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Objectives: The en-bloc rotation of the outflow tracts (EBR) enables an anatomic correction of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction. The anatomical condition or previous palliative procedures may allow choosing an elective date for the anatomic correction. The aim of this study was to evaluate the optimal age for performing the EBR based on the largest series published so far., Methods: At the Children's Heart Center Linz, the EBR was performed in 33 patients between 2003 and 2021. Median age at operation was 74 [interquartile range (IQR) 17-627] days. Twelve patients were newborns (≤28 days), 9 older than 369 days. These 2 groups were compared to the remaining patients each regarding peri- and postoperative data, complications, reinterventions and mortality. The median follow-up period was 5.4 (IQR 0.99-11.74) years., Results: In-hospital mortality was 6.1%. All-cause mortality was lower in patients younger than 369 days at the time of EBR (4.2% vs 44.4% in patients >369 days old, P = 0.013). In newborns, intensive care unit stay (median 18.5 days vs 8 days, P = 0.008) and in-hospital stay (median 29.5 days vs 15 days, P = 0.026) were significantly longer, the risk for postoperative AV block was higher (33.3 vs 0%, P = 0.012) than in patients who had been corrected anatomically after the neonatal period., Conclusions: The results of this study suggest the deferral of the EBR to the post-newborn period. A significantly higher mortality rate in patients of older age at operation seems to recommend the anatomic correction during the first year of life., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: Patients with severe left ventricular outflow tract obstruction often suffer from impaired left ventricular function, endocardial fibroelastosis and borderline-sized structures. The early Ross-Konno operation can offer complete repair due to outflow tract stenosis and enable the functional recovery and growth of small structures., Methods: Between 2008 and March 2017, 44 early Ross-Konno procedures were performed at our centre. Thirty-five patients were neonates, and 9 were infants less than 3 months of age. A retrospective single-centre investigation was carried out analysing early and late deaths, postoperative complications and reoperations during the follow-up period. Potential prognostic influence factors as previous fetal intervention, associated lesions as presence of a VSD or hypoplastic aortic arch or severe endocardial fibroelastosis were examined., Results: The in-hospital mortality rate was 7% (3 of 44), and the late mortality rate was 2%. There were no deaths in the group with ventricular septal defects (9 cases), and there was 1 death in the group with critical aortic stenosis without arch repair (1 of 24; 4%). The most deaths occurred in patients with critical aortic stenosis and aortic arch repair (3 of 11; 27%) (P = 0.012). Fifteen patients with foetal intervention had a mid-term survival rate of 87%. Reoperations were necessary in 19 of 40 surviving patients during a mean follow-up period of 5.9 years., Conclusions: The early Ross-Konno procedure can enable biventricular repair even in borderline left ventricles with good long-term outcome. Thus far, there were no reoperations at the level of the autograft or the left ventricular outflow tract in our cohort. The highest operative risk was observed in patients with critical aortic stenosis and aortic arch hypoplasia.

Objectives: The standard surgical management of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis is the Rastelli operation. Recently, en bloc rotation of the arterial trunk, by cutting out the aortic and the pulmonary root in one block and by rotating it 180°, has been introduced as a new option for anatomical repair., Methods: To evaluate the effects of this surgical method on the conduction system, pre-operative, post-operative, and follow-up electrocardiograms as well as patient charts were reviewed retrospectively. A total of 16 consecutive patients with transposition of the great arteries and left outflow tract obstruction were treated with en bloc rotation., Results: During the post-operative period, there were two patients with complete atrio-ventricular block, one with junctional ectopic tachycardia, one with ventricular tachycardia, and one with supraventricular tachycardia. None of the patients had a typical right bundle branch block pattern before surgery; however, this pattern was detectable after surgery in eight out of 16 patients (50%), which persisted during the follow-up. All patients without typical right bundle branch block pattern showed a median QRS duration of 65 ms (54-112 ms) before surgery, 62 ms (54-122 ms) after surgery, and 84 ms (66-128 ms) at the last follow-up visit. This compares well with a similar Rastelli cohort, where a right bundle branch block prevalence of 77% was reported. Out of 16 patients, 12 showed non-specific ST changes and negative T-waves, which persisted during follow-up with an unknown significance for the future., Conclusion: Our data suggest that en bloc rotation of the arterial trunk seems not to have more negative effects on the conduction system than the Rastelli operation.

Objectives: Transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction are commonly called complex transposition. The traditional method of repair is the Rastelli procedure. Aortic translocation (Nikaidoh 1984) provides a more anatomic repair of this malformation. En bloc rotation of the truncus arteriosus (double root translocation, half turn truncal switch procedure) was introduced in 2003 (Yamagishi), and offers a complete anatomic repair with growth potential in all tubular structures. The aim of this study was to analyse our general experience with this method and the mid-term results concerning growth of the tubular structures as well as the function of the semilunar valves, if preserved., Methods: Nineteen patients with transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction or similar cases of double outlet right ventricle (DORV) have been treated by an en bloc rotation of the truncus arteriosus in our centre since 2003. Patient age ranged between 4 days and 6.46 years. The median age was 0.39 [0.1; 2.25] years. Weight ranged between 3.1 and 18.8 kg. Median weight was 5.6 [3.6; 9] kg. Five patients had received between 1 and 4 palliative procedures prior to the definitive repair. The pulmonary valve could be preserved in 15 cases, whereas a transannular patch was necessary in 4 cases., Results: One patient died of chronic left ventricular failure during the hospital stay. One patient acquired a severe cerebral haemorrhage 3 weeks after the operation. She was discharged and died 6 months later. One patient is not in a follow-up programme. Sixteen patients are now followed over a period of 153 days to 9.96 years. Aortic and pulmonary valves showed proportional growth during the follow-up period. The preserved pulmonary valves were small for age, but kept their competence satisfactorily. Three patients required a reoperation: one aortic valve repair, 1 permanent pacemaker, VSD closure., Conclusions: Up to now, reoperations had been caused by technical issues. The reoperation rate can be kept low, by understanding some important features of this procedure and avoiding these problems. Complete anatomic repair with growth potential and satisfactory preserved pulmonary valve function is possible., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Open in new tab Download slide OBJECTIVES Taussig–Bing anomaly (TBA) and transposition of the great arteries (TGA) with hypoplastic or interrupted aortic arch (AA) are rare anomalies. Various operative techniques and a high incidence of reinterventions are described. The aim of this retrospective single-centre study was to evaluate operative data, mortality and reintervention rate with special regard to the AA. METHODS At the Children's Heart Center Linz, 50 patients with the above-mentioned diagnosis have been corrected by a simultaneous repair between 2001 and 2022. Thirty-seven children had TBA, 13 had TGA and 5 of them had an interrupted AA. The median age at operation was 7 [interquartile range (IQR) 5–9] days, weight 3.38 (IQR 2.9–3.8) kg and follow-up 9.3 (IQR 3.1–14.5) years. The AA reconstruction was performed without patch material in 49 cases. RESULTS There was 1 in-hospital mortality in a TBA patient and 1 late mortality (7 years later, neuroblastoma). 14/49 patients needed at least 1 reoperation (28.6%, all TBA) and 3 further patients had catheter reintervention or radiofrequency ablation only (6.1%, 2 TBA). Seventy-five percent of these procedures affected the right heart/pulmonary arteries; there was 1 re-coarctation repair. CONCLUSIONS The simultaneous correction of TBA and TGA with AA obstruction or interruption is a safe operation with very low mortality. The AA reconstruction with minimized use of patch material resulted in a low restenosis rate. [ABSTRACT FROM AUTHOR]