Outcome after extracorporeal membrane oxygenation therapy in Norwood patients before the bidirectional Glenn operation.

OBJECTIVES: Patients after the Norwood procedure are prone to postoperative instability. Extracorporeal membrane oxygenation (ECMO) can help to overcome short-term organ failure. This retrospective single-centre study examines ECMO weaning, hospital discharge and long-term survival after ECMO therapy between Norwood and bidirectional Glenn palliation as well as risk factors for mortality. METHODS: In our institution, over 450 Norwood procedures have been performed. Since the introduction of ECMO therapy, 306 Norwood operations took place between 2007 and 2022, involving ECMO in 59 cases before bidirectional Glenn. In 48.3% of cases, ECMO was initiated intraoperatively post-Norwood. Patient outcomes were tracked and mortality risk factors were analysed using uni- and multivariable testing. RESULTS: ECMO therapy after Norwood (median duration: 5 days; range 0-17 days) saw 31.0% installed under CPR. Weaning was achieved in 46 children (78.0%), with 55.9% discharged home after a median of 45 (36-66) days. Late death occurred in 3 patients after 27, 234 and 1541 days. Currently, 30 children are in a median 4.8 year (3.4-7.7) follow-up. At the time of inquiry, 1 patient awaits bidirectional Glenn, 6 are at stage II palliation, Fontan was completed in 22 and 1 was lost to follow-up post-Norwood. Risk factor analysis revealed dialysis (P < 0.001), cerebral lesions (P = 0.026), longer ECMO duration (P = 0.002), cardiac indication and lower body weight (P = 0.038) as mortality-increasing factors. The 10-year mortality probability after ECMO therapy was 48.5% (95% CI 36.5-62.9%). CONCLUSIONS: ECMO therapy in critically ill patients after the Norwood operation may significantly improve survival of a patient cohort otherwise forfeited and give the opportunity for successful future-stage operations.

En Bloc Rotation of the Outflow Tracts - Double Root Translocation.

En bloc rotation of the outflow tracts or double root translocation offers an anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction and closely related forms of double outlet right ventricle. The technical principle is to excise aortic and pulmonary root en bloc, rotate them as a whole by 180°, and reimplant them. The left ventricular outflow tract is enlarged with the patch closing the ventricular septal defect. In our experience, two thirds of the pulmonary valves could be preserved. Growth of the aortic and pulmonary root could be demonstrated in several studies performed by our group. It is still a complex and technically demanding procedure with long cardiopulmonary bypass periods and cross-clamp times. However, perioperative mortality and complications do not differ significantly from other forms of reconstruction. The reoperation rate is significantly lower. Presently, the best time to perform this operation seems to be after the newborn period within the first year of life.

The right age for the en-bloc rotation of the outflow tracts: a single-centre experience.

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.

Long-term follow-up after simultaneous arterial switch operation and aortic arch repair.

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.

Surgical and interventional rescue strategies for Fontan failure.

OBJECTIVES: Fontan patients are at lifelong risk for developing complications, which may result in Fontan failure. Survival rates after heart transplantation (HTX) are still unsatisfying in these patients. Long-term survival of extracardiac Fontan patients in the modern era was investigated. The objective of this study was to investigate if surgical and interventional procedures in patients with protein-losing enteropathy (PLE) and/or plastic bronchitis (PB) and a failing Fontan circulation can postpone or avoid HTX. METHODS: Retrospective data collection of all children who underwent a Fontan procedure between January 1999 and July 2021 at our centre was performed. Patients were surveyed regarding the occurrence of PLE or PB and their outcome was reported descriptively. HTX-free survival of patients who underwent a rescue procedure due to PLE/PB was evaluated. RESULTS: Three hundred and seventy [94.1% (95% confidence interval, 91.4-96.3)] Fontan patients were free of HTX or death at last follow-up after a median follow-up time of 6.7 years. PB/PLE was diagnosed in 34 patients during the observation period. A rescue procedure was undertaken in 16 pts. at a median time of 6.5 months (range: 1 day to 9.4 years) since the initial diagnosis of PLE/PB. In these patients, HTX-free survival was 75% (95% confidence interval, 47.6-92.7) at a median follow-up time of 4.0 years after the procedure. Range: 3.5 months to 13.9 years. CONCLUSIONS: Extracardiac Fontan patients in the modern era expect reasonable HTX-free survival rates. Surgical and/or interventional rescue strategies for Fontan failure can postpone HTX for a sustained period of time.

Outcome of patients with tetralogy of Fallot with pulmonary atresia.

