was detected in relation to left ventricular diastolic function (p = ns). No correlation was detected between aortic stiffness and diastolic function in simple aortic coarctation and aortic coarctation/ventricular septal defect groups (both p = ns).

In both normotensive isolated aortic coarctation and aortic coarctation/ventricular septal defects subgroups, aortic stiffness is increased in a similar way in comparison with controls. Diastolic function was normal and similar in both groups. Aortic stiffness was not related to left ventricular diastolic function in this specific setting.

To evaluate the outcomes of patients with single ventricle physiology supported with extracorporeal membrane oxygenation as a bridge to first-stage palliation.

This was a retrospective registry-based study. Data from the Extracorporeal Life Support Organization registry were used to identify single ventricle physiology patients supported with extracorporeal membrane oxygenation prior to palliation from 2016 to 2021. Descriptive statistics and multivariate analyses for associations with mortality were conducted.

Primary outcome was death before hospital discharge. Patient characteristics including demographics and associated complications were evaluated as secondary outcomes. Sixty-five patients met inclusion criteria. Survival to discharge was 42%. Twenty-four (37%) patients died while on extracorporeal membrane oxygenation. There was no significant difference in demographics between survivors and non-survivors. Non-survivors had a significantly longer median duration on extracorporeal membrane oxygenation compared to survivors, 99-hrs [IQR (Interquartile Range), 160, 300] vs. 59-hrs [43, 124] (p<0.001). Multivariate analysis demonstrated extracorporeal membrane oxygenation duration (adjusted-OR [Odds Ratio] 1.01, 95% CI [Confidence Interval] 0.98, 0.99; p = 0.03) and requiring renal replacement therapy (42% vs. 19%; p = 0.04) were associated with mortality prior to discharge.

Clinicians managing decompensated patients with single ventricle physiology may consider extracorporeal membrane oxygenation as a bridge to palliation. Survival to discharge was 42%. Evidence of renal injury and longer extracorporeal membrane oxygenation durations were associated with mortality. These data may be used to guide providers and to counsel families. However, more data are needed to refine indications and assess associations related to outcomes and decision-making.

In paediatric cardiac patients requiring staged palliation, superior cavopulmonary anastomosis is common. Pain control is a crucial aspect of postoperative care as agitation, untreated pain, and hypoventilation can cause increased pulmonary vascular resistance reduction and pulmonary blood flow.

This was a large volume single-centre, retrospective cohort study evaluating the impact of gabapentin on opioid and benzodiazepine exposure in infants undergoing superior cavopulmonary anastomosis between January 2018 and December 2022. The primary endpoint was opioid exposure in morphine milligram equivalents per kilogram in infants receiving gabapentin compared to no gabapentin.

The study analysed 85 infants, 40 of which received perioperative gabapentin. Other than there being more males in the gabapentin group (70% versus 47%; p = 0.03), there was no difference in baseline characteristics. Opioid use, measured in morphine milligram equivalents per kilogram, was similar in the no gabapentin group compared to the gabapentin group during the first 5 POD’s (2.66 (interquartile range1.76, 3.30) versus 2.27 (interquartile range R 1.75, 3.40); p = 0.93. However, there was a lower benzodiazepine exposure, measured in midazolam equivalents per kilogram, in the gabapentin group both on POD 2 (0.05 (interquartile range 0.00, 0.11) versus 0 (interquartile range 0.00, 0.08); p = 0.031) and cumulative (0.15 (interquartile range 0.00, 0.35) versus 0.05 (interquartile range 0.00, 0.15); p = 0.031).

Gabapentin did not significantly reduce opioid exposure; however, its use was associated with modest reduction in benzodiazepine exposure. There were no differences in adverse events. Our findings suggest gabapentin is safe in infants undergoing superior cavopulmonary anastomosis; however, additional studies should be conducted to evaluate optimal gabapentin dosing.

Current research suggests that a small pulmonary artery can cause adverse events and reduce exercise capacity after the Fontan procedure. This study aimed to evaluate the impact of pulmonary artery size on early haemodynamic and laboratory variables after total cavopulmonary connection.

We reviewed all patients who underwent staged Fontan between 2012 and 2022. Pulmonary artery index before bidirectional cavopulmonary shunt and before total cavopulmonary connection was calculated according to Nakata and colleagues. We sought to analyse the impact of the pulmonary artery index on early haemodynamic and laboratory variables, including pulmonary artery pressure and mean arterial pressure 12 hours after extubation and lactate levels 6 hours after extubation.

A total of 263 patients were included. Median age and weight at total cavopulmonary connection were 2.2 (interquartile ranges: 1.8–2.7) years and 11.7 (interquartile range: 10.7–13.3) kg, respectively. Before that, all patients underwent bidirectional cavopulmonary shunt at a median age of 4.1 (interquartile range: 3.2–5.8) months. In the multivariable analysis, pre-bidirectional cavopulmonary shunt pulmonary artery index (p = 0.016, odds ratio 0.993), with a cut-off value of 154 mm2/m2 was an independent risk factor for a higher pulmonary artery pressure (> 17 mmHg). No variable was identified as a significant risk factor for lower mean arterial pressure (< 57 mmHg). Regarding lactate levels (> 4.5 mg/dl), pre-bidirectional cavopulmonary shunt right pulmonary artery index (p < 0.001, odds ratio 0.983), with a cut-off value of 70 mm2/m2 was identified as an independent risk factor.

In patients with staged Fontan palliation, a small pulmonary artery size before bidirectional cavopulmonary shunt and total cavopulmonary connection was a determinant factor associated with unfavourable early postoperative haemodynamics after total cavopulmonary connection.