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.

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.

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.

In the presence of fetal cardiomegaly, when there is no cardiac malformation or dysfunction, systemic or pulmonary arteriovenous malformations that may cause volume loading should be sought. We aimed to present a fetus who had cardiomegaly and left pulmonary artery-left atrial fistula and who underwent transcatheter closure in the early postnatal period.

23-week fetus referred because of severe cardiomegaly on screening obstetric ultrasonography. Fetal echocardiography revealed fistulous connection between dilated left pulmonary artery and left atrium with high velocity continuous flow at the left atrial orifice of fistula and retrograde flow from the ductus arteriosus to the pulmonary artery. Initially, the fetus followed by one-to-two weeks intervals for fetal heart failure and hydrops fetalis. Pregnancy was uneventful and the baby was born by caesarean section at 37 weeks, and oxygen saturation level was 95 %. Transthoracic echocardiography confirmed the prenatal diagnosis of a fistula between the left pulmonary artery and the left atrium (CTA showed left lung aplasia. Transcatheter closure was performed from antegrade route with Amplatzer Piccolo® Duct Occluder due to hypoxaemia. The baby showed normal growth and development at 15 months of ageThere is no pulmonary hypertension during the 15-month follow-up.

Pulmonary artery-to-left atrial fistula is a rare anomaly and is frequently described between the right pulmonary artery and the left atrium. Presentation of age depends on the size of the fistulous connection. Patients with large connections are presented in fetal age with cardiomegaly and heart failure or presented in early infancy with profound cyanosis. Although lung hypoplasia has been reported in patients with pulmonary artery-to-left atrial fistula/connection lung aplasia has never been reported in these patients. Surgical or transcatheter closure can be achieved successfully in these patients at neonatal period or early infancy like in our case.

Pulmonary oedema is a common complication after paediatric cardiac surgery, and it is linked to increased morbidity. Lung ultrasound has been recognised as an alternative to chest radiography, offering rapid and accurate diagnosis without exposure to ionising radiation.

The study aimed to investigate the association between the severity of pulmonary oedema identified through a B-lines score—a lung ultrasound score used to assess the severity of pulmonary oedema—and ventilatory outcomes in the postoperative period of cardiac surgery. It was a prospective single-centre cohort study conducted at a quaternary paediatric hospital. Patients up to 18 years old who underwent cardiac surgery for CHD were included. The primary outcome was extubation failure within 48 hours after surgery, and the secondary outcome was mechanical ventilation time.

Among the 131 included patients, 116 were extubated with an extubation failure rate of 18.3%. Patients with extubation failure were younger and had a longer duration of mechanical ventilation. A robust association between higher B-lines score on lung ultrasound within the first 24 hours after surgery and extubation failure was observed (p < 0.001). The correlation between B-lines score and mechanical ventilation time was positive, with stronger correlation preoperatively.

Severe pulmonary oedema detected through lung ultrasound in the first postoperative day of paediatric cardiac surgery show better accuracy to predict patients at greater risk of extubation failure and prolonged mechanical ventilation.

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.

Parvovirus B19 (PVB19) myocarditis is a life-threatening condition with high morbidity and mortality in children. While electrocardiograms are commonly used in the early assessment of myocarditis, no specific electrocardiogram pattern has been consistently linked to PVB19. The objective of this study is to identify a distinctive electrocardiogram pattern associated with PVB19 myocarditis and evaluate its diagnostic accuracy.

This retrospective case–control study included 77 paediatric patients diagnosed with acute myocarditis at a single centre in Barcelona over 16 years (August 2008–September 2024). Twenty patients had PVB19 myocarditis, confirmed by polymerase chain reaction in blood or endomyocardial biopsy, while 57 patients had myocarditis caused by other viruses. Electrocardiogram were assessed by three cardiologists blinded to the aetiological diagnosis.

A specific electrocardiogram pattern in the limb leads, characterised by peaked P waves, low QRS complex voltages, and altered repolarisation (manifesting as negative or flat T waves, with or without QTc prolongation), was observed in 14 of 20 patients (70%) with PVB19 myocarditis. Two additional patients exhibited low voltages and altered repolarisation without peaked P waves, and all demonstrated repolarisation abnormalities. In contrast, only 1 of 57 patients with myocarditis from other viruses exhibited the full electrocardiogram pattern. The pattern demonstrated a specificity of 98% and a sensitivity of 70% for PVB19 myocarditis.

The identified electrocardiogram pattern shows strong diagnostic specificity for PVB19 myocarditis in paediatric patients and may serve as a useful early diagnostic tool. Further multicentre studies are needed to confirm these findings and explore their clinical implications.

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.