UNASSIGNED: Neoaortic root dilatation (NeoARD) and neoaortic regurgitation (NeoAR) are common sequelae following the arterial switch operation (ASO) for transposition of the great arteries.
UNASSIGNED: The authors aimed to estimate the cumulative incidence of NeoAR, assess whether larger neoaortic root dimensions were associated with NeoAR, and evaluate factors associated with the development of NeoAR during long-term follow-up.
UNASSIGNED: Electronic databases were systematically searched for articles that assessed NeoAR and NeoARD after ASO, published before November 2022. The primary outcome was NeoAR, classified based on severity categories (trace, mild, moderate, and severe). Cumulative incidence was estimated from Kaplan-Meier curves, neoaortic root dimensions using Z-scores, and risk factors were evaluated using random-effects meta-analysis.
UNASSIGNED: Thirty publications, comprising a total of 6,169 patients, were included in this review. Pooled estimated cumulative incidence of ≥mild NeoAR and ≥moderate NeoAR at 30-year follow-up were 67.5% and 21.4%, respectively. At last follow-up, neoaortic Z-scores were larger at the annulus (mean difference [MD]: 1.17, 95% CI: 0.52-1.82, P < 0.001; MD: 1.38, 95% CI: 0.46-2.30, P = 0.003) and root (MD: 1.83, 95% CI: 1.16-2.49, P < 0.001; MD: 1.84, 95% CI: 1.07-2.60, P < 0.001) in patients with ≥mild and ≥moderate NeoAR, respectively, compared to those without NeoAR. Risk factors for the development of any NeoAR included prior pulmonary artery banding, presence of a ventricular septal defect, aorto-pulmonary mismatch, a bicuspid pulmonary valve, and NeoAR at discharge.
UNASSIGNED: The risks of NeoARD and NeoAR increase over time following ASO surgery. Identified risk factors for NeoAR may alert the clinician that closer follow-up is needed. (Risk factors for neoaortic valve regurgitation after arterial switch operation: a meta-analysis; CRD42022373214).

OBJECTIVE: 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.
METHODS: 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).
RESULTS: 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.
CONCLUSIONS: 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.

In the transposition of the great arteries (TGA), alterations in hemodynamics and oxygen saturation could result in fibrotic remodeling, but histological studies are scarce. We aimed to investigate fibrosis and innervation state in the full spectrum of TGA and correlate findings to clinical literature. Twenty-two human postmortem TGA hearts, including TGA without surgical correction (n = 8), after Mustard/Senning (n = 6), and arterial switch operation (ASO, n = 8), were studied. In newborn uncorrected TGA specimens (1 day-1.5 months), significantly more interstitial fibrosis (8.6% ± 3.0) was observed compared to control hearts (5.4% ± 0.8, p = 0.016). After the Mustard/Senning procedure, the amount of interstitial fibrosis was significantly higher (19.8% ± 5.1, p = 0.002), remarkably more in the subpulmonary left ventricle (LV) than in the systemic right ventricle (RV). In TGA-ASO, an increased amount of fibrosis was found in one adult specimen. The amount of innervation was diminished from 3 days after ASO (0.034% ± 0.017) compared to uncorrected TGA (0.082% ± 0.026, p = 0.036). In conclusion, in these selected postmortem TGA specimens, diffuse interstitial fibrosis was already present in newborn hearts, suggesting that altered oxygen saturations may already impact myocardial structure in the fetal phase. TGA-Mustard/Senning specimens showed diffuse myocardial fibrosis in the systemic RV and, remarkably, in the LV. Post-ASO, decreased uptake of nerve staining was observed, implicating (partial) myocardial denervation after ASO.

