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OBJECTIVES Coronary transfer remains the most crucial part of the arterial switch operation (ASO); yet, certain coronary anatomies prohibit the use of button or trap-door transfer techniques. In the rare setting of ‘non-separable’ single sinus coronary arteries with intramural course, the modified Yacoub aortocoronary flap technique is a viable option. The aim of this study is to describe this operative technique and review its early- and mid-term outcomes. METHODS This retrospective analysis included all cases with ‘non-separable’ single sinus coronary arteries with intramural course where the modified Yacoub aortocoronary flap technique served as a bail-out option. RESULTS Of 516 patients who underwent ASO at our institution between January 1977 and April 2022, 14 underwent the modified Yacoub aortocoronary flap technique. The median age at ASO was 10 (interquartile range 7–19) days. Hospital mortality occurred in 3 patients (21.4%), all being related to coronary complications. All hospital survivors were still alive at a median of 9.1 (interquartile range 4.2–18.3) years after the ASO. None of them developed complaints of ischaemia, ventricular arrhythmias, ventricular dysfunction or exercise intolerance. Surveillance computed tomography angiography showed stable aortocoronary relationships free from stenosis, compression and kinking. No reoperations for coronary artery problems and/or neoaortic valve or root problems were needed. CONCLUSIONS Although close monitoring of early coronary events seems crucial to prevent perioperative mortality, the modified Yacoub aortocoronary flap technique may serve as a viable bail-out option in patients with ‘non-separable’ single sinus coronary anatomy with intramural course, with excellent results among hospital survivors.

Background Coronary anomalies are present in one-third of all patients with transposition of the great arteries (TGA) and have been associated with increased risk of adverse outcomes after the arterial switch operation. Therefore, knowledge about coronary anatomy remains key. Case summary A 5-day-old girl with prenatal diagnosis of Taussig–Bing anomaly (double outlet right ventricle with TGA and large subpulmonary ventricular septal defect) along with aortic arch hypoplasia and coarctation of the aorta underwent the arterial switch operation with closure of the ventricular septal defect and aortic arch repair. On preoperative echocardiography, the right (R) and left coronary artery (LCx) connected both to aortic sinus 1, suggesting 1RLCx coronary anatomy according to the Leiden Convention coronary coding system. However, intraoperative inspection led to a reclassification of the coronary anatomy: the right coronary artery and left anterior descending coronary artery connected to aortic sinus 1 (1RL) as had been observed on echocardiography, but—remarkably—the circumflex coronary artery (Cx) connected to the posterior sinus of the pulmonary trunk. As a consequence, cardioplegia was administered into both the aortic and pulmonary roots, and the circumflex coronary artery could stay in its native position without having to be transferred during the arterial switch operation. Discussion Various disruptions during embryological development can lead to unusual coronary anatomy in TGA patients. While anomalous connection of a coronary artery to the pulmonary trunk remains exceedingly rare, care should be taken to identify this pattern when present as failure to do so may result in adverse outcomes.

Background: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.Methods: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 2according to pRIFLE criteria. Logistic regression was used to adjust for confounders.Results: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.Conclusions: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.

ObjectivesDocumentation of semilunar valve growth in fetal transposition of the great arteries (TGA) and the relationship between neo-aortic root (NAoR) dilatation, a cause for postoperative reinterventions after the arterial switch operation (ASO), and pulmonary valve (PV) annulus dimensions prenatally.MethodsThis retrospective multicenter observational study included TGA fetuses suitable for ASO. Semilunar valve annuli pre-ASO and NAoR diameters (post-ASO) were measured. Trends in annulus diameters were analyzed using a linear mixed-effects model and compared with normal values. Prenatal semilunar valve Z-scores were correlated with NAoR diameters post-ASO.ResultsWe included 137 TGA fetuses (35.8% with significant ventricular septal defects [VSDs]). One hundred twenty-one underwent ASO. Fetal TGA-PV diameters were significantly larger than control aortic valve (AoV) and PV annuli from 23 and 27 weeks, respectively, especially when a VSD was present. Fetal TGA-AoV annuli were significantly larger than control AoV and PV annuli from 26 and 30 weeks, respectively.Z-scores of fetal TGA-PV and NAoR diameter at last follow-up correlated significantly (P ConclusionFetal TGA semilunar valve annuli are larger than control annuli, especially when there is a significant VSD. Factors besides postoperative hemodynamics, including fetal anatomy, PV Z-score, prenatal flow, connective tissue properties, and genetics, may influence the risk for late reintervention in these fetuses.What's already known about this topic? Transposition of the great arteries (TGA) is treated with the arterial switch operation (ASO). Neo-aortic root (NAoR) dilatation is seen postoperatively and may require late reintervention.What does this study add? The first serial data on semilunar valve size in fetal TGA in a large cohort are provided. TGA semilunar valve diameters are larger than in normal fetal hearts, giving a larger left ventricular outflow tract. The prenatal pulmonary valve size may predispose to NAoR dilatation post-ASO.

