Pulmonary atresia with intact ventricular septum is a rare congenital cardiac lesion with significant anatomical heterogeneity. Surgical planning of borderline cases remains challenging and is primarily based on echocardiography. The aim was to identify echocardiographic parameters that correlate with surgical outcome and to develop a discriminatory calculator.

Retrospective review of all pulmonary atresia with intact ventricular septum cases at a statewide tertiary paediatric cardiac centre was performed between 2004 and 2020. Demographic, clinical, and echocardiographic data were collected. Logistic regression was used to develop a discriminatory tool for prediction of biventricular repair.

Forty patients were included. Overall mortality was 27.5% (n = 11) and confined to patients managed as univentricular (11 vs 0, p = 0.027). Patients who underwent univentricular palliation were more likely to have an associated coronary artery abnormality (17 vs 3, p = 0.001). Fifteen surviving patients (51.7%) achieved biventricular circulation while 14 (48.3%) required one-and-a-half or univentricular palliation. Nineteen patients (47.5%) underwent percutaneous pulmonary valve perforation. No patients without tricuspid regurgitation achieved biventricular repair. The combination of tricuspid valve/mitral valve annulus dimension ratio and right ventricle/left ventricle length ratio identified biventricular management with a sensitivity of 93% and specificity of 96%. An online calculator has been made available.

Pulmonary atresia with intact ventricular septum is a challenging condition with significant early and interstage morbidity and mortality risk. Patient outcomes were comparable to internationally reported data. Right ventricle/left ventricle length and tricuspid valve/mitral valve annulus dimension ratios identified a biventricular pathway with a high level of sensitivity and specificity. Absent tricuspid regurgitation was associated with a univentricular outcome.

The most common cause of morbidity and mortality in type 1 diabetes mellitus is cardiovascular system involvement. Sudden death has been reported in type 1 diabetes mellitus patients. To analyse the use of the cardiac electrophysiological balance index in predicting ventricular arrhythmias in children with type 1 diabetes mellitus disease.

One hundred type 1 diabetes mellitus paediatric patients and 100 healthy children, divided into three groups according to their haemoglobin A1C levels, were included in the study. All participants were evaluated with transthoracic echocardiography, 12-lead electrocardiography, and 24-h Holter after a detailed physical examination. Systolic and diastolic function parameters, electrocardiography intervals, and heart rate variability parameters were evaluated.

The mean age in the type 1 diabetes mellitus and control groups was 11.52 (± 3.52) and 11.78 (± 3.45) years, respectively. In total, 57% of the type 1 diabetes mellitus group and 56% of the control group were male. The mean haemoglobin A1C value was 9.14% (± 1.79) and the disease duration was 3.71 years (± 3.13). The type 1 diabetes mellitus group had a higher QTc duration, deceleration time duration, A velocity, and a lower E/A ratio than the control group. In the type 1 diabetes mellitus group, Tpe, Tpe/QT ratio, QTc/QRS ratio, Tpe/QRS ratio, Tpe/(QT × QRS) ratio values were significantly higher than in the control group.

Children with type 1 diabetes mellitus are at risk for atrial and ventricular arrhythmias without valvular disease, ischemic heart disease, or diastolic dysfunction despite normal systolic function. No correlation was found between disease duration, metabolic control, autonomic function parameters, and arrhythmia risk; however, it was associated with diastolic function parameters.

Acquired chylothorax is an established complication of CHD surgery, affecting 2–9% of patients. CHD places a child at risk for failure to thrive, with subsequent chylothorax imposing additional risk.

We conducted a retrospective chart review to ascertain quantitative markers of nutrition and growth in children affected by chylothorax following CHD surgery between 2018 and 2022 compared to controls.

We utilised electronic medical record system, EPIC, at Children’s Hospital, New Orleans, targeting subjects < 18 years old who underwent CHD surgery between 2018 and 2022 and developed a subsequent chylothorax. Study subjects were identified using the 10th revision of the International Classification of Diseases codes (ICD-10 codes: J94.0, I89.8, and J90.0). Each chylothorax case (n = 20) was matched by procedure type and age to a control with no chylothorax (n = 20). Data were recorded in REDCap and analysed using SPSS.

