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.

Atrial septum stenting in a foetus with hypoplastic left heart syndrome and restrictive foramen ovale: an alternative to emergency atrioseptectomy in the newborn-a case report.

BACKGROUND: In patients with hypoplastic left heart syndrome (HLHS) premature closure or restriction of the interatrial communication causes severe cyanosis directly after birth with rapid deterioration in clinical state. An ex-utero intrapartum treatment (EXIT) procedure, extracorporal membrane oxygenation (ECMO), and emergency interventional cardiac catheterization or cardiac surgery has to be anticipated and prepared. We report the first case performing foetal atrial septum stenting in such a patient directly before birth to enable uncomplicated interatrial shunting postnatally. CASE SUMMARY: A 31-year-old pregnant woman was referred to our centre for further evaluation of the foetus due to HLHS. In the follow-up study before birth severe restriction of the foramen ovale with increased retrograde flow in the pulmonary veins [0.33 ratio antegrade/retrograde time velocity integral (TVI)] was detected. After careful consideration foetal atrial septum stenting was performed at 38 + 3 weeks of gestation. At 39 + 2 weeks of gestation the baby was born by caesarean section followed by an uncomplicated postnatal adaption. On the 7th day of life a Norwood procedure was performed and the baby was discharged on the 63rd postoperative day. DISCUSSION: Evaluation of the interatrial communication in foetuses with HLHS should be done carefully just before birth. In the case of severe restriction or closure of the foramen ovale atrial septum stenting just before birth can be considered as an alternative treatment to an EXIT procedure, ECMO, or emergency atrioseptectomy on bypass. However, a very experienced team of paediatric cardiologists and perinatologists with expertise in foetal cardiac interventions is needed to perform this technical difficult procedure.

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.

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.

Aminoterminal pro-B-type natriuretic peptide: heart or lung disease in the neonate?.

OBJECTIVES: B-type natriuretic peptides have been shown to enable differentiation between heart and lung diseases in adults and children. In neonates, the role of natriuretic peptides for diagnosis of congenital heart defect (CHD) is not yet ascertained. The purpose of this single-center prospective study was to investigate aminoterminal B-type natriuretic peptide concentrations and their time courses during the first 5 days of life in neonates with CHD compared with neonates with respiratory distress. DESIGN: Single-center prospective study. SETTING: Tertiary-care neonatal ICU. PATIENTS: Aminoterminal B-type natriuretic peptide levels of 40 neonates with arterial duct-dependent CHD and of 40 neonates with respiratory distress without CHD were analyzed on the first, second, third, and fifth day of life. MAIN RESULTS: Mean aminoterminal B-type natriuretic peptide concentrations in the CHD group were significantly higher on the second (14191 vs. 4872 pg/mL), third (17790 vs. 3524 pg/mL), and fifth day (17015 vs. 4044 pg/mL), but not on the first day of life. Repeated measurements analysis of variance revealed a significantly different time course of aminoterminal B-type natriuretic peptide concentrations between the two groups. CONCLUSIONS: On the first day of life, aminoterminal B-type natriuretic peptide cannot differentiate between CHD and respiratory distress without CHD in the neonate. From the second day onwards, aminoterminal B-type natriuretic peptide in neonates with CHD shows higher values and a different time course and enables differentiation between CHD and respiratory distress due to other than cardiac reasons.