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.

Left bundle branch area pacing is a recent technique gaining rapid acceptance due to its broader target area and excellent electrical parameters. The aim of this study was to demonstrate the feasibility of left bundle branch area pacing in children and share short-term results.

A retrospective study conducted at a single centre between December 2021 and April 2024 involved 19 children who underwent left bundle branch area pacing using Select Secure leads. The study included echocardiographic evaluations, pacing parameters, and follow-up outcomes.

The cohort comprised 10 males and 9 females. Median age was seven years (range 2–18), and median weight was 38 kg (range 13–56). All patients had complete atrioventricular block, with seven having isolated congenital complete atrioventricular block and 12 postoperative complete atrioventricular block. In nine patients, transitioning from epicardial to endocardial pacing resulted in ventricular dysfunction due to chronic right ventricular pacing. The remaining patients received left bundle branch area pacing initially. One patient underwent implantation in a septal position close to the left bundle due to left bundle branch area pacing infeasibility. The median post-procedure QRS duration was 92 msec (range 80–117). Median R wave amplitude, threshold, and impedance values were 14.7 mV (range 13.3–16.8), 0.7 mV (range 0.5–1.1), and 728 ohms (range 640–762), respectively. Atrioventricular (DDD mode) leads were implanted in 10 patients, and ventricular leads (VVIR mode) were implanted in nine patients. Median fluoroscopy dose was 18.7 mGy (13.5–34.52).

Left bundle branch area pacing can be safely conducted in paediatric patients exhibiting a narrow QRS duration and stable pacing parameters.

The American College of Cardiology has published clinical practice algorithms for common congenital heart lesions, including atrial septal defect, patent ductus arteriosus, valvar pulmonary stenosis, aortic coarctation, and ventricular septal defect. The purpose of this study was to define the current practice patterns in the management of these lesions and describe the impact of departure from these recommendations.

This was a retrospective analysis of the most recent 100 outpatient appointments for each lesion at our centre. Electronic medical records were queried to determine whether the scheduling, testing, and follow-up plan for each appointment were consistent with the published algorithms.

A total of 500 visits were evaluated (150 new visits; 350 follow-up visits); 32% (n = 162) of encounters did not receive appropriate testing, 37% (n = 186) departed from recommended follow-up plans, and of the 350 follow-up visits, 45% (n = 156) departed from scheduling guidelines. Impact of these departures was quantified in reference to over- or under-expenditure of clinical resources. Of the aberrant testing encounters, 60% (n = 97) saw too few tests. Of the deviant follow-up plans created, 74% (n = 138) brought patients back to clinic too soon.

This study explores the deviation between current practice patterns and published clinical care guidelines. There is considerable variation across domain of analysis, diagnosis, and encounter type, resulting in uneven resource utilisation. Standardisation of care in these areas will improve utilisation and can be a starting point for improvement work.

Dysphagia is common in infants born with critical CHD. Thickened liquids are often used to treat dysphagia, but associated risks limit widespread use among feeding specialists. This survey aims to assess dysphagia treatment patterns and thickened liquid use across paediatric cardiac surgical centres.

A 24-question, cross-sectional survey. Convenience and snowball sampling methods were used to engage 52 paediatric cardiac surgical centres affiliated with the Cardiac Newborn Neuroprotective Network. Descriptive statistics were used to analyse and compare responses.

Twenty-six individual respondents represented 21 unique paediatric cardiac surgical centres. Most responses were from experienced, speech–language pathologists (78%) at medium size centres (88%). Ninety-three percent of responding centres used thickened liquids to treat dysphagia and 81% only after formal instrumental assessment of swallowing. Thickened oral feeding was used for single-ventricle patients by 85% versus 69% for two-ventricle patients. Barriers to recommending thickened oral feedings included the cost of thickening agents, parental non-adherence, and gastrointestinal concerns.

This is the first survey to report multi-institutional dysphagia treatment practice variation at United States congenital cardiac surgical centres. Thickened oral feedings are frequently used across centres in high-risk critical CHD patients but treatment benefit remains unclear. This survey highlights a broad scientific community poised to direct dysphagia research in critical CHD to address practice variation, short- and long-term impact of thickened oral feeding on feeding outcomes, and barriers to use and access of thickening agents.