Objective: Tetralogy of Fallot patients with pulmonary atresia (TOFPA) have a largely varying source of pulmonary perfusion with often hypoplastic and even absent central pulmonary arteries. A retrospective single center study was undertaken to assess outcome of these patients regarding type of surgical procedures, long-term mortality, achievement of VSD closure and analysis of postoperative interventions. Methods: 76 consecutive patients with TOFPA operated between 01.01.2003 and 31.12.2019 are included in this single center study. Patients with ductus dependent pulmonary circulation underwent primary single stage full correction including VSD closure and right ventricular to pulmonary conduit implantation (RVPAC) or transanular patch reconstruction. Children with hypoplastic pulmonary arteries and MAPCAs without double supply were predominantly treated by unifocalization and RVPAC implantation. The follow up period ranges between 0 and 16,5 years. Results: 31 patients (41%) underwent single stage full correction at a median age of 12 days, 15 patients could be treated by a transanular patch. 30 days mortality rate in this group was 6%. In the remaining 45 patients the VSD could not be closed during their first surgery which was performed at a median age of 89 days. A VSD closure was achieved later in 64% of these patients after median 178 days. 30 days mortality rate after the first surgery was 13% in this group. The estimated 10-year-survival rate after the first surgery is 80,5% ± 4,7% showing no significant difference between the groups with and without MAPCAs (p > 0,999). Median intervention-free interval (surgery and transcatheter intervention) after VSD closure was 1,7 ± 0,5 years [95% CI: 0,7-2,8 years]. Conclusions: A VSD closure could be achieved in 79% of the total cohort. In patients without MAPCAs this was possible at a significant earlier age (p < 0,01). Although patients without MAPCAs predominantly underwent single stage full correction at newborn age, the overall mortality rate and the interval until reintervention after VSD closure did not show significant differences between the two groups with and without MAPCAs. The high rate of proven genetic abnormalities (40%) with non-cardiac malformations did also pay its tribute to impaired life expectancy.

Aortic arch reconstruction in the Norwood procedure using a curved polytetrafluorethylene patch.

OBJECTIVES: The aortic arch enlargement in the Norwood procedure is classically carried out using a curved homograft patch on the inner curvature of the neoaortic arch. The study investigates the outcome of a newly used artificial patch from a vascular prosthesis as an alternative to a homograft patch. METHODS: Since April 2007, we used curved polytetrafluorethylene (PTFE) patches cut out of a prosthesis as an alternative to homograft patches for the aortic arch reconstruction. The decision for either patch material was made due to anatomic reasons, preferring PTFE patches in larger aortas. In this study, 224 Norwood patients, operated between April 2007 and April 2018, were analysed. A total of 104 patients received a PTFE patch (group PTFE), and 120 patients got a pulmonary homograft patch (group homograft). A single-centre retrospective analysis was carried out concerning postoperative course and long-term follow-up regarding aortic arch interventions and reoperations and comparing the 2 material groups. RESULTS: There were no material associated operative or postoperative complications. In-hospital mortality was 13% in group PTFE. Six children died late during follow-up (6%). One aortic isthmus dilatation (1%) was carried out 12 months after the Norwood procedure in this group, no arch reoperation was necessary during the complete follow-up. CONCLUSIONS: The curved PTFE patch showed good qualities in operative technical demands and excellent long-term results. In selected cases of hypoplastic left heart syndrome, it can be well used as alternative to the pulmonary homograft.

Surgical therapy of infective endocarditis following interventional or surgical pulmonary valve replacement.

OBJECTIVES: Percutaneous pulmonary valve prostheses and right ventricle-to-pulmonary artery conduits are at risk for infective endocarditis (IE). In children and adults with a congenital heart disease, a pulmonary valve implant is frequently necessary. Prosthetic valve endocarditis is a conservatively barely manageable, serious life-threatening condition. We investigated the results of surgical pulmonary valve replacements in patients with IE. METHODS: A total of 20 patients with congenital heart disease with the definite diagnosis of IE between March 2013 and July 2020 were included in this single institutional, retrospective review. Infected conduits were 11 Melody, 5 Contegra, 3 homografts and 1 Matrix P Plus. All of the infected prosthetic material was removed from the right ventricular outflow tract up to the pulmonary bifurcation. Pulmonary homografts were implanted after pulmonary root resection as right ventricle-to-pulmonary artery conduits. RESULTS: All patients survived and were discharged infection-free. The mean time from the conduit implant to the operation for IE was 4.9 years [95% confidence interval (CI), 3.0-6.9]. The median intensive care unit stay was 3.0 days (95% CI, 2.0-4.7), and the median hospital time was 25.0 days (95% CI, 19.2-42.0). Median follow-up time was 204.5 days (range 30 days to 5 years) without death or recurrent endocarditis. CONCLUSIONS: The surgical treatment of IE of percutaneous pulmonary valve prostheses and right ventricle-to-pulmonary artery conduits is a safe and effective therapeutic concept. Early surgical referral of patients with suspicion of IE should be pursued to avoid sequelae such as right ventricular failure, septic emboli, intracardiac expansion and antibiotic resistance.