Background: Pericardial effusion (PCE) is a significant complication after pediatric cardiac surgery. This study investigates PCE development after the arterial switch operation (ASO) and its short-term and longitudinal impacts. Methods: A retrospective review of the Pediatric Health Information System database. Patients with dextro-transposition of the great arteries who underwent ASO from January 1, 2004, to March 31, 2022, were identified. Patients with and without PCE were analyzed with descriptive, univariate, and multivariable regression statistics. Results: There were 4896 patients identified with 300 (6.1%) diagnosed with PCE. Thirty-five (11.7%) with PCE underwent pericardiocentesis. There were no differences in background demographics or concomitant procedures between those who developed PCE and those who did not. Patients who developed PCE more frequently had acute renal failure (N = 56 (18.7%) vs N = 603(13.1%), P = .006), pleural effusions (N = 46 (15.3%) vs N = 441 (9.6%), P = .001), mechanical circulatory support (N = 26 (8.7%) vs N = 199 (4.3%), P < .001), and had longer postoperative length of stay (15 [11-24.5] vs 13 [IQR: 9-20] days). After adjustment for additional factors, pleural effusions (OR = 1.7 [95% CI: 1.2-2.4]), and mechanical circulatory support (OR = 1.81 [95% CI: 1.15-2.85]) conferred higher odds of PCE. There were 2298 total readmissions, of which 46 (2%) had PCE, with no difference in median readmission rate for patients diagnosed with PCE at index hospitalization (median 0 [IQR: 0-1] vs 0 [IQR: 0-0], P = .208). Conclusions: PCE occurred after 6.1% of ASO and was associated with pleural effusions and mechanical circulatory support. PCE is associated with morbidity and prolonged length of stay; however, there was no association with in-hospital mortality or readmissions.

Background Information on maternal and fetal outcomes of pregnancy in women with D-transposition of the great arteries is limited. We conducted a systematic literature review on pregnancies in women with transposition of the great arteries after atrial and arterial switch operations to better define maternal and fetal risk. Methods and Results A systematic review was performed on studies between 2000 and 2021 that identified 676 pregnancies in 444 women with transposition of the great arteries. A total of 556 pregnancies in women with atrial switch operation were tolerated by most cases with low mortality (0.6%). Most common maternal complications, however, were arrhythmias (9%) and heart failure (8%) associated with serious morbidity in some patients. Worsening functional capacity, right ventricular function, and tricuspid regurgitation occurred in ≈20% of the cases. Rate of fetal and neonatal mortality was 1.4% and 0.8%, respectively, and rate of prematurity was 32%. A total of 120 pregnancies in women with arterial switch operation were associated with no maternal mortality, numerically lower rates of arrhythmias and heart failure (6% and 5%, respectively), significantly lower rate of prematurity (11%; P<0.001), and only 1 fetal loss. Conclusions Pregnancy is tolerated by most women with transposition of the great arteries and atrial switch operation with low mortality but important morbidity. Most common maternal complications were arrhythmias, heart failure, worsening of right ventricular function, and tricuspid regurgitation. There was also a high incidence of prematurity and increased rate of fetal loss and neonatal mortality. Outcome of pregnancy in women after arterial switch operations is more favorable, with reduced incidence of maternal complications and fetal outcomes similar to women without underlying cardiac disease.

Background: Dextro transposition of the great arteries (d-TGA) is the most common critical congenital cardiac defect surgically treated in the neonatal period by arterial switch operation (ASO). Major aortopulmonary collaterals (MAPCAs) can be present in this population and may complicate the early postoperative period. Our aim was to review our institutional data and systematically review the available literature to provide further insight on the clinical significance of MAPCAs during the early postoperative course after ASO. Methods: This is a retrospective study of patients with simple d-TGA who underwent ASO between March 1998 and September 2020 at Boston Children\'s Hospital. The MEDLINE, Embase, and Cochrane databases were searched from inception to June 2020. Results: Of the 671 d-TGA patients who underwent ASO at our center, 13 (1.9%) were diagnosed with MAPCAs. Five were diagnosed before ASO, while eight were diagnosed after ASO. Of these, two patients required catheterization for MAPCAs coiling during the same hospitalization on the 2nd and 11th postoperative days. The systematic review retrieved a total of 34 articles after duplicates were removed. Finally, nine studies reporting on 23 patients were deemed eligible for our analysis. The average time to MAPCAs coiling was 12 days, while the mean hospital stay was 36 days. Conclusions: MAPCAs should be included in the differential diagnosis of ASO complicated by cardiac or respiratory failure, or pulmonary hemorrhage acutely postoperatively. Once managed, recovery of these patients is predictable, and mortality is low. Further studies investigating the diagnostic value of echocardiography and the long-term outcomes of these MAPCAs are necessary.

