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.

Racial disparities in healthcare have been well documented in the United States. We hypothesise that there will be a racial variance in different clinical variables in single-ventricle patients through stages of palliation.

Retrospective single-centre study stratified all single-ventricle patients who reached stage 2 palliation by race: Black and White. Other races were excluded. Demographic and clinical characteristics were compared, alongside follow-up survival data. Primary outcomes were progression to Fontan and overall survival.

Among 526 patients, 325 (61.8%) were White, and 201 (38.2%) were Black. Median age at stage 2 palliation was 150 days for White and 165 for Black patients (p = 0.005), with similar weights. Black patients exhibited higher median cardiopulmonary bypass times (87 vs. 74 minutes, p = 0.001) and a greater frequency of genetic syndromes (30.1% vs. 22.1%, p = 0.044). No significant differences were observed in outcomes between groups from stage 2 to stage 3, pre-stage 3 cardiac catheterisation variables, or perioperative outcomes. Multivariable regression analysis identified hypoplastic pulmonary arteries as the risk factor for mortality after stage 2. Survival analysis showed no difference in survival by race; however, occurrence of combined cardiovascular event was significantly higher in Black race.

Significant racial disparities exist among single-ventricle patients regarding the timing of stage 2 palliation, cardiopulmonary bypass duration, and frequency of genetic syndromes. Black race was a risk factor for sub-optimal long-term outcome, although perioperative mortality was similar. These race-related factors warrant further studies to improve our understanding of the impact of race on outcomes.

Ebstein’s anomaly represents 40% of congenital tricuspid valve abnormalities. Studies about paediatric Ebstein’s anomaly patients are limited.

To evaluate clinical characteristics, treatment (medical/arrhythmia ablation/surgical) results, and outcome of Ebstein’s anomaly patients, and to determine factors affecting arrhythmia presence and mortality.

Clinical data, echocardiography, treatment results, and outcomes of patients followed in our centre between 2000 and 2017 were retrospectively evaluated.

A total of 79 patients (61 children, median diagnosis age: 1.5 years [1 day–24 years]) were included. Eight patients (10.1%) were deceased during the study period. Common associated anomalies were atrial septal defect/patent foramen ovale (56.9%), mitral regurgitation (25.3%), pulmonary stenosis/atresia (17.7%), and ventricular septal defect (16.5%). Genetic diseases/congenital anomalies were present in 5/3.8%. Tricuspid regurgitation was present in 75.9%, and severe in 50%. Arrhythmias were detected in 30.4%, and accessory pathway-mediated re-entrant tachycardia was the most common (67%). Wolff-Parkinson-White syndrome was present in 12.7%. Twenty-one ablation procedures (radiofrequency ablation [85.7%]/cryoablation [14.3%]) were performed in 16 patients (median age: 13.3 years [4.9–17]). Acute success/recurrence rates: 87.5/25%. Surgery was performed in 31.6% (median age: 6.5 years [4 days–29 years]), 7.6% were operated during the first month, and 12.6% during the first year. Second surgery was required in 28%. Perioperative mortality rate was 12%, and median mortality age was 25 days (1 day–17 years). Median follow-up period was 5.3 years (1 day–32 years). Older diagnosis age (p = 0.005) and mild-moderate mitral regurgitation (p = 0.036) were associated with arrhythmias. Younger age at diagnosis (p = 0.012), younger age at first surgery (p = 0.004), surgery before age three years (p = 0.037), and presence of pulmonary atresia (p = 0.000014) were associated with mortality. Gender, diagnosis age, congenital anomalies/genetic disorders, tricuspid regurgitation, arrhythmias, and surgery history did not have an independent effect on survival.

In children and young adults presenting with Ebstein’s anomaly, younger age at presentation and at surgery, surgery before age three years, pulmonary atresia were associated with death. Ablation procedures can be successfully performed but recurrence rate is still high.

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.

The delivery of paediatric cardiac care across the world occurs in settings with significant variability in available resources. Irrespective of the resources locally available, we must always strive to improve the quality of care we provide to our patients and simultaneously deliver such care in the most efficient and cost-effective manner. The development of cardiac networks is used widely to achieve these aims.

This paper reports three talks presented during the 56th meeting of the Association for European Paediatric and Congenital Cardiology held in Dublin in April 2023.

The three talks describe how centres of congenital cardiac excellence can be developed in low-income countries, middle-income countries, and well-resourced environments, and also reports how centres across different countries can come together to collaborate and deliver high-quality care. It is a fact that barriers to creating effective networks may arise from competition that may exist among programmes in unregulated and especially privatised health care environments. Nevertheless, reflecting on the creation of networks has important implications because collaboration between different centres can facilitate the maintenance of sustainable programmes of paediatric and congenital cardiac care.

This article examines the delivery of paediatric and congenital cardiac care in resource limited environments, well-resourced environments, and within collaborative networks, with the hope that the lessons learned from these examples can be helpful to other institutions across the world. It is important to emphasise that irrespective of the differences in resources across different continents, the critical principles underlying provision of excellent care in different environments remain the same.

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.

Syncope is common among pediatric patients and is rarely pathologic. The mechanisms for symptoms during exercise are less well understood than the resting mechanisms. Additionally, inert gas rebreathing analysis, a non-invasive examination of haemodynamics including cardiac output, has not previously been studied in youth with neurocardiogenic syncope.

This was a retrospective (2017–2023), single-center cohort study in pediatric patients ≤ 21 years with prior peri-exertional syncope evaluated with echocardiography and cardiopulmonary exercise testing with inert gas rebreathing analysis performed on the same day. Patients with and without symptoms during or immediately following exercise were noted.