Valve sparing neoaortic root replacement following a Norwood type reconstruction of the aorta.

A neoaortic aneurysm after a Norwood type reconstruction of the aorta can develop due to systemic pressure on the former pulmonary artery wall. A complex valve sparing procedure can preserve native valves and avoid a valve replacement with requirement for anticoagulation. This type of operation was carried out in 3 patients, 2 of them after a Fontan palliation, 1 after a Norwood-Rastelli repair. The reconstruction was done using Dacron prostheses for the replacement of the dilated wall, similar to a Yacoub modification in 2 cases and to a David's modification in 1 patient. The postoperative course was uneventful and postoperative valve function was good in all cases.

En Block Rotation of the Outflow Tracts: Intermediate Follow-up After 15 Years of Experience.

BACKGROUND: The en bloc rotation of the outflow tracts is a surgical option for anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction with preserved growth potential in all tubular structures and the option for keeping the native pulmonary valve. The aim of this study was to analyze our 15-year experience with this operation. METHODS: A retrospective single-center study including 27 consecutive patients, who underwent the en bloc rotation between 2003 and 2019, was performed. Median age at operation was 103 days (interquartile range [IQR], 17-117 days), and median body weight was 5.2 kg (IQR, 3.66-8.4 kg). Six patients had undergone 1 to 4 previous operations. In 18 patients (66.7%), the pulmonary valve could be preserved, and in 9 patients (33.3%) a transannular patch was performed. Median follow-up was 5.7 years (IQR, 1.6-9.0 years). RESULTS: One patient died of chronic left ventricular failure during the hospital stay. Two more died after discharge: 1 of aspiration after a cerebral hemorrhage and 1 of sudden death. Five other patients required reoperations or had reinterventions: 1 ventricular septal defect closure, 1 aortic valve repair, 1 patch plasty, 2 dilatations of supravalvular pulmonary stenosis, and 1 stent into a left pulmonary artery. During the follow-up period, no gradient in the left ventricular outflow tract was found, and postoperative growth of the pulmonary valve was shown. CONCLUSIONS: The en bloc rotation allows an anatomic correction of the complex transposition with growth potential in all tubular structures. Reoperations or reinterventions on the pulmonary valve or on the left ventricular outflow tract have not been necessary to date.

Health-related quality of life for children and adolescents in school age with hypoplastic left heart syndrome: a single-centre study.

Data from neurological and radiological research show an abnormal neurological development in patients treated for hypoplastic left heart syndrome. Thus, the aim of this study was to survey the quality of life scores in comparison with healthy children and children with other heart diseases (mild, moderate, and severe heart defects, heart defects in total). Children with hypoplastic left heart syndrome (aged 6.3-16.9 years) under compulsory education requirements, who were treated at the Children's Heart Center Linz between 1997 and 2009 (n = 74), were surveyed. Totally, 41 children and 44 parents were examined prospectively by psychologists according to Pediatric Quality of Life Inventory, a health-related quality of life measurement. The results of the self-assessments of health-related quality of life on a scale of 1-100 showed a wide range, from a minimum of 5.00 (social functioning) to a maximum of 100 (physical health-related summary scores, emotional functioning, school functioning), with a total score of 98.44. The parents' assessments (proxy) were quite similar, showing a range from 10 (social functioning) up to 100. Adolescent hypoplastic left heart syndrome patients rated themselves on the same level as healthy youths and youths with different heart diseases. The results show that patients with hypoplastic left heart syndrome aged 6-16 years can be successfully supported and assisted in their psychosocial development even if they show low varying physical and psychosocial parameters. The finding that adolescent hypoplastic left heart syndrome patients estimated themselves similar to healthy individuals suggests that they learnt to cope with a severe heart defect.

External Prosthetic Reinforcement of the Pulmonary Autograft.

