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.

Carvajal syndrome, a rare autosomal recessive disorder caused by mutations in the DSP gene, is characterised by woolly hair, palmoplantar keratoderma, and left ventricular dilated cardiomyopathy. Although less frequently reported, noncompaction cardiomyopathy can co-occur, further complicating the clinical picture. Early diagnosis and management are crucial due to the high risk of progressive heart failure and sudden cardiac death in affected individuals.

A 13-year-old male with autism presented with a 1.5-month history of persistent cough and worsening clinical symptoms, including hepatomegaly and signs of heart failure. Physical examination revealed woolly hair, patchy alopecia, nail anomalies, and ectodermal dysplasia. Echocardiography demonstrated left ventricular dilated cardiomyopathy, noncompaction, and a severely reduced ejection fraction of 23%. Initial management in the paediatric intensive care unit included inotropic support, diuretics, and beta-blockers. Genetic analysis confirmed a homozygous c.7912G > T nonsense variant in the DSP gene, establishing the diagnosis of Carvajal syndrome. The patient was referred to an advanced cardiac centre.

Carvajal syndrome involves multisystem manifestations, with prominent dermatologic and cardiovascular features. Unlike Naxos disease, which primarily affects the right ventricle, Carvajal syndrome predominantly involves the left ventricle, as observed in this case. Notably, left ventricular noncompaction was a striking feature in our patient, further exacerbating cardiac dysfunction and complicating the clinical course. Although noncompaction cardiomyopathy is less frequently reported in Carvajal syndrome, its pronounced presence in this case underscores the phenotypic variability and severity of myocardial involvement. Intensive care management with a multidisciplinary approach was essential in stabilising this patient. Genetic testing confirmed the diagnosis and highlighted the importance of molecular diagnostics in differentiating cardiocutaneous syndromes.

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.

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.