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.

Lung perfusion scintigraphy in the assessment of pulmonary circulation after completion of surgical treatment of a hypoplastic left heart syndrome (HLHS).

INTRODUCTION: Hypoplastic left heart syndrome (HLHS) is an inborn complex heart malformation. A multi-stage treatment is initiated in a neonatal period with a Norwood surgery. The next step is Glenn surgery - a bidirectional superior cavo-pulmonary anastomosis. At the last stage anastomosis of inferior vena cava (IVC) with the right pulmonary artery (RPA) is formed as a result of a Fontan surgery. The aim of this study was to assess lung perfusion in patients with HLHS after completion of a surgical therapy, using a scintigraphic method. MATERIAL AND METHODS: In 92 patients with HLHS a planar lung scintigraphy in anterior and posterior projections after administration of 99mTc-macroaggregates in activity 18-111MBq was carried out twice (in several day intervals). At first, a radiopharmaceutical was administered to the right extremity in order to assess the lung distribution of blood flowing through the anastomosis of superior vena cava (SVC) with RPA. In the next study, after administration of the tracer to the right lower extremity, the distribution of blood flowing through the anastomosis of IVC with RPA was assessed. The relative percentage of each lung in the total lung perfusion was calculated on a Xeleris workstation using the "Lung perfusion analysis" program. Lung perfusion was considered close to symmetrical when the proportion was in the range of 40-60%. RESULTS: In spite of the fact that mean relative values of distribution of blood flowing through the anastomosis of SVC with RPA to the left lung (LL) and right lung (RL) in the entire study group did not differ significantly: LLmean = 47%; RLmean = 53%, p = 0.14, relative values of perfusion of both lungs were differentiated - in 26% of patients LL was better perfused, in 38% RL was better perfused and in 36% a perfusion of both lungs was similar. The analysis of blood distribution by anastomosis of IVC with RPA showed that the mean relative perfusion of RL was significantly higher than that of LL (70% vs. 30%, p < 0.0000001). No signs of pulmonary emboli were detected. In 13% of studied patients, uptake of the radiopharmaceutical in kidneys was shown indicating the presence of shunt "from right to left". CONCLUSIONS: After completion of surgical treatment of patients with HLHS, differentiated blood supply of the lungs was observed through SVC with RPA anastomosis and a tendency to higher blood supply of RL than LL by anastomosis of IVC with RPA. No signs of pulmonary embolism were detected. The study revealed a "right to left" shunt in some patients.

La fusión de imágenes tridimensionales por TAC y RM antes del cateterismo facilita el implante de stent en defectos cardiacos congénitos / Three-dimensional image fusion of precatheter CT and MRI facilitates stent implantation in congenital heart defects

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Stenting of the inter-atrial septum in infants and small children: Indications, techniques and outcomes.

OBJECTIVES: To evaluate the procedural success and outcome of inter-atrial stenting. BACKGROUND: Inter-atrial stenting has been shown to be an effective way to maintain inter-atrial blood flow, however it is considered a high risk procedure, usually performed urgently in patients with significant hemodynamic compromise. METHODS: Between September 2004 and August 2016, inter-atrial stenting was attempted in 29 children. Procedural, clinical, and follow-up data were collected retrospectively. RESULTS: The procedures were completed successfully in 27 patients. Twenty-five procedures were undertaken percutaneously, with the remaining four being performed as hybrid procedures. The patients were considered as high risk for adverse events (82% scored as CRISP 4 and 5) with four deaths during the first 24 hr (14%). Procedural complications occurred in eight patients (28%) with related death in three patients (10%). One further patient died after an uncomplicated technically successful stent implantation performed as a salvage procedure. Procedural complications (71% vs. 14%) and mortality (43% vs. 5%) were higher in those, who weighed 3 kg or less (P < 0.05). Patency of the stents was maintained until planned staged surgery in 22 patients at a mean of 302 days. Three patients underwent further balloon dilation for flow restriction at 58-201 days. In two un-operated patients the stents remained patent at follow-up. One patient with severe pulmonary hypertension died with a patent stent. CONCLUSIONS: Inter-atrial stenting produces reliable patency with a very good success rate. Morbidity and mortality were related to low weight at the time of the procedure.

