30 years' experience with the arterial switch operation: risk of pulmonary stenosis and its impact on post-operative prognosis.

Pulmonary stenosis is one of the most common complications in patients with transposition of the great arteries after the arterial switch operation. The reason for this is multifactorial and related to the anterior shift of the pulmonary trunk during the LeCompte manoeuvre, the complex suture line required to fill the gaps after harvesting the coronary arteries, and the need for patch implantation to maintain a tensionless anastomosis.We reviewed all patients with transposition of the great arteries operated on at our institute between 1991 and 2020 to establish the frequency of pulmonary stenosis during post-operative follow-up, reinterventions, and reoperations related to pulmonary stenosis and its potential risk factors.During the analysed period, we performed 848 arterial switch operations for simple and complex cases of transposition of the great arteries. The overall early mortality was 6.96%, and the late mortality was 2.53%. Among all study groups, 243 (28.66%) patients developed mild pulmonary stenosis, 43 patients (5.07%) developed moderate, and 45 patients (5.31%) developed severe pulmonary stenosis. During follow-up, 21 patients required interventions related to pulmonary stenosis. Pulmonary reconstruction with patches, aortic arch anomalies, and ventricular septal defects associated with transposition of the great arteries were significant risk factors. Nine patients required reoperation because of pulmonary artery stenosis with patch reconstruction of the pulmonary artery, aortic arch anomalies, and aortic cross-clamping time, increasing the risk of reoperation.Pulmonary stenosis in patients with transposition of the great arteries after the arterial switch operation is a common complication. If significant, it occurs early after surgery and is the most frequent reason for post-operative interventions and reoperations.

Predictors of Left Ventricular Outflow Tract Obstruction After Conventional Repair for Patients with Interrupted Aortic Arch or Coarctation of the Aorta, Combined with Ventricular Septal Defect: A Single-Center Experience.

Left ventricular outflow tract obstruction (LVOTO) affects survival and reoperation rates after surgical treatment of patients with interruption of the aortic arch (IAA) or coarctation of the aorta (CoA) with ventricular septal defect (VSD). The aim of the study was to determine predictors of LVOTO and to evaluate the relationship between aortic valve (AoV) morphology and the re-intervention rate. Retrospective review of patients, who underwent a conventional repair for IAA/CoA with VSD at a tertiary referral center between 1996 and 2017. The preoperative demographic data as well as pre- and post-operative echocardiographic parameters and re-interventions were reviewed. In the median follow-up of 8.3 years (range of 6.15-10.27) 5 patients (11.9%) from a total of 47 patients included in the study presented with a significant LVOTO. Four of them required reoperation after median period of 2.3 years (range of 0.3-7.9) after the initial surgery. Multivariable logistic regression identified AoV z-score (OR 0.44, p = 0.017) as predictor of LVOTO. The mean AoV z-score before the primary repair was significantly smaller in those with LVOTO as compared to those with unobstructed flow from the LV (- 3.58 ± 1.96 vs. - 1.44 ± 1.55; p = 0.0016). At 1-year follow-up, both groups showed an increase in the AoV z-score (p = 0.98). The re-intervention rate after primary repair (both surgical procedures and percutaneous interventions), either for LVOTO or reCoA, was higher in patients with AoV z-score ≤ - 3 (p = 0.007 vs. p = 0.46) and those, whose aortic annulus was less or equal than patient's weight (kg) + 1.5 mm as compared to those with larger aortic annulus (p = 0.03 vs. p = 0.16). In patients after surgical treatment of IAA/CoA with VSD, the AoV z-score at diagnosis is a significant risk factor for reoperation for LVOTO. With age, AoV growth and z-score improvement is expected. Small AoV at diagnosis is correlated with increased rate of re-intervention for LVOTO and reCoA.

Intramural coronary pattern in patients with transposition: incidence and impact on follow-up.