After removal of outliers, we analysed 19 total matched pairs. There was no statistical difference in growth velocity (p = 0.12), weight change (operation to discharge) (p = 0.95), weight change (admission to discharge) (p = 0.35), Z-score change (operation to discharge) (p = 0.90), Z-score change (admission to discharge) (p = 0.21), serum protein (p = 0.88), or serum albumin (p = 0.82). Among cases, linear regression demonstrated no significant association between maximum chylous output and growth velocity (p = 0.91), weight change (operation to discharge) (p = 0.15), or weight change (admission to discharge) (p = 0.98).

We did not observe statistically significant markers of growth or nutrition in children with chylothorax post-CHD surgery compared to those without chylothorax. Multisite data collection and analysis is required to better ascertain clinical impact and guide clinical practice.

Radiation exposure and the effect of anti-scatter grids are not well studied in premature infants during transcatheter patent ductus arteriosus closure.

This study aimed to investigate whether the use of anti-scatter grids altered the level of radiation exposure to premature infants undergoing transcatheter patent ductus arteriosus closure.

Demographic and radiation exposure data for premature infants who underwent transcatheter patent ductus arteriosus closure at the Stead Family Children’s Hospital from 10/2019 to 10/2021 were retrospectively reviewed and analyzed. Twenty-four patients (41%) underwent transcatheter patent ductus arteriosus closure with anti-scatter grids, while 34 (59%) underwent the procedure without using anti-scatter grids. At the time of catheterization, the median age, corrected age, and weight were 4.3 weeks (3.4–6), 29 weeks (28.1–30.9), and 1200g (1000-1600), respectively. Total radiation exposures for the dose area product and air kerma were 2.73 µGy.m2 (1.65–4.16), and 1.63 mGy (1.15–2.58), respectively. Radiation doses were higher in the group in whom the anti-scatter grids were utilized with dose area product of 3.33 µGy.m2 (2.39–5.43) and air kerma of 2.27 mGy (1.41–3.06) versus 1.86 µGy.m2 (1.46–3.60) and air kerma of 1.40 mGy (1.08–1.92). When radiation doses were adjusted to the radiation time, no difference in radiation exposure was noticed between the groups.

Transcatheter patent ductus arteriosus closure in premature infants can be safely performed with minimal radiation exposure. In the authors’ laboratory, the use of anti-scatter grids does not impact radiation exposure in premature infants.

Cardiac intensive care providers require a comprehensive understanding of cardiac output and oxygen delivery. The estimation of cardiac output in clinical practice often relies on thermodilution and the Fick principle. Central venous saturation and lactate levels are commonly used indicators for cardiac output assessment. However, the relationship between venous lactate levels and venous oxygen saturation in paediatric cardiac intensive care patients remains unclear.

This is a single-centre retrospective pilot study aimed to investigate the correlation between venous lactate and venous oxygen saturation in paediatric patients. Data collected included venous saturation, heart rate, mean arterial blood pressure, arterial saturation by pulse oximetry, cerebral and renal near-infra-red spectroscopy values, and the presence of a functionally univentricular heart. Statistical analyses included Bayesian Pearson correlation and regression analyses.

A total of 203 data points from 37 unique patients were included in the analysis. There was no significant correlation between serum lactate and venous saturation (correlation coefficient = –0.01; Bayes factor 10 = 0.06). Serum lactate also did not correlate with other haemodynamic metrics. Venous saturation showed correlations with arterial saturation and cerebral and renal near-infra-red spectroscopy. Regression analysis revealed that parallel circulation, arterial saturation, and cerebral near-infra-red spectroscopy were predictive of venous saturation. The following equation resulted from the regression analysis: 68.0 – (12.7 x parallel circulation) – (0.8 x arterial saturation) + (0.3 x cerebral near-infra-red spectroscopy). This model had a Bayes factor 10 of 0.03 and adjusted R-squared was 0.29.

In paediatric cardiac intensive care patients, there is no significant correlation between venous lactate and venous saturation, suggesting that lactate may not be a reliable marker for assessing the adequacy of oxygen delivery in this population. Only a weak correlation could be identified once the venous saturation was 70% or lower. Additional research is needed to explore alternative markers for monitoring oxygen delivery in critically ill paediatric patients.

Procedure duration is an important predictor of patient outcomes in surgery. However, the relationship between procedure duration and adverse events in congenital cardiac catheterization is largely unexplored.

All cases entered into the Congenital Cardiac Catheterization Project on Outcomes from 2014 to 2017 were included. Cases were ordered from shortest to longest case length, minus time spent managing adverse events, for each case type. The outcomes, Level 3bc/4/5 and 4/5 adverse event rates, were calculated for cases above and below the 75th percentile for case length. To identify an independent relationship between case length and outcomes, the case length percentile was added to the CHARM II risk model.