BACKGROUND: Neo-aortic root dilatation accounts for the majority of reoperations needed after the Ross procedure with implantation of the pulmonary autograft as complete root replacement. This study evaluates early results of external prosthetic reinforcement of the autograft. METHODS: From July 2015 to October 2017, 16 adolescent and adult patients received a Ross procedure at our department by this technique. A congenital-dysplastic valve was present in 13 patients, including 9 patients with a bicuspid aortic valve. Clinical and echocardiographic follow-up is complete with a mean duration of 19.7 ± 5.8 months. RESULTS: The mean age at operation was 27.1 ± 16.1 years. Mean aortic cross-clamping time was 102 ± 39 minutes. No bleeding complication occurred. The median stay on the intensive care unit was 2 days. In-hospital mortality was 0%. All patients were discharged with no or trivial aortic regurgitation. In one patient both the autograft and homograft were replaced because of endocarditis 3 months after the primary operation, leading to 93.8% freedom from reoperation at 2 years. There were no late deaths during the study period. The latest echocardiography confirmed absence of aortic regurgitation grade >I in all patients. Neo-aortic root diameters remained stable during follow-up. CONCLUSION: The presented modification of the Ross procedure does not prolong ischemia time, and can be performed with a low operative morbidity and mortality and an excellent early valve function.

Double-arterial cannulation: a strategy for whole body perfusion during aortic arch reconstruction.

OBJECTIVES: Double-arterial cannulation enables cerebral perfusion and lower body perfusion during aortic arch reconstruction. The aim of this study was to analyse and report our experience of using this cannulation and perfusion technique on paediatric patients. METHODS: A retrospective single-centre study was carried out on 407 consecutive paediatric patients who underwent an aortic arch reconstruction under double-arterial cannulation between 2003 and 2015. The median age of the patients at surgery was 8 (range 2-5570) days, and body weight was 3.3 (range 1.8-60) kg. All operations were performed through standard median sternotomy. One arterial cannula was inserted into the innominate artery and the second one into the supradiaphragmatic descending aorta. Primary end points were 30-day mortality, acute renal failure requiring dialysis and time until lactate level decreased to ≤2 mmol/l postoperatively. RESULTS: We found an in-hospital mortality of 8.6%. Lethal incident was not associated with the cannulation method, and 1 intraoperative lesion of the descending aorta could be repaired immediately. The median lactate level of the patients on arrival at the intensive care unit was 3.5 mmol/l [quartile (Q)1: 2.3-Q3: 4.7] and creatinine was 0.48 mg/100 ml (Q1: 0.40-Q3: 0.58). The longest duration until the lactate level decreased to ≤ 2 mmol/l was found in the group of 264 univentricular patients (median 11 h, Q1: 6-Q3: 24). Seven (1.7%) patients of the whole cohort required peritoneal dialysis postoperatively. CONCLUSIONS: Double-arterial cannulation is a simple and safe method for perfusing the brain and the lower parts of the body during aortic arch reconstruction. Perioperative survival and freedom from procedure-related complications in this demanding patient population are encouraging.

The Ross-Konno procedure in neonates and infants less than 3 months of age.

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.

Double-arterial cannulation during aortic arch reconstruction in pediatric patients.

Aortic arch reconstruction is a highly demanding procedure in congenital heart surgery. The 2 conventional options for cardiopulmonary bypass - deep hypothermic circulatory arrest or antegrade cerebral perfusion - both result in lack of perfusion of the entire or lower body.  In 2003, the Children's Heart Center Linz started to use a double-arterial cannulation technique for aortic arch reconstructions in order to provide whole body perfusion during the entire operation. Our technique, demonstrated in this video tutorial, is as follows: After inserting an arterial cannula into the innominate artery, followed by venous cannulation, the cardiopulmonary bypass is started. The left pleural cavity is opened directly above the diaphragm. The inferior pulmonary ligament is transected and the descending aorta is then visualized and can be cannulated directly. Both arterial cannulas are linked via a Y-connector. The surgeons in our center have used this method in more than 450 uni- and biventricular newborns and children. There has not been a lethal incident associated with this cannulation technique; one intraoperative lesion of the descending aorta was repaired immediately.  Our double-arterial cannulation technique is a simple and safe method for perfusing the brain and the lower parts of the body during aortic arch reconstruction.

Long-term Evaluation of the Ross Procedure in Acute Infective Endocarditis.