OBJECTIVES: Coronary complications are still the main reason for early mortality after an arterial switch operation. The high incidence of coronary anomalies in patients with transposition of the great arteries may increase the difficulty of coronary transfer, and among them, an intramural pattern was shown to be an independent risk factor of early mortality. However, recently published studies have reported that this rare coronary variant has no impact on the survival rate. The aim of this study was to assess the frequency of intramural coronary patterns in patients with transposition and the impact on overall mortality after an arterial switch operation. Additionally, we presented all coronary arrangements associated with intramural patterns in our cohort and the surgical techniques used to manage them successfully. METHODS: All arterial switch operations were retrospectively reviewed. In each case, the surgical reports contained detailed graphical representations and coronary anatomy patterns. All operatively confirmed intramural patterns were included in the analysis. RESULTS: Among 806 patients, 271 patients had coronary anomalies (33.62%), and 28 patients had an intramural pattern (3.47%), which was frequently associated with other complex coronary anomalies (P < 0.001). Overall survival was significantly higher in patients with intramural coronary artery patterns than in those with other coronary variants (21.34% vs 8.74%, P = 0.024, log-rank test). CONCLUSIONS: Intramural patterns associated with transposition remain a surgical challenge and increase overall mortality in our cohort. To reliably confirm or reject the significance of the observed impact of this rare coronary variant, a multicentre data analysis is required.

Neoaortic Regurgitation in Patients with Transposition Long Term After an Arterial Switch Operation and Its Relation to the Root Diameters and Surgical Technique Used.

Neoaortic regurgitation and root dilatation are common findings in patients with transposition after an arterial switch operation. The aim of this study was to describe the relation between neoaortic regurgitation long term after an arterial switch procedure, aortic root diameters, and surgical technique used. We also assessed the agreement of the neoaortic regurgitation grade and root diameters in different imaging modalities. For this retrospective study, we qualified 56 consecutive patients who, according to our institutional protocol, had a routine postoperative evaluation of more than 16 years with multimodality imaging studies. Neoaortic regurgitation was assessed by both transthoracic echocardiography and magnetic resonance imaging, and the root diameters obtained by echocardiography and tomography were compared to the reference values and associated with the presence of neoaortic insufficiency. Neoaortic insufficiency was present in 75% of examined patients; the vast majority of them had trace or mild regurgitation, and its qualitative evaluation was significantly different between echocardiography and magnetic resonance imaging. In our study group, the neoaortic valve and aortic sinus were larger in relation to the normal values, and they were significantly correlated with the presence of neoaortic insufficiency, but not with the surgical technique used. Values obtained by echocardiography and tomography correlated well but were significantly different. Transthoracic echocardiography has a tendency to overestimate the severity of regurgitation compared to magnetic resonance imaging. Neoaortic valve and sinus dilatation are significantly correlated with valve insufficiency, but in most cases of root dilatation, the valve remains competent.

Can we predict potentially dangerous coronary patterns in patients with transposition of the great arteries after an arterial switch operation?

INTRODUCTION: Coronary artery complications are the main reason for early mortality after an arterial switch operation. Late complications are relatively rare, and there is no consensus regarding the need or indications for routine follow-up coronary artery evaluations or the best first-line assessment modality. The aim of this study was to present the long-term post-operative frequency of coronary abnormalities in asymptomatic patients with transposition of the great arteries discovered by coronary CT angiography and potential "red flags" revealed by other examinations. PATIENTS AND METHODS: A group of 50 consecutive asymptomatic patients who underwent routine long-term coronary artery evaluation after an arterial switch operation according to our institutional protocol were qualified for this study. This routine in-hospital visit included a detailed medical interview, electrocardiography, echocardiography, Holter electrocardiography examinations, and laboratory and cardiopulmonary exercise tests. Patients who showed significant abnormalities were qualified for perfusion scintigraphy. RESULTS: Unfavourable coronary abnormalities were detected in 30 patients (60%) and included ostial stenosis, muscular bridge, coronary fistula, interarterial course, proximal kinking, high ellipticity index, proximal acute angulation (<30 degree) of the left coronary artery, and proximal acute angulation of the right coronary artery. These features could not be predicted based on the medical interviews, surgical reports, or non-invasive screening test results. CONCLUSION: Complex coronary configurations with potentially dangerous coronary features are common in patients with transposition after an arterial switch operation. Such high-risk patients cannot be identified indirectly, and coronary CT angiography provides accurate information that is useful for post-operative management.

Successful Late Recruitment of the Occluded Left Main Coronary Artery After Initial Arterial Switch Operation.

