Ductal stenting in late presenters with transposition of great arteries with intact ventricular septum retrains the left ventricle before arterial switch operation. However, the experience is limited for its efficacy and safety. This study aims to highlight the efficacy and safety of ductal stenting for retraining the left ventricle.

Eight children with transposition of great arteries-intact ventricular septum and regressed left ventricle underwent ductal stenting. Serial echocardiographic measurements of left ventricle shape, mass, volume, free wall thickness, and function were done, and arterial switch operation was performed once the left ventricle was adequately prepared. Post-operative outcome in terms of duration of mechanical ventilation, ICU stay, and improvement in left ventricle function were monitored.

The procedure was successful in all patients. Babies were divided into two groups on basis of age at ductal stenting (group 1 age less than 90 days and group 2 age more than 90 days) and were evaluated for the degree of left ventricle retraining as evidenced by echocardiographic parameters and post-operative variables. The left ventricle posterior wall thickness and mass index after ductal stenting increased significantly in both the groups. Postoperatively, one baby of group two expired after seven days due to severe left ventricle dysfunction. Rest babies had an uneventful post-operative ICU stay with no statistical difference in the duration of invasive mechanical ventilation or ICU stay. On six-month follow-up, all surviving babies were doing well with normal left ventricle function.

Ductal stenting is a good alternative measure as compared to surgical procedures for left ventricle retraining in transposition of great arteries with regressed left ventricle.

The aims of this study were to evaluate the 16-year experience with  arterial switch operation at Beijing Children’s Hospital and to determine early and late mortality and late morbidity, to explore risk factors for late complications and reintervention, and finally to evaluate whether the neoaortic sinotubular junction reconstruction technique reduces late complications of arterial switch operation.

The clinical data of 185 patients with transposition of the great arteries who underwent arterial switch operation in Beijing Children’s Hospital from January 2006 to January 2022 and 30 patients who underwent modified arterial switch operation with neoaortic sinotubular junction reconstruction technique in Fuwai Hospital during the same period were retrospectively analysed. Propensity score matching was also used to match the neoaortic sinotubular junction reconstruction patients in Fuwai Hospital with 30 non-neoaortic sinotubular junction reconstruction patients in Beijing Children’s Hospital.

There were 13 early deaths (7.03%) and five late deaths (3.01%). Nineteen patients (11.45%) developed new aortic valve regurgitation and 28 patients (16.87%) developed aortic root dilation. Late right ventricular outflow tract obstruction occurred in 33 patients (19.88%). Late reintervention occurred in 18 cases (10.84%). Multivariate analysis showed that aorto-pulmonary diameter mismatch, previous pulmonary artery banding, and mild moderate or above new aortic valve regurgitation at discharge were independent risk factors for late new aortic valve regurgitation and aortic root dilation. Low surgical weight was an independent risk factor specific to new aortic valve regurgitation, and bicuspid native pulmonary valve was an independent risk factor specific to aortic root dilation. Older surgical age and aortic root dilation were independent risk factors for late right ventricular outflow tract obstruction. Older surgical age, operation before 2014, late right ventricular outflow tract obstruction, and late aortic root dilation were independent risk factors for late intervention. Propensity score matching showed that new aortic valve regurgitation and aortic root dilation were not followed up in the neoaortic sinotubular junction reconstruction group, while seven cases of aortic root dilation and five cases of new aortic valve regurgitation occurred in the non-neoaortic sinotubular junction reconstruction group, respectively, and the differences were statistically significant (P = 0.003; P = 0.015).

The increased incidence of new aortic valve regurgitation, aortic root dilation, and right ventricular outflow tract obstruction as children age is a major concern outcome in the future and may mean more late reintervention. neoaortic sinotubular junction reconstruction technique may reduce the incidence of new aortic valve regurgitation and aortic root dilation, and improve the late prognosis of arterial switch operation. Careful follow-up of neo-aortic valve and root function is imperative, especially in patients with aorto-pulmonary diameter mismatch, previous pulmonary artery banding, mild new aortic valve regurgitation at discharge, low surgical weight, and bicuspid native pulmonary valve structures.

