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.

Patients diagnosed with hypertension (HT) are at high risk for end-organ damage. With changing living conditions and access to healthcare facilities worldwide, the rate of diagnosis in childhood is increasing. In this study, healthy children were compared with a group of pediatric patients diagnosed with hypertension. Cardiac findings in the hypertensive group were compared at presentation and at six months. We aimed to determine the discriminatory value of epicardial adipose tissue (EAT) measurements as an early imaging marker for cardiac involvement in children with HT compared to healthy children and to determine its prognostic feature for HT treatment response.

Fifty-nine primary hypertension patients and 76 control patients aged 0-18 years were compared. EAT values measured between the healthy group and the patient group and at the beginning of treatment and subsequent follow-ups in the patient group were evaluated with M-mode measurements.

There was no difference between the groups in terms of sex, and age. EAT was found to be significantly higher in the patient group than in the healthy group. There was a statistically significant difference between the EAT measurements evaluated before and after treatment in the patient group.

Hypertension is an important cause of morbidity and mortality. Using EAT measurements as a noninvasive parameter may provide information about early cardiac involvement due to HT. EAT is promising as an imaging marker that can be used in diagnosis and follow-up.

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.

Atherosclerosis is the leading cause of vascular disease worldwide, and traditionally it has been considered a disease of older individuals. However, this atherosclerotic process begins early in childhood, and when exposed to critically high levels of atherogenic risk factors, coronary artery disease may develop even during childhood. There are very few reports of coronary artery disease in young children, and most are linked to Kawasaki disease and congenital coronary abnormalities. Involvement of the mitral valve due to hypercholesterolaemia is rare and under-reported.

We did a retrospective audit of all children (age <14 years) who underwent coronary angiogram between January 2005 and July 2024 in our tertiary care hospital. Only those children with atherosclerotic coronary artery disease were included.

We studied four paediatric cases of atherosclerotic coronary artery disease with concomitant valvular involvement despite ongoing lipid-lowering therapy. We highlight the mechanisms of valvular involvement and the challenges to the diagnosis and treatment of familial hypercholesterolaemia.

These cases highlight the cardiovascular changes associated with this “malignant” atherosclerosis and emphasise the need for early recognition and prompt initiation of aggressive lipid-lowering therapy at diagnosis.

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.

Children with CHD are at risk of neurodevelopmental impairment. Modifiable risk factors associated with hospitalisation that could impact neurodevelopment include being left alone for long periods of time with minimal interaction or opportunity to engage in developmentally appropriate play. Volunteers are an underutilised resource to help the medical team and families support neurodevelopment in cardiac care. Our Cardiac Inpatient Neurodevelopmental Care Optimization or CINCO team aimed to develop a volunteer programme specific to paediatric cardiac inpatient units.

CINCO volunteers were recruited from the hospital volunteer pool and, in 2022, partnered with the University of Colorado to recruit health profession-interested students from under-represented backgrounds. All underwent hospital volunteer orientation and CINCO-specific training with cardiac child life, including education and shadowing. Volunteers completed an activity log and provided qualitative feedback.

Between September 2021 and October 2024, 43 volunteers were onboarded and worked a total of 754 shifts. There were 2310 patient interactions, with an average of 3 patients seen per shift. Volunteers held patients 1231 times, played with patients 1230 times, and read to patients 780 times.

A dedicated cardiac volunteer programme is a feasible, low-cost, and low-risk way to enhance neurodevelopmental care for inpatient children with CHD. When parents or caregivers are not present, volunteers participate as therapy extenders and may offset the care burden for nurses. Furthermore, allowing parents breaks may support their mental health, and increasing neurodevelopmental stimulation through volunteer interactions may mitigate disadvantageous aspects of a hospitalisation for neurodevelopment.

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.

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.

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.