We describe a surgical technique for late recruitment of the proximally occluded left main coronary artery (LMCA) after initial arterial switch operation, which had been complicated by severe left ventricular dysfunction. This technique allowed for LMCA recanalization, using the patent conal artery branching off the LMCA close to the ostium. It is particularly useful in small children, where surgical options for coronary arteries are limited and associated with a high risk of restenosis. It was successfully used in a two-month-old boy with successful revascularization confirmed by angiography.

Deposition of mannose-binding lectin and ficolins and activation of the lectin pathway of complement on the surface of polyurethane tubing used for cardiopulmonary bypass.

The artificial surface used for cardiopulmonary bypass (CPB) is a crucial factor activating the complement system and thus contributing to the generation of a systemic inflammatory response. The activation of classical and alternative pathways on this artificial surface is well known. In contrast, lectin pathway (LP) activation has not been fully investigated, although noted during CPB in several studies. Moreover, we have recently proved the contribution of the LP to the generation of the systemic inflammatory response syndrome after pediatric cardiac surgery. The aim of this study was to assess LP-mediated complement activation on the surface of polyurethane CPB circuit tubing (noncoated Chalice ® ), used for CPB procedures in children with congenital heart disease. We found deposition of mannose-binding lectin, ficolin-1, -2, and -3 on the surface of unused tubing and on tubing used for CPB from a small minority of patients. Furthermore, we observed deposition of complement C4 activation products on tubing used for CPB and previously unused tubing after incubation with normal serum. The latter finding indicates LP activation in vitro on the polyurethane surface. © 2017 Wiley Periodicals, Inc. J Biomed Mater Res Part B: Appl Biomater, 106B: 1202-1208, 2018.

Usefulness of Routine Coronary CT Angiography in Patients with Transposition of the Great Arteries After an Arterial Switch Operation.

Coronary complications in patients with transposition of the great arteries (TGA) after an arterial switch operation (ASO) are relatively rare, but of all the possible postoperative adverse events, they are potentially the most dangerous. The fate of the coronary arteries, which are transplanted during the neonatal ASO, remains uncertain. There is also no consensus regarding their postoperative evaluation, especially in asymptomatic patients. The aim of this study was to present the early results of routinely performed coronary computed tomography angiography (CCTA) in asymptomatic adolescents and young adults with TGA after an ASO. An initial series of 50 CCTAs performed in asymptomatic patients with TGA after an ASO were evaluated. In each case, a detailed examination of the coronary anatomy, its relationship to the surrounding structures, its exact position in the neoaortic sinus, and the presence of significant coronary abnormalities was performed. The CT scans revealed significant coronary abnormalities in 12 asymptomatic patients: three had acute proximal angulation and stenosis, four had an intra-arterial course, seven had a muscular bridge, one had a left anterior descending artery with an intramuscular course, and one had coronary fistulas to the pulmonary arteries. Additionally, in 25 patients, proximal acute angulation of at least one coronary artery was detected, and four of them had a high ellipticity index. Most of the potentially severe anatomical features were related to the left coronary artery or the left anterior descending artery. CCTA routinely performed on asymptomatic patients with TGA after an ASO provides accurate and useful information for postoperative management. The frequency of coronary anomalies and potentially dangerous anatomical features in this group of patients is high, and their impact on postoperative follow-up remains unknown.

Coronary Artery Anomalies in Patients With Transposition of the Great Arteries and Their Impact on Postoperative Outcomes.

BACKGROUND: Coronary anomalies are frequently present in children with transposition of the great arteries (TGA). Such anomalies significantly increase the complexity of arterial switch operations and may have an effect on postoperative outcomes. In this study, we aimed to assess the frequency of coronary anomalies in children with TGA and describe their effect on postoperative outcomes. METHODS: All patients with TGA who underwent an arterial switch operation between 1991 and 2015 were consecutively enrolled in this study. Patient coronary patterns were obtained from corresponding operative reports and analyzed to determine their associations with adverse postoperative outcomes. RESULTS: The study included 715 patients with isolated and complex TGA. Coronary anomalies were present in 33.7% of patients and were significantly correlated with the side-by-side configuration of the great arteries. Coronary sinuses with more than 1 ostium were associated with a significantly increased risk of postoperative death (hazard ratio [HR], 2.58), and coronary arteries with a single ostium were associated with an increased risk of postoperative reinterventions (HR, 2.49). In contrast, the trap-door technique of coronary artery transplantation was associated with a reduced risk of reinterventions during postoperative follow-up (HR, 0.40). Complex coronary anomalies were significantly associated with postoperative coronary events (HR, 2.12). CONCLUSIONS: With the exception of patients whose circumflex artery branches off of the right coronary artery, an anomaly that clearly has no effect on postoperative follow-up, patients with unusual coronary patterns are at higher risk for adverse postoperative outcomes than patients with normal coronary anatomy and must be monitored carefully.