Congenital Heart Defects are the most common abnormalities at birth, resulting in many short- and long-term consequences.

In patients with transposition of the great arteries, surgical correction may achieve definitive treatment, so a thorough knowledge of the long-term outcomes, particularly neurodevelopment outcomes, is essential. Therefore, we conducted a systematic review and meta-analysis to study the neurodevelopment outcomes in the first 5 years of the life of children submitted to corrective surgery for transposition of the great arteries in the neonatal period.

A total of 17 studies from 18 reports were included, assessing 809 individuals with surgically corrected transposition of the great arteries. The neurodevelopmental outcomes were assessed with the Bayley Scales of Infant and Toddler Development (BSID) and the Wechsler Intelligence Scale for Children (WISC).

Mean Mental Development Index (MDI) and Psychomotor Development Index (PDI) were within the average values from 1 to 3 years of age, although the proportion of children scoring more than 1 standard deviation below the mean in PDI, MDI, motor, and language composite scores was significantly higher than in the general population. From 4 to 5 years, mean full-scale global intelligence quotient (IQ), verbal IQ, and performance IQ scores did not differ significantly from the general population.

This study revealed neurodevelopment scores within the normal range at 5 years of age in children submitted to corrective surgery for transposition of the great arteries in the neonatal period. However, these early outcomes may not adequately predict long-term outcomes. Further studies are needed to identify specific risk factors and early markers of later impairment to guide the establishment of early interventions.

Transposition of the great arteries is a severe CHD that affects term neonates. The presence of major aortopulmonary collateral arteries in neonatal transposition of the great arteries patients is rare. This study investigated the clinical and haemodynamic implications of the presence of major aortopulmonary collateral arteries in neonatal transposition of the great arteries patients who underwent an arterial switch operation.

The study was a retrospective analysis conducted on neonates diagnosed with transposition of the great arteries who underwent arterial switch operation within the period from 1 May 2020 to 1 January 2023 at two high-patient-volume paediatric cardiac surgery centres in Turkey. The patients’ demographic characteristics, echocardiographic features, and clinical data were analysed. Additionally, the possible clinical effects of the presence of major aortopulmonary collateral arteries were statistically evaluated.

Two hundred cases of neonatal transposition of the great arteries were included in this study, with 55% of the cases male. All the patients underwent arterial switch operation. The median age at the time of arterial switch operation was 5 days (interquartile range 3–7), with a median weight of 3,100 g (interquartile range 2,900–3,400). The median pre-operative saturation level was 76% (interquartile range 70–82%). Prior to arterial switch operation, 32 patients underwent balloon atrial septostomy.

In all the patients, the interatrial septum was checked to determine if the atrial septum was intact. A patent foramen ovale (≤ 3 mm) was found in 112 patients, and a non-restrictive atrial septal defect (> 3 mm) was found in 88. Forty-eight patients had ventricular septal defects, and 72 had coronary anomalies. Major aortopulmonary collateral arteries were found in 4 patients pre-operatively and in 12 patients after arterial switch operation (echocardiography, n = 8; angiography, n = 4). Of the patients with post-operative detection of cumulative number of major aortopulmonary collateral arteries were on post-operative day 1 in 2 patients, on post-operative day 3 in 5 patients, on post-operative day 7 in 6 patients, and on post-operative day 14 in 11 patients.

Transcatheter closure was performed in 3 cases due to recurrent extubation failure. Major aortopulmonary collateral artery shrinkage was observed in one case under medical treatment. The length of paediatric cardiac intensive care unit stay (10 days versus 8 days; p < 0.005), mechanical ventilator time (4 days versus 2 days; p = 0.02), and inotrope use time (5 days versus 3 days; p = 0.04) were higher in the major aortopulmonary collateral artery cases than patients without major aortopulmonary collateral artery.