Among 14,704 catheterizations, longer cases (>75th percentile for case length) had Level 4/5 rates that were 2.2% and 2.7% compared to cases ≤75th percentile with adverse event rates of 0.9% and 1.4% for diagnostic and interventional cases, respectively. Level 3bc/4/5 rates were 5.0% and 8.4% in longer cases compared to 2.4% and 5.4% for diagnostic and interventional cases, respectively. After adding case length to the CHARM II risk model, case length 50th–75th percentile had an odds ratio (OR) of 1.4, 75th–90th percentile an OR of 1.56, and >90th percentile an OR of 2.24 as compared to cases with case length <50th percentile (p ≤ 0.001 for all).

Longer case lengths are associated with clinically important and life-threatening adverse events in congenital cardiac catheterization, even after accounting for known risk factors. Case length may be an important target for future quality improvement work.

Sudden cardiac death is a significant concern among patients with congenital heart disease (CHD). We assessed the risk of remote sudden cardiac death after congenital heart surgery.

Patients undergoing congenital heart surgery before 21 years of age between 1982 and 2003 in the Pediatric Cardiac Care Consortium registry were linked to National Death Index data through 2019. Sudden cardiac death was defined as death associated with a cardiac arrest or ventricular fibrillation diagnosis code. Standardised mortality ratios relative to the general population were calculated using Centers for Disease Control and Prevention data.

Among 30,566 patients discharged after their initial surgery, 2,718 deaths occurred over a median period of 23 years (IQR 19–27). Of 463 (17%) sudden cardiac deaths, the median age was 1.7 years (IQR 0.5–16.5). The mean incidence was 7 per 10,000 person-years (95% CI: 0.64–0.77), ranging from 2.7 for left-to-right shunt lesions to 37 for single-ventricle physiology. Cardiac comorbidities including heart failure (13.6%) and arrhythmias (7.1%) were more frequent among sudden cardiac death patients. Standard mortality ratios for sudden cardiac death were elevated across all CHD types, ranging from 8.0 (95% CI: 6.3–9.6) for left-to-right shunts to 107.7 (95% CI: 88.9–126.5) for single-ventricle physiology.

Sudden cardiac death risk is higher post-congenital heart surgery compared to the general population. Even patients with mild CHD are at risk, highlighting the need for long-term follow-up for all patients. Heart failure and arrhythmia prevalence suggest potential therapeutic targets to reduce sudden cardiac death risk.

The population of adult CHD patients is continuously increasing. The underlying CHD affects performance and prognosis, but also has a significant impact on quality of life, psychosocial behaviour, anxiety and emotional disturbances. This study analyzes these parameters of patients after one or more heart operations and the possible psychological effects of medical and psychosocial complications at the Department of Cardiology of the Kepler University Hospital Linz.

A total of 81 subjects participated in the questionnaire survey of the Institute of Cardiology and Clinical Psychology during their annual cardiological check-up. Of these, 80 participants were included in the study and three showed a mild CHD, 49 a moderate one, and 28 a severe one. This study has an exploratory design to assess possible stress factors and limitations in quality of life. For this purpose, a self-administered sociodemographic questionnaire and three standardised questionnaires were used. In summary, the quality of life of adult CHD is considered depending on the severity of the symptoms and compared with the healthy population. Differences in this regard are observed in individuals with lower symptom severity, who report higher psychological well-being. Sex differences are observed in physical role function and physical functioning.

Based on the results, regular repetitions of the study, as well as continuous psychological and psychosocial support, are necessary, since challenges are predictable with the increasing age of adult CHD patients and since the upholding of good quality of life and dealing with difficult life circumstances must be supported.

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.

Children with congenital adrenal hyperplasia are considered to be at an elevated risk for cardiovascular morbidity and mortality. The objective of this study was to evaluate the association between periaortic fat thickness and the cardiometabolic profile in children diagnosed with congenital adrenal hyperplasia.

A total of 20 children and adolescents with congenital adrenal hyperplasia and 20 healthy control subjects were enrolled in the study. We investigated metabolic and anthropometric parameters, comparing these values to those of the control group. Periaortic fat thickness was assessed using an echocardiographic method that has not previously been applied to paediatric patients with congenital adrenal hyperplasia.