Reoperations and catheter interventions in patients with transposition of the great arteries after the arterial switch operation.

OBJECTIVES: Reoperations and catheter interventions after the arterial switch operation (ASO) are relatively rare, but their frequency varies among different centres. They significantly impact the postoperative course of children with transposition of the great arteries (TGA). The aim of this study was to assess the frequency of reoperations and catheter interventions in patients with TGA after the ASO and to identify the potential risk factors. METHODS: For this retrospective case review study we included all consecutive 715 patients with TGA who underwent the ASO in the Department of Cardiac Surgery between the years 1991 and 2015. All of the surgical procedures were performed by one cardiac surgery team led by J.J.M., using the same surgical technique with his own specific modifications. RESULTS: The overall early mortality after the ASO was 7.4%; late mortality occurred in 15 cases (2.3%) and the mean clinical follow-up of our cohort was 10.5 years. Early reoperations (<30 days after surgery) were performed in 37 patients (5.1%). Reoperations were performed in 31 patients (4.7% of survivors), and their risk factors were previous early reoperation and left ventricle outflow tract obstruction, while isolated TGA reduced the risk of reoperations. Catheter interventions were performed in 25 patients (3.8% of survivors). In the majority of the cases, the indications for percutaneous procedures were pulmonary stenosis and recoarctation of the aorta. The statistically significant risk factors were aortic arch anomalies associated with TGA and neopulmonary artery anastomosis with a patch, while isolated TGA decreased the risk of reintervention. Freedom from cumulative reinterventions after the ASO was 90.4% at 5 years; 88.0% at 10 years; 86.5% at 15 years and 86.5% from 20 to 25 years. CONCLUSION: The frequency of reoperations and percutaneous interventions in patients with TGA after the ASO remains low. The majority of the procedures are performed because of pulmonary stenosis and recoarctation of aorta. Cardiac anomalies associated with TGA have a significant impact on the incidence of reoperation and reintervention.

Mannose-binding lectin (MBL) insufficiency protects against the development of systemic inflammatory response after pediatric cardiac surgery.

We investigated MBL2 and MASP2 genotypes, serum MBL (mannose-binding lectin) levels and activities of its complexes with associated serine proteases (MASP-1, MASP -2), in relation to complications following cardiac surgery in 195 children. The incidence of SIRS was lower in patients carrying MBL2 A/O and O/O genotypes (p=0.024). Children with MBL levels <500ng/ml had a lower risk of SIRS (p=0.014) and fever (p=0.044). Median MBL concentration was higher in patients who developed SIRS (p=0.048) but lower in those with post-operative infections (p=0.046). MBL-MASP-2 activities <100mU/ml protected from SIRS (p=0.007), low cardiac output syndrome (p=0.03) and multiorgan failure (p=0.012). In contrast, MBL2 YA/YA genotypes were associated with SIRS (p=0.018), low cardiac output syndrome (p=0.018), fever (p=0.018) and high inotropic score (VIS>30) (p=0.021). Thus, low MBL concentrations and associated genotypes may protect patients from systemic inflammation while high MBL serum levels and corresponding genotypes are risk factors of postoperative complications.

Primary Ficolin-3 deficiency--Is it associated with increased susceptibility to infections?