Major aortopulmonary collateral arteries are frequent in transposition of the great arteries patients and may have clinical effects. The presence of major aortopulmonary collateral arteries should be investigated in patients who do not have a favourable post-operative course after arterial switch operation.

To study the impact of out-of-hours delivery on outcome for neonates with antenatally diagnosed transposition of the great arteries.

Tertiary paediatric cardiology centre (Yorkshire, United Kingdom), with co-located tertiary neonatal unit.

Neonates with antenatally diagnosed simple transposition of the great arteries delivered out-of-hours (Monday to Friday 17:00–08:00 and weekends) versus in-hours between 2015 and 2020.

The primary outcome was survival to hospital discharge. Secondary outcomes included neurological morbidity, length of stay, and time to balloon atrial septostomy.

Of 51 neonates, 38 (75%) were delivered out-of-hours. All neonates born in the tertiary centre survived to discharge. Time to balloon atrial septostomy was slightly longer for out-of-hours deliveries compared to in-hours (median 130 versus 93 mins, p = 0.33). Neurological morbidity occurred for nine (24%) patients in the out-of-hours group and one (8%) in-hours (OR 3.72, 95% CI: 0.42–32.71, p = 0.24). Length of stay was also similar (18.5 versus 17.3 days, p = 0.59). Antenatal diagnosis of a restrictive atrial septum was associated with a lower initial pH (7.03 versus 7.13; CI: 0.03–0.17, p = 0.01), longer length of stay (22.6 versus 17.3 days; CI: 0.37–10.17, p = 0.04), and increased neurological morbidity (44% versus 14%; OR 4.80, CI 1.00–23.15, p = 0.05). A further three neonates were delivered in surrounding hospitals, with a mortality of 67% (versus 0 in tertiary centre; OR 172, CI 5-5371, p = 0.003).

Neonates with antenatally diagnosed transposition of the great arteries have similar outcomes when delivered out-of-hours versus in-hours. Antenatal diagnosis of restrictive atrial septum is a significant predictor of worse outcomes. In our region, delivery outside the tertiary cardiac centre had a significantly higher risk of mortality.

In the absence of evidence-based guidelines, paediatric cardiologists monitor patients in the ambulatory care setting largely according to personal, patient, institutional, and/or financial dictates, all of which likely contribute to practice variability. Minimising practice variability may optimise quality of care while incurring lower costs. We sought to describe self-reported practice patterns and physician attitudes about factors influencing their testing strategies using vignettes describing common scenarios in the care of asymptomatic patients with tetralogy of Fallot and d-transposition of the great arteries.

We conducted a cross-sectional survey of paediatric cardiologists attending a Continuing Medical Educational conference and at our centre. The survey elicited physician characteristics, self-reported testing strategies, and reactions to factors that might influence their decision to order an echocardiogram.

Of 267 eligible paediatric cardiologists, 110 completed the survey. The majority reported performing an annual physical examination (66–82%), electrocardiogram (74–79%), and echocardiogram (56–76%) regardless of patient age or severity of disease. Other tests (i.e. Holter monitors, exercise stress tests or cardiac MRIs) were ordered less frequently and less consistently. We observed within physician consistency in frequency of test ordering. In vignettes of younger children with mild disease, higher frequency testers were younger than lower frequency testers.

These results suggest potential practice pattern variability, which needs to be further explored in real-life settings. If clinical outcomes for patients followed by low frequency testers match that of high frequency testers, then room to modify practice patterns and lower costs without compromising quality of care may exist.

Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure.

A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included.

Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation.

A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.

The aim of this retrospective cohort study was to determine the incidence, potential risk factors, characteristics, and outcomes of acute kidney injury in children following the arterial switch operation for transposition of the great arteries.

Retrospective review of children who underwent ASO between 2000 and 2020 in our tertiary children’s hospital in the Netherlands. Pre-and post-ASO serum creatinine levels were collected. Severe AKI was defined as 100% serum creatinine rise or estimated creatinine clearance <35 ml/min/1.73 m2 according to pRIFLE criteria. Logistic regression was used to adjust for confounders.