Optimal valve substitute for young patients with aortic valve endocarditis remains controversial. Given its better resistance to infection, the Ross procedure is an attractive alternative to prosthetic valve replacement or homograft implantation. The objective of this study was to assess long-term outcomes of the Ross procedure in this indication. From January 1991 to April 2017, 190 patients underwent a Ross procedure at our institution. Acute endocarditis was the indication for operation in 19 patients, including 6 patients with a bicuspid aortic valve. The pulmonary autograft was implanted as freestanding root replacement in all patients. The clinical follow-up is 100% complete, with a mean of 12.0 ± 5.7 years. The mean age of the study population was 35.9 ± 11.5 years. Moderate or severe aortic regurgitation was present in 84.2% of the patients. Systemic embolization had occurred in 36.8% of the patients. The mean aortic cross-clamp time was 126 ± 24 minutes. The median length of stay on the intensive care unit was 1 day. Mortality at 30 days was 5.3% (1 patient with gastrointestinal bleeding). Echocardiography at hospital discharge documented no or trivial aortic regurgitation in all patients. No case of recurrent endocarditis affecting the autograft occurred. One patient (0.4% per patient-year) was reoperated 1.8 years after the Ross procedure for homograft endocarditis. Three patients (15.8%) were reoperated for autograft aneurysm. The Ross procedure is a safe and effective alternative to prosthetic valve replacement or homograft implantation in selected young patients with acute endocarditis with a low rate of recurrent infection.

Cognitive Development of School-Age Hypoplastic Left Heart Syndrome Survivors: A Single Center Study.

Neurological and radiologic research results show an abnormal cerebral microstructure as well as abnormal neurodevelopment in patients treated for hypoplastic left heart syndrome. The aim of this study was to assess the varying cognitive performance these children have developed in dependence upon prenatal diagnosis, surgical techniques, surgical learning effects, anatomy, perfusion techniques, gender, pedagogic, and sociodemographic parameters in comparison to age-adjusted normative values. School-age children (6.3-16.9 years) with hypoplastic left heart syndrome, who were treated at the Children's heart Center Linz between 1997 and 2009, (n = 74), were surveyed in reference to cognitive achievements. 43 patients were examined prospectively by psychologists using the Wechsler intelligence scale for children IV in order to determine the respective total intelligence quotient index for each child's developmental stage. The mean index was 84.5 (percentile rank 26.4). The statistical spread and standard deviation ranged from a minimum of 40 to a maximum of 134 ± 20.8. The results for verbal comprehension, perceptual reasoning, and processing speed corresponded with total index results and were thus lower than the mean value of the normative values. The assessment of working memory showed results in the average. Prenatal diagnosis, type of lung perfusion, anatomy, and various cerebral perfusion techniques did not significantly affect the cognitive results of the patients. The results show that hypoplastic left heart syndrome patients can be successfully tutored formally as well as personally in cognitive areas, although when compared to healthy children, they showed lower results for intellectual area parameters.

Unplanned cardiac reoperations and interventions during long-term follow-up after the Norwood procedure†.

OBJECTIVES: Patients with hypoplastic left heart syndrome or related malformations are predominantly treated with a 3-stage palliation. Anatomic or physiologic problems can lead to unplanned additional surgical or catheter interventions during single ventricle palliation. Changes in operative technique may have an impact on the reoperation rate. METHODS: Between 1997 and 2014, 317 Norwood procedures were performed at our centre. A retrospective single centre investigation was carried out concerning incidence, timing, indication and type of unplanned interstage cardiac reoperations and catheter interventions during follow-up of Norwood patients. Patients were followed from birth until the end of 2015. Cardiac procedures taking place at the time of the bidirectional Glenn or Fontan procedure or heart transplantation were not included. RESULTS: Sixty-five of the Norwood patients (20.5%) had at least one additional surgical cardiac procedure. Nine patients (2.8%) needed open procedures prior to the Norwood operations, 11.0% had procedures in the interstage I, 3.5% in the interstage II and 9.1% of the Fontan patients had cardiac reoperations afterwards. Main indications for unplanned surgery were insufficient pulmonary perfusion and tricuspid regurgitation. Eighty-one patients (25.6%) had at least one interstage catheter intervention during follow-up mainly addressing stenosis of the pulmonary arteries, aortic arch stenosis or aortopulmonary collaterals. CONCLUSIONS: The number of unplanned reoperations and interventions during staged palliation is remarkably high showing surgical peaks in the interstage I and after the Fontan procedure and an interventional peak in the interstage II. Thorough early information of the parents about possibly anticipated additional procedures is necessary.

Effects of surgical en bloc rotation of the arterial trunk on the conduction system in children with transposition of the great arteries, ventricular septal defect and pulmonary stenosis.

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.