The subjects in the congenital adrenal hyperplasia group were significantly shorter than the control subjects (p = 0.021) and exhibited a higher body mass index (p = 0.044) and diastolic blood pressure (p = 0.046). No significant differences were observed between the congenital adrenal hyperplasia group and control subjects concerning age, weight, high-density lipoprotein cholesterol, and low-density lipoprotein cholesterol levels. Additionally, dyslipidemia was identified in 5% (N = 1) of the congenital adrenal hyperplasia group. The mean fasting glucose, fasting insulin, homeostasis model assessment of insulin resistance, and fasting glucose-to-fasting insulin ratio were similar between the congenital adrenal hyperplasia group and the control subjects. However, 15% (n = 3) of the congenital adrenal hyperplasia group had insulin resistance. Two children with congenital adrenal hyperplasia (10%) were diagnosed with hypertension.

Periaortic fat thickness was significantly greater in the congenital adrenal hyperplasia group compared to the control group (p = 0.000), with measurements of 0.2039 ± 0.045 mm in the congenital adrenal hyperplasia group and 0.1304 ± 0.022 mm in the control group. In children with congenital adrenal hyperplasia, periaortic fat thickness exhibited a negative correlation with high-density lipoprotein cholesterol (r = −0.549, p = 0.034) and a positive correlation with the dose of hydrocortisone (r = 0.688, p = 0.001).

Our results provide further evidence of subclinical cardiovascular disease in children with congenital adrenal hyperplasia. It is crucial to regularly assess cardiometabolic risk in children with congenital adrenal hyperplasia. The measurement of periaortic fat thickness in this population may serve as a valuable tool for identifying individuals at high risk for developing early atherosclerosis.

A subgroup of CHDs can only be treated palliatively through a Fontan circulation. In case of a failing Fontan situation, serum proteins are lost unspecifically and can also lead to a loss of vaccine antibodies. In a failing Fontan situation, heart transplantation may be the only feasible option.

We describe a 17-year-old patient born with a hypoplastic left heart complex, who underwent Fontan completion at the age of 4 years and developed a failing Fontan physiology. Therefore, a Fontan takedown with creation of a reverse 1½-circulation was performed. Multiple exacerbations of protein losing enteropathy occurred with a hypoproteinaemia, hypalbuminaemia, and hypogammaglobulinaemia. The patient was hospitalised several times and treated with intravenous application of immunoglobulins and albumin for symptom control. Before one of this substitutions, the immunoglobulin G against measles, mumps, and rubella was determined: the patient’s serum demonstrated a positive measles and rubella, but mumps was negative. After administration of the iv-therapy, the lacking antibodies were replenished, and there was a positive test for mumps.

Serum samples were analysed by neutralisation test and enzyme-linked immunosorbent assay (ELISA).

Although the patient had been vaccinated according to national guidelines, we saw an intermittent immune deficiency for mumps, but not for rubella and measles. For patient with a failing Fontan circulation, we recommend to test to vaccine antibodies for mumps, measles, and rubella with an ELISA an if its negative with a neutralisation test, especially in view of a possible heart transplantation to find a possible immune deficiency.

Understanding complex three-dimensional cardiac structures is the key to knowing CHD. Many learners have limited access to cadaveric specimens, and most alternative teaching modalities are two-dimensional. Therefore, we have developed virtual cardiac models using photogrammetry of actual heart specimens to address this educational need.

A descriptive study was conducted at a single institution during a week-long cardiac morphology conference in October 2022 and 2023. Conference attendees viewed virtual cardiac models via laptop screen and virtual reality headset. Learners were surveyed on their opinions of the virtual models and their perceived effectiveness compared to existing educational materials.

Forty-six learners completed the survey. Participants reported the virtual cardiac models to be more effective than textbook diagrams (60%), and equally or more effective compared to didactic teaching (78%) and specimen videos (78%). Approximately half of participants (54%) found the virtual models to be less effective than hands-on cadaveric specimen inspection. Attitudes towards the virtual specimens were overall positive with most responders finding the tool engaging (87%) and enjoyable (85%). A majority reported that the models deepened their understanding of cardiac morphology (79%) and that they would recommend them to other trainees (87%).

This study demonstrates that a novel teaching tool, virtual cardiac specimens, is equivalent to or more effective than many current materials for learning cardiac morphology. While they may not replace direct cadaveric specimen review, virtual models are an engaging alternative with the ability to reach a wider audience.