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.

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.

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.

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 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.

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.

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.

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.

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.