Ficolin-3 (also called H-ficolin or Hakata antigen) is the most potent activator of the lectin pathway of complement in vitro. Its genetically determined deficiency in Caucasians is associated with a frame-shift mutation +1637delC (rs28357092) of the FCN3 gene. When it was described for the first time, it was postulated to be strictly associated with enhanced susceptibility to infections. At present, with our knowledge extended by several other patients that issue seems to be more complicated and less clear-cut. Two new cases of primary Ficolin-3 deficiency are reported here: a 50-year old male, suffering from membranous nephropathy and an 11-month old male infant who was operated on to repair congenital heart disease. Based on those cases and a literature review, we conclude that the clinical consequences of congenital Ficolin-3 deficiency are still unclear and such questions as whether it may be life-threatening or acts as a disease modifier remain to be elucidated.

Are the surgeon's movements repeatable? An analysis of the feasibility and expediency of implementing support procedures guiding the surgical tools and increasing motion accuracy during the performance of stereotypical movements by the surgeon.

The developments in surgical robotics suggest that it will be possible to entrust surgical robots with a wider range of tasks. So far, it has not been possible to automate the surgery procedures related to soft tissue. Thus, the objective of the conducted studies was to confirm the hypothesis that the surgery telemanipulator can be equipped with certain routines supporting the surgeon in leading the surgical tools and increasing motion accuracy during stereotypical movements. As the first step in facilitating the surgery, an algorithm will be developed which will concurrently provide automation and allow the surgeon to maintain full control over the slave robot. The algorithm will assist the surgeon in performing typical movement sequences. This kind of support must, however, be preceded by determining the reference points for accurately defining the position of the stitched tissue. It is in relation to these points that the tool's trajectory will be created, along which the master manipulator will guide the surgeon's hand. The paper presents the first stage, concerning the selection of movements for which the support algorithm will be used. The work also contains an analysis of surgical movement repeatability. The suturing movement was investigated in detail by experimental research in order to determine motion repeatability and verify the position of the stitched tissue. Tool trajectory was determined by a motion capture stereovision system. The study has demonstrated that the suturing movement could be considered as repeatable; however, the trajectories performed by different surgeons exhibit some individual characteristics.

The neoaortic root in children with transposition of the great arteries after an arterial switch operation.

OBJECTIVES: Neoaortic root changes in children with transposition of the great arteries (TGA) are reportedly risk factors for the development of neoaortic regurgitation (NeoAR). The aims of this study were to assess the neoaortic root diameter and relative proportion in children with TGA after surgical correction and to identify possible correlations with the development of neoaortic insufficiency. METHODS: Of the 611 children who had the arterial switch operation performed in the Cardiology Department of the Polish Mother's Memorial Hospital, 172 consecutive patients were qualified for this study. The inclusion criteria were: anatomical correction performed during the neonatal period, more than 10 years of postoperative observation and at least two full echocardiographic examinations. RESULTS: NeoAR increased during postoperative follow-up and at the end of the observation period, 76% of the patients had NeoAR (27%-trace, 42%-mild, 7%-moderate and 0.6%-severe). Among the analysed risk factors for NeoAR development, the significant ones were arterial valve discrepancy (OR = 2.05; 95% CI: 1.04-4.02; P = 0.031) and the non-facing commissures (OR = 4.05; 95% CI: 1.34-11.9; P = 0.01). The neoaortic root diameter was not statistically significantly correlated with the presence of NeoAR or with the heart defects associated with transposition. The neoaortic root was initially, on average, 37% (z-score = 1.58) bigger than the aortic root in healthy children. This disproportion increased during the follow-up evaluations to 57% (z-score = 2.09). CONCLUSIONS: The neoaortic root in children after the arterial switch procedure develops differently from that in healthy children, but this is not evidently related to NeoAR development or associated heart defects.

Long-term outcome of direct neopulmonary artery reconstruction during the arterial switch procedure.