Of the 101 patients (15.2 ± 2.3 years; 31% male), there were 22 patients with symptoms during exercise testing or recovery. Resting echocardiography stroke volume correlated with resting (r = 0.53, p < 0.0001) and peak stroke volume (r = 0.32, p = 0.009) by inert gas rebreathing and with peak oxygen pulse (r = 0.61, p < 0.0001). Patients with syncopal symptoms peri-exercise had lower left ventricular end-diastolic volume (Z-score –1.2 ± 1.3 vs. –0.36 ± 1.3, p = 0.01) and end-systolic volume (Z-score –1.0 ± 1.4 vs. −0.1 ± 1.1, p = 0.001) by echocardiography, lower percent predicted peak oxygen pulse during exercise (95.5 ± 14.0 vs. 104.6 ± 18.5%, p = 0.04), and slower post-exercise heart rate recovery (31.0 ± 12.7 vs. 37.8 ± 13.2 bpm, p = 0.03).

Among youth with a history of peri-exertional syncope, those who become syncopal with exercise testing have lower left ventricular volumes at rest, decreased peak oxygen pulse, and slower heart rate recovery after exercise than those who remain asymptomatic. Peak oxygen pulse and resting stroke volume on inert gas rebreathing are associated with stroke volume on echocardiogram.

To assess the prevalence of obesity and investigate any changes in body mass index in children with CHD compared to age-matched healthy controls, in Southwestern Ontario.

The body mass index z-scores of 1259 children (aged 2–18) with CHD were compared with 2037 healthy controls. The body mass index z-scores of children who presented to our paediatric cardiology outpatient clinic from 2018 to 2021 were compared with previously collected data from 2008 to 2010. A longitudinal analysis of patients with data in both cohorts was also completed.

In total, 21.4% of patients with CHD and 26.6% of healthy controls were found to be overweight or obese (p < 0.001). The 2018–2021 cohort of CHD patients and controls had significantly higher body mass index z-scores compared to the 2008–2010 cohort (p < 0.001). Longitudinal analysis showed that body mass index z-scores significantly increased over time for CHD patients with data in both cohorts (2018–2021: M = 0.59, SD = 1.26; 2008–2010: M = −0.04, SD = 1.05; p < 0.001).

The prevalence of obesity in all children, irrespective of CHD, is rising. The coexistence of obesity and CHD may pose additional cardiovascular risks and complications.

The Wolff–Parkinson–White pattern is a delta wave frequently detected in school-based cardiovascular screening programs in Japan. Although most children with Wolff–Parkinson–White pattern are asymptomatic, initial symptoms may include syncope or sudden death, necessitating accurate diagnosis and management. Delta waves can also indicate a fasciculoventricular pathway, which poses no risk and does not require management.

We reviewed the medical records of patients referred to our hospital between April 2008 and March 2022 to evaluate the electrocardiographic signs of the Wolff–Parkinson–White pattern. The existence of Wolff–Parkinson–White syndrome and fasciculoventricular pathway were determined based on atrioventricular block and QRS waveform changes after adenosine administration during sinus rhythm.

The study cohort included 127 children (65 males; median age: 12.8 years, resting heart rate: 75 beats/min, PR interval: 109 ms, and QRS duration: 101 ms). The adenosine administration test revealed a fasciculoventricular pathway, Wolff–Parkinson–White syndrome, and indeterminate findings in 64, 54, and 9 children, respectively. More than 60% of children with a QRS duration ≤ 120 ms had a fasciculoventricular pathway. Age ≤ 12 years, QRS duration >120 ms, and type A category (children with R/S ratios >1 in lead V1) were identified as independent risk factors for Wolff–Parkinson–White syndrome. No adverse events were observed in any child.

The adenosine administration test is safe and feasible for differentiating Wolff–Parkinson–White syndrome from fasciculoventricular pathways and can reduce the unnecessary management of children with fasciculoventricular pathways.

High takeoff of the right coronary artery suspected by echocardiography is widely considered a normal variant. However, in our experience, some patients initially thought to have a high takeoff of the coronary artery were later found to have an anomalous coronary origin with high-risk features. The aim of this study was to test the hypothesis that high takeoff of the right coronary artery suspected by echocardiography may indicate the presence of an anomalous coronary artery lesion with an intramural course requiring further investigation.

A retrospective chart review was performed of patients evaluated at the University of Florida Congenital Heart Center from January 2010 through September 2015. Charts of all 62 patients who were noted to have an anomalous coronary artery or concern for an anomalous coronary artery were reviewed to identify those who were initially identified as having simply a high takeoff of the right coronary artery by initial echocardiogram. A total of 24 patients met these criteria.

Out of 24 patients identified as having high takeoff of the right coronary artery on their initial echocardiogram, 20 had confirmatory computerized tomographic angiography. On review of these patients, 9 had a right coronary origin from the left. This included 3 patients with an anomalous right coronary artery from the left coronary sinus and 6 with an anomalous right coronary artery origin just above the left coronary sinus. Six of these 9 patients had an intramural course. The remaining patients had high takeoff above the right coronary sinus or normal coronary origins.

Additionally, on review of all patients with computerized tomographic angiographic confirmation of high takeoff of the coronary artery, those with high takeoff above the left coronary sinus were more likely to have an intramural course (6 out of 9). Meanwhile, none of the 6 patients with high takeoff above the right coronary sinus confirmed by computerized tomographic angiography had an intramural course.

Accurate identification of the coronary origin and course of the anomalous coronary artery is difficult by echocardiogram. Correct diagnosis of origin and course is important for appropriate risk stratification and treatment decisions. Therefore, patients with high takeoff of the right coronary artery suspected by echocardiography should undergo additional evaluation to assess for the presence of a potentially malignant course.