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Open in new tab Download slide 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. [ABSTRACT FROM AUTHOR]

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]

Open in new tab Download slide Open in new tab Download slide 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. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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 andhomograftwere 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 operativemorbidity andmortality and an excellent early valve function. [ABSTRACT FROM AUTHOR]

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 neo- nates, and 9 were infants less than 3months 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 interven- tion, 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 ven- tricular 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 interven- tion 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

OBJECTIVES The Norwood procedure is commonly used as first-step palliation in children with hypoplastic left heart syndrome or related congenital malformations. In most cases, the operation is carried out during the first week of life. Excessive pulmonary blood flow in univentricular physiology can soon lead to compromised cardiac function and pulmonary hypertension and could have an influence on postoperative outcomes. METHODS Between 1997 and 2011, 257 Norwood procedures were performed at the Kinderherz-Zentrum Linz. Twenty-seven patients were older than 20 days at the time of the Norwood operation without previous pulmonary artery banding. A retrospective analysis of the two age groups was carried out concerning early mortality, interstage mortality and major events until the bidirectional Glenn operation. RESULTS Patients older than 20 days had a significantly higher mortality rate before the bidirectional Glenn operation than younger patients (44.4 vs 20.3%; P = 0.002). They also showed a significantly higher need for postoperative inhalative nitric oxide therapy (40.7 vs 14.5%, P = 0.002). Summarized risk for either successful postoperative cardiopulmonary resuscitation (CPR), extracorporeal membrane oxygenation therapy or mortality before the bidirectional Glenn operation was 59.3% in the older age group vs 23.8% in the younger age group (P < 0.001). CONCLUSIONS Age older than 20 days seems to be an independent risk factor for early postoperative mortality and fatal events after the Norwood procedure. Long-persisting excessive pulmonary blood flow and preoperative cardiac decompensation can be the reason for postoperative lethal pulmonary hypertensive crisis and compromised ventricular function. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract: Objective: Because of their poor clinical status, infants may require surgery for congenital heart disease regardless of weight or prematurity. This retrospective review describes a single-centre experience with open-heart surgery in low-weight infants. Methods: From November 1997 to December 2006, 411 open-heart surgery procedures were performed in neonates. This included 46 consecutive infants weighing less than 2500g, who underwent cardiopulmonary bypass for correction of congenital heart defects (n =34) or Norwood stage I palliation of hypoplastic left heart syndrome (HLHS) (n =12). In the low-weight group were 23 males and 23 females with a median age of 10 days and a median weight of 2.26kg (range: 1.28–2.49kg). Results: Early mortality was 8.2% in patients weighing more than 2.5kg and 13% in the low-weight group. Within the low-weight group, weight at surgery, history of prematurity and prevalence of additional extracardiac malformations did not influence early mortality. At a median follow-up time of 32 months overall mortality was 21%. Thirty-four patients had a neurological follow-up examination 30 months postoperatively. Of the 34 survivors, 11 showed neurological deficits. Conclusions: In our patient population, early mortality was higher for infants weighing less than 2.5kg. However, within the low-weight group, lower weight at surgery or history of prematurity was not associated with a higher mortality or bad neurological outcome. [Copyright &y& Elsevier]

Unlabelled: Successful therapy of vasodilatory shock in adults and children with arginine-vasopressin (AVP) has been reported previously. Data on the use of vasopressin in neonates is limited. This retrospective study reports the effects of AVP-treatment in neonates with catecholamine-resistant systemic vasodilatation after cardiopulmonary bypass. From March 2003 through December 2005, 172 neonates underwent open-heart surgery, 17 developed vasopressor-resistant hypotension and were treated with AVP. Thirteen patients had a stage I palliation of single ventricle, two had a Ross-operation and two had an arterial switch operation. All patients received multiple traditional inotropes and vasopressors prior to administration of AVP. AVP was started at median 0.0001 U x kg(-1) x min(-1) (range 0.00005-0.0002) and titrated up to a maximum of median 0.0003 U x kg(-1) x min(-1) (range 0.0001-0.001). AVP led to a significant increase in blood pressure (from 49+/-8 mmHg to 69+/-7 mmHg) and the requirement of traditional vasopressors decreased significantly. No peripheral vasoconstriction or ischemia was observed. Four of 13 patients, all with single ventricle palliation, died. In two patients death occurred due to additional complications 6 days after AVP was discontinued. One patient, who was still on AVP, died 42 hours postoperatively after prolonged hypoxemia not responding to inhaled nitric oxide. One patient arrested on the third postoperative day when AVP was almost weaned.Conclusion: In neonates with vasodilatory shock after cardiopulmonary bypass AVP is a potent agent to increase blood pressure when traditional vasopressors are failing. [ABSTRACT FROM AUTHOR]