BACKGROUND: Neopulmonary stenosis at anastomosis site is one of the most frequent complications after the arterial switch procedure for transposition of the great arteries. The surgical technique is a crucial factor associated with the frequency of stenotic complications. We present the outcomes of direct neopulmonary anastomosis during the arterial switch procedure in patients with simple transposition. This research was to assess the efficacy of this surgical technique based on the incidence of postprocedural supravalvular neopulmonary stenosis (SVPS). METHODS: Among 545 patients operated on in our department between 1992 and 2009, the 346 consecutive survivors who had undergone simple transposition in the first month of life were included in this analysis. Switch procedures were performed with direct neopulmonary artery anastomosis in 318 patients (92%); in the remaining 28 (8%), the risk of coronary artery compression required the use of a pericardial patch for pulmonary reconstruction. RESULTS: Neopulmonary stenosis occurred in 9 patients (2.6%): 5 had undergone direct neopulmonary reconstruction, and 4 had been treated with a patch. Balloon angioplasty of SVPS was performed twice in 1 patient. No patients required reoperation to treat neopulmonary stenosis. In multivariate analysis (logistic regression), patch reconstruction (odds ratio, 27.5; p=0.001) and nonfacing commissures (odds ratio, 11.1; p=0.004) were correlated significantly with the incidence of SVPS. CONCLUSIONS: Direct neopulmonary artery anastomosis during arterial switch is an interesting alternative to patch reconstructions and ensures a good postoperative result with low rates of complications and SVPS.

Neoaortic valve function 10 to 18 years after arterial switch operation.

Anatomical correction is a procedure of choice for transposition of the great arteries (TGA) in neonates. During surgery, the aorta and pulmonary artery are switched-the native pulmonary valve becomes the neoaortic valve. The fate of this valve remains uncertain. Many reports suggest that its ability to function worsens with time after surgery. Of 519 patients with TGA operated on between 1991 and 2008, 161 met inclusion criteria for this retrospective study and were followed 10 years or more to assess neoaortic valve regurgitation (NeoAR) occurrence and development and to estimate potential risk factors. The subjects were divided into 2 groups: group 1 (simple TGA) and group 2 (TGA + ventricle septal defect). Within the analyzed group, the frequency of significant regurgitation increased from 9% 1 year after the operation to 47% at the most recent follow-up. No severe regurgitation necessitating reoperation was observed. Analysis of potential risk factors revealed that pulmonary/aortic valve diameter discrepancy and nonfacing commissures were associated with increased risk of development of neoaortic insufficiency. NeoAR arises and develops over time after correction of the defect. No hemodynamic repercussions necessitating cardiac surgical interventions were observed. The majority of insufficiencies are detected between 2 and 6 years after surgery. The degree of incompetence is usually mild and increases during follow-up by about 0.5 or 1 degree. The risk factors for NeoAR appearance are pulmonary artery/aortic annulus discrepancy and nonfacing commissures.

Left atrial approach to close the atrial septal defect type II in case of right lung agenesis with extreme dextroversion.

This paper presents a case of a rare combination of right lung agenesis and atrial septal defect type II (ASD II). Successful surgical repair of ASD II was performed at the age of six years with total circulatory arrest (TCA) and deep hypothermia, because of difficult approach. Computed tomography and intraoperative findings are described. From patho-embryological consideration, defects in the primitive respiratory apparatus in embryo stages were presumably responsible for agenesis of the lung. The patient was found to have a very short stump of the right pulmonary artery, but this malformation seems to be secondary.

Effects of cardiopulmonary bypass on the expression of adhesive molecules in children undergoing cardiac surgery due to congenital heart disorders.

BACKGROUND: Infants and small children who undergo cardiac surgery due to congenital heart disorders, are at risk of developing inflammatory complications and multi-organ failure. AIM: To assess the changes in the expression of adhesive molecules on neutrophils and in peripheral blood plasma in children undergoing cardiac surgery with or without the use of cardiopulmonary bypass (CPB). METHODS: In 18 children who underwent surgery with the use of CPB and in 7 children who underwent surgery without CPB, the expression of CD11b, CD11c and CD62L molecules on neutrophils and soluble adhesive molecules - L-selectin and ICAM-1, was assessed before, during and after cardiac surgery. RESULTS: A significant increase in the expression of CD11b on neutrophils at the time of the initiation of CPB was observed. The most pronounced decrease in the expression of CD11c was detected at the end of surgical procedure. There was also a negative correlation between L-selectin concentration measured at the end of CPB and CPB duration as well as between ICAM-1 concentration and duration of hypothermia. CONCLUSIONS: Cardiac surgery influences the expression of adhesive molecules on neutrophils and in blood plasma. These changes are more pronounced in children who undergo CPB, and depend on CPB duration as well as the use of hypothermia.