Item does not contain fulltext Mid-aortic syndrome is characterized by narrowing of the abdominal aorta, usually with the involvement of renal arteries and other visceral branches. The combination of the presence of an abdominal bruit, diminished or absent pulsations of the lower extremities, and a blood pressure discrepancy between upper and lower extremities is the classic triad associated with mid-aortic syndrome. However, it has a wide variety of clinical symptoms, and awareness of the variable presentation can lead to early diagnosis of the vascular anomaly. We report three cases presenting at three different stages of this disease, such as hydrops fetalis, refractory hypertension, and intracerebral bleeding. In conclusion, these cases highlight the importance of blood pressure measurements in all patients and accurate physical examination for early recognition of a mid-aortic syndrome. 01 maart 2013

After the arterial switch operation (ASO), disproportional neo-aortic growth during childhood has been reported. Even though it has been suggested neo-aortic dilation will stabilise in adulthood, data are lacking. The aim of this study was to assess the change in neo-aortic dimensions, prevalence of neo-aortic dilation >40 mm and long-term outcome in adults who underwent ASO in childhood. All 116 ASO patients operated in a tertiary referral centre and born before 1995 were included. Of these, 83 (72%) survived to adulthood (>17 years) and six were lost to follow-up. Neo-aortic measurements performed in adulthood were collected from available echocardiographic, cardiovascular magnetic resonance and CT images. The time trend was analysed using a mixed model, adjusted for imaging modality. Clinical data with at least one measurement of the neo-aortic diameter were available in 77 (93%) adult patients and serial measurements in 65 (78%). At baseline (median age 18.1 years), mean neo-aortic diameter was 36±5 mm. Mean neo-aortic growth was 0.31 mm/year (p

To the Editor: With great interest we read the article by Chen et al. regarding factors that affect heart-to-mediastinal ratio (H/M ratio) in I-123 meta-iodobenzylguanidine (MIBG) cardiac sympathetic imaging [1]. The authors describe the variation of H/M ratio among institutions and the factors that contribute to this. Furthermore, they provide different protocols that could minimize this variation. One of the significant contributors to the variation of H/M ratio in cardiac imaging, are the settings of the regions of interest (ROIs) that are used. Currently, as correctly described by Chen et al. [1, 2], there are three main approaches for drawing the heart ROI. One is to place the ROI over the whole heart, thereby neglecting the “dilution” effect in patients with a dilated heart (ie, lower blood pool activity in the cavity than the myocardium) but resulting in high reproducibility [1, 3]. The second approach is to place the ROI along the left ventricular (LV) wall, inter alia described by Gerson et al. [4], which possibly leads to greater inter- and intraobserver bias [1]. In the third approach a smaller ROI is placed along the anterior part of the LV wall [2]. In our current ongoing study in pediatric patients by van der Palen et al.., we compared both approaches described by Chen et al. (ie, the “whole heart ROI” and the “small anterior wall ROI”). The reason for comparing those instead of the “LV wall ROI” by Gerson et al. is twofold: possible comparison of our results with Chen’s references and potential inaccurate placement of an ROI along the complete LV wall in small pediatric hearts (ie, the child’s heart is often too small for exact placement over the LV wall). Several studies in pediatric cardiology also use this approach [5, 6]. When studying the subsequently calculated H/M ratios we noticed that the interobserver variability when using these methods of ROI placement was only minor, when H/M ratios were obtained independently by two observers at 4 and 24 h post injection in our population of 30 pediatric patients. Intraclass correlation coefficients (ICCs) were as follows: for the small anterior wall ROI H/M ratio 0.882 (95 % CI: 0.578–0.956) and for the whole heart ROI H/M ratio 0.955 (95 % CI: 0.904–0.979) at 4 h. At 24 h, the ICC was 0.911 (95 % CI: 0.716–0.965) for the small anterior wall ROI H/M ratio and 0.951 (95 % CI: 0.896–0.977) for the whole heart ROI H/M ratio. Therefore, it can be concluded that the use of both ROI definition methods in children results in substantial to almost perfect interobserver agreement concerning H/M ratio. Furthermore, this suggests that whole H/M ratio has better agreement than small H/M ratio for both 4 and 24 h post injection scans. Besides, both methods also showed a high within-patient ICC ratio of 0.865. This implies that intrapatient variability is fairly small and both methods give almost identical results. Although the ICCs of both methods of ROI placement are rather similar, the most promising method is the whole H/M ratio 4 h post injection. To reduce differences in H/M ratios due to interobserver variability the method of ROI placement should be standardized, and it is reasonable to define this method as the gold standard for H/M ratio measurement. However, as stated in the article by Chen et al. [1], this method could underestimate the H/M ratio in patients with a dilated heart (being the target population in the article by Chen et al.) and a relatively high negative contribution of the myocardial cavity. This, however, has no impact on our population, which does not contain patients with cardiomyopathy. Small anterior wall H/M ratios do not have this disadvantage and showed adequate intraobserver agreement. Therefore, we can conclude that the best method to determinate the H/M ratio in cardiac sympathetic imaging depends on the patient characteristics of the study group. In pediatric patients we recommend the use of the whole heart H/M ratio, since it shows the best intraobserver agreement. With Chen we would like to emphasize the importance to standardize ROI placement methods to compare study results and minimize the intrapatient, interpatient, and interinstitutional variation.

A 13-year-old boy presented with erythema nodosum on the anterior and lateral side of his lower legs due to enteritis caused by Salmonella enteritidis.