Coronary artery status in adults long after the arterial switch operation (ASO) is unclear. We conducted a systematic review to provide an overview of coronary complications during adulthood and to evaluate the value of routine coronary imaging in adults after ASO, in light of current guidelines. Articles were screened for the inclusion of adult ASO patients and data on coronary complications and findings of coronary imaging were collected. A total of 993 adults were followed with a median available follow-up of only 2.0 years after reaching adulthood. Myocardial ischemia was suspected in 17/192 patients (8.9%). The number of coronary interventions was four (0.4%), and coronary death was reported in four (0.4%) patients. A lack of ischemia-related symptoms cannot be excluded because innervation studies indicated deficient cardiac innervation after ASO, although data is limited. Anatomical high-risk features found by routine coronary computed tomography (cCT) included stenosis (4%), acute angle (40%), kinking (24%) and inter-arterial course (11%). No coronary complications were reported during pregnancy (n = 45), although, remarkably, four (9%) patients developed heart failure. The 2020 European Society of Cardiology (ESC) guidelines state that routine screening for coronary pathologies is questionable. Based on current findings and in line with the 2018 American ACC/AHA guidelines a baseline assessment of the coronary arteries in all ASO adults seems justifiable. Thereafter, an individualized coronary follow-up strategy is advisable at least until significant duration of follow-up is available.

Dextro-transposition of the great arteries (D-TGA) is the most frequent cyanotic congenital heart pathology in neonates. Surgical correction of this condition is possible using the arterial switch operation (ASO) which was first performed by Jatene in 1975.
The aim of this study was to summarise the evidence on short- (less than 1 year), medium- (1-20 years), and long-term (more than 20 years) outcomes of children with D-TGA treated with the ASO. The primary outcome was survival. Secondary outcomes were freedom from cardiac reoperations, occurrence of aortic insufficiency, pulmonary stenosis, coronary artery anomalies, neuropsychological development problems and quality of life.
We searched MEDLINE, EMBASE, CINAHL, LILACS, and reference lists of included articles for studies reporting outcomes after ASO for D-TGA. Screening, data extraction and risk of bias assessment were done independently by two reviewers. We pooled data using a random-effects meta-analysis of proportions and, where not possible, outcomes were synthesized narratively. We used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system to assess the certainty of the evidence for each outcome.
Following ASO for TGA, short-term survival was 92.0% (95% CI 91.0-93.0%; I2 = 85.8%, 151 studies, 30,186 participants; moderate certainty evidence). Medium-term survival was 90.0% (95% CI 89.0-91.0%; I2 = 84.3%, 133 studies; 23,686 participants, moderate certainty evidence), while long-term survival was 87.0% (95% CI 80.0-92.0 %; I2 = 84.5%, 4 studies, 933 participants, very low certainty evidence). Evaluation of the different secondary outcomes also showed satisfactory results in the short, medium and long term. Subgroup analysis suggests slightly higher survival following ASO for TGA in the second surgical era (1998 to 2018) than in the first surgical era (1975 to 1997) in the short and medium term [93.0% (95% CI 92.0-94.0) vs 90.0% (95% CI 89.0-92.0) and 93.0% (95% CI 91.0-94.0) vs 88.0% (87.0-90.0%) respectively] but not in the long term [81.0% (95% CI 76.0-86.0%) vs 89.0% (80.0-95.0%)].
Pooled data from many sources suggests that the ASO for D-TGA leads to high rates of survival in the short, medium, and long term.

Atrioventricular (AV) discordance and ventriculoarterial (VA) concordance in the setting of visceral situs inversus are one of the rarest forms of cardiac malformations. To our knowledge, this is the first reported case of prenatal diagnosis of such rare cardiac anatomy in association with double-outlet right ventricle on fetal echocardiography. The physiology of this cardiac anomaly is similar to that of transposition of the great arteries, and the best surgical option is the atrial switch operation.

To examine the results of various myocardial revascularization techniques in pediatric patients to better understand the strategies for surgical treatment of coronary artery pathologies.
We analyzed 61 publications dedicated to the indications, methods, and results of coronary bypass surgery in children. Due to the small size of this cohort, case reports are also included in our review.
The main indications for coronary bypass grafting in children are Kawasaki disease, myocardial revascularization as a necessary procedure during the congenital cardiac surgery, to manage intraoperative iatrogenic damage to coronary arteries, and homozygous familial hypercholesterolemia. The use of internal thoracic arteries as conduits for coronary bypass grafting in children with Kawasaki disease showed significantly better results in long-term functionality compared to autovenous conduits (87% and 44%, respectively, P<0.001). Acute and late coronary events after arterial switch operation for the transposition of the great arteries, anomalous origin of the left coronary artery from the pulmonary artery, and left main coronary artery atresia are the main congenital heart diseases where surgical correction involves interventions on the coronary arteries.
The internal thoracic artery is a reliable and durable conduit that demonstrates proven growth potential in children.