A total of 242 children were included. Fifty-seven (24%) children developed severe AKI after ASO. Four patients with severe AKI were treated with renal replacement therapy. Children with severe AKI had a longer duration of mechanical ventilation 4.5 (1.0–29) versus 3 (1.0–12) days (p = 0.001), longer PICU stay 7 (2–76) versus 5 (1–70) days, (p = 0.001), higher rate of myocardial infarction 5% versus 0.5% (p = 0.001), sepsis 24% versus 9% (p = 0.002), post-operative pulmonary hypertension 19% versus 6% (p = 0.002), post-operative bleeding 9% versus 3% (p = 0.044), longer time to sternal closure 3 (1–19) versus 2 (1–6) days, (p = 0.009), and a higher mortality rate 9.0% versus 0.5% (p = 0.001) compared to children without severe AKI. Sepsis was a risk factor for developing severe AKI.

In this single-centre cohort, 24% of our patients developed severe AKI after ASO, which is associated with increased morbidity, longer PICU stay, and higher mortality.

This study examined longitudinal associations between performance on the Rey–Osterrieth Complex Figure–Developmental Scoring System (ROCF-DSS) at 8 years of age and academic outcomes at 16 years of age in 133 children with dextro-transposition of the great arteries (d-TGA).

The ROCF-DSS was administered at the age of 8 and the Wechsler Individual Achievement Test, First and Second Edition (WIAT/WIAT-II) at the ages of 8 and 16, respectively. ROCF-DSS protocols were classified by Organization (Organized/Disorganized) and Style (Part-oriented/Holistic). Two-way univariate (ROCF-DSS Organization × Style) ANCOVAs were computed with 16-year academic outcomes as the dependent variables and socioeconomic status (SES) as the covariate.

The Organization × Style interaction was not statistically significant. However, ROCF-DSS Organization at 8 years was significantly associated with Reading, Math, Associative, and Assembled academic skills at 16 years, with better organization predicting better academic performance.

Performance on the ROCF-DSS, a complex visual-spatial problem-solving task, in children with d-TGA can forecast academic performance in both reading and mathematics nearly a decade later. These findings may have implications for identifying risk in children with other medical and neurodevelopmental disorders affecting brain development.

The association of atrioventricular septal defect and transposition of the great arteries is very rare. As a rule, these patients have unbalanced ventricles. However, there have been no studies describing the results of single-ventricle palliation in these children.

All children who underwent surgery with a diagnosis of atrioventricular septal defect and transposition of the great arteries were included in the study. Data were obtained from medical records.

A total of 38 patients with atrioventricular septal defect and transposition of the great arteries underwent single-ventricle palliation at the study institution between 1971 and 2016. The mean follow-up was 12.4 years (median: 14.6 years, range 2–43.3 years). Most children had unbalanced atrioventricular septal defect (94.7%, 36/38). Survival was 67.6% (95% confidence interval [CI]: 50.0–80.2%) at 10 years and 57.8% (95% CI: 38.0–73.4%) at 20 years. By 10 years, 58.6% (95% CI: 40.8–72.7%) had progressed to Fontan completion, while 32.5% (95% CI: 18.2–47.6%) had died. In patients achieving Fontan completion, 20-year event-free survival was 73.3% (95% CI: 34.8–91.3%), while 5.0% (95% CI: 0.4–20.5%) had undergone cardiac transplantation and 21.7% (95% CI: 3.2–50.8%) had undergone takedown of the Fontan circulation. Freedom from atrioventricular valve surgery was 57.0% (95% CI: 37.2–72.7%) at 10 and 20 years.

The association of atrioventricular septal defect and transposition of the great arteries is very rare, and most of these children have unbalanced ventricles. Single-ventricle palliation results in 25-year overall survival of 50%. However, in patients, who had Fontan completion, survival was 75% at 25 years after Fontan operation.