Children with CHD are at increased risk for neurodevelopmental disabilities and neuropsychological impairments throughout their life span. The purpose of this report is to share our experience building a sustainable, novel, inpatient, interdisciplinary Neurocardiac Critical Care Program to mitigate risks and optimize outcomes during the ICU stay.

A descriptive review was chosen to identify meaningful characteristics, challenges and lessons learned related to the establishment, expansion of and sustainability of Neurocardiac Critical Care Program in a 26-bed pediatric cardiac ICU.

We successfully launched, expanded, and sustained an interdisciplinary Neurocardiac Critical Care Program. Here, we share the foundation, framework, challenges, and lessons learned as we established and sustained the Neurocardiac Critical Care Program. The key elements of our program are (1) consistent engagement by pediatric neurologists in the cardiac ICU, (2) comprehensive education initiatives, (3) evidence-based clinical practice changes, and (4) quality improvement and research projects.

The development of a pediatric Neurocardiac Critical Care Program is feasible and sustainable. This program was informed by recent research related to perioperative and psychosocial risk factors that impact brain development and neurodevelopmental outcomes in this vulnerable population. By aligning our efforts, our multidisciplinary team is helping shift the paradigm in pediatric cardiac critical care to actively manage complex heart disease, while simultaneously and proactively mitigating risks to the developing brain and family unit.

Vitamin D is crucial for normal organ function, vascular health and exercise performance, yet its deficiency is widespread. Patients with CHD often exhibit reduced exercise capacity. Limited research exists on vitamin D in CHD.

This study investigates serum 25-hydroxy vitamin D levels in 55 adult CHD patients (median age 31 years) compared to 55 age- and gender-matched controls without cardiac disease and examines associations with exercise capacity, peripheral microvascular function, muscle strength and biventricular function in CHD. Therefore, patients underwent fingertip arterial tonometry, transthoracic echocardiography, muscle strength measurements and cardiopulmonary exercise testing.

Results indicated that 93% of CHD patients and 91% of controls had 25-hydroxy vitamin D levels <30 ng/ml, with both groups showing varying values depending on the season in which the studies were conducted. No significant difference in 25-hydroxy vitamin D levels was found between patients and controls. While vitamin D levels in CHD patients did not significantly correlate with age, body mass index, blood pressure, peripheral microvascular function, high-sensitivity C-reactive protein, cholesterol levels, N-terminal-pro hormone B-type natriuretic peptide, ventricular function or muscle strength, a significant correlation was found with percent-predicted peak oxygen consumption (ρ=0.41, p = 0.005 and ρ=0.34, p = 0.02 for reference values following Wasserman and the LowLands registry, respectively), even after adjusting for season (p = 0.03 and 0.05, respectively).

In conclusion, vitamin D levels were similar between CHD patients and controls, but vitamin D insufficiency is common and linked to reduced exercise capacity in CHD. Further research is needed to determine whether vitamin D supplementation combined with exercise could be beneficial in CHD with vitamin D insufficiency.

Neurodevelopmental follow-up programmes for children with CHD help identify neurodevelopmental impairments and support the delivery of recommended interventions. The Cardiac Neurodevelopmental Outcome Collaborative, Diversity, Equity, and Inclusion Special Interest Group surveyed members to assess perceived patient barriers to neurodevelopmental follow-up, previous diversity and inclusion education, and confidence in caring for historically marginalised populations.

A link to a Redcap online survey was emailed to Cardiac Neurodevelopmental Outcome Collaborative members on 23 April 2022, with 4 weeks given to complete.

Eighty-four participants from 37 institutions in North America completed the survey. Respondents acknowledged that education on the importance of neurodevelopmental follow-up and frequent accommodations for cancellations or rescheduling clinic visits is essential. Language interpretation and written materials were available in languages other than English, but a limited number provided fully translated evaluation reports. Driving distance and the caregiver’s lack of understanding of the rationale for neurodevelopmental follow-up were the top perceived barriers to programme attendance. At the institutional level, training for cultural competency was typically provided, and most respondents felt comfortable caring for patients from diverse backgrounds. However, many agreed their programmes could do more to make evaluations accessible to historically marginalised/underserved populations.

Multiple barriers exist to cardiac neurodevelopmental follow-up, particularly for patients from under-represented minorities and for those whose primary language is not English. Surveying families will be valuable to understand how we may overcome these barriers. Further education about the importance of neurodevelopmental follow-up programmes continues to be essential.