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.

Novel Three-Dimensional Image Fusion Software to Facilitate Guidance of Complex Cardiac Catheterization : 3D image fusion for interventions in CHD.

We report initial experience with novel three-dimensional (3D) image fusion software for guidance of transcatheter interventions in congenital heart disease. Developments in fusion imaging have facilitated the integration of 3D roadmaps from computed tomography or magnetic resonance imaging datasets. The latest software allows live fusion of two-dimensional (2D) fluoroscopy with pre-registered 3D roadmaps. We reviewed all cardiac catheterizations guided with this software (Philips VesselNavigator). Pre-catheterization imaging and catheterization data were collected focusing on fusion of 3D roadmap, intervention guidance, contrast and radiation exposure. From 09/2015 until 06/2016, VesselNavigator was applied in 34 patients for guidance (n = 28) or planning (n = 6) of cardiac catheterization. In all 28 patients successful 2D-3D registration was performed. Bony structures combined with the cardiovascular silhouette were used for fusion in 26 patients (93%), calcifications in 9 (32%), previously implanted devices in 8 (29%) and low-volume contrast injection in 7 patients (25%). Accurate initial 3D roadmap alignment was achieved in 25 patients (89%). Six patients (22%) required realignment during the procedure due to distortion of the anatomy after introduction of stiff equipment. Overall, VesselNavigator was applied successfully in 27 patients (96%) without any complications related to 3D image overlay. VesselNavigator was useful in guidance of nearly all of cardiac catheterizations. The combination of anatomical markers and low-volume contrast injections allowed reliable 2D-3D registration in the vast majority of patients.

Fenestration closure with Amplatzer Duct Occluder II in patients after total cavo-pulmonary connection.

INTRODUCTION: Creation of a fenestration during completion of a total cavopulmonary connection (TCPC) has been associated with a reduction in early mortality and morbidity. However, the long-term benefits are negated by an associated limitation in exercise tolerance and the potential risks of thrombo-embolic complications. We sought to describe the safety and efficacy of an Amplatzer Duct Occluder II (ADO II) for transcatheter fenestration closure following TCPC. MATERIAL AND METHODS: Between January 2000 and July 2014, 102 patients underwent percutaneous closure of extra-cardiac TCPC fenestrations with a range of devices. Patients in whom fenestration closure was performed with an ADO II and who had at least 6 months of follow-up were included in this study. RESULTS: Forty-seven patients had successful fenestration occlusion with an ADO II. The mean oxygen saturation and mean systemic venous pressures increased from 84.8 ±6.1% before to 97.6 ±2.9% (p < 0.001) after and from 14.2 ±2.15 mm Hg before to 15.6 ±2.2 mm Hg after closure (p < 0.001). Eight patients developed heart failure symptoms, managed by optimization of medical therapy, with 1 patient requiring device removal to reopen the fenestration. Color Doppler transthoracic echocardiography demonstrated residual flow across the device in 18 (38%), 10 (22%), 5 (11%) and 4 (9%) patients before discharge, at 1 and 6 months, and at the latest outpatient visit, respectively. CONCLUSIONS: The ADO II can be safely and effectively used to close fenestrations in extra-cardiac type Fontan completions. Many of the design features of this device confer potential benefit in this population.

Immediate and long-term outcomes of percutaneous transcatheter pulmonary valve implantation.

BACKGROUND: Transcutaneous pulmonary valve replacement (TPVR) has become an alternative to heart surgery for patients after previous right ventricular outflow tract (RVOT) or pulmonary artery (PA) surgical interventions. The objective was to present immediate and long-term outcomes of trans¬cutaneous pulmonary valve replacement. METHODS: Between 06/2009 and 06/2016, 46 patients underwent TPVR. Initial diagnoses included tetralogy of Fallot, common arterial trunk, transposition of great arteries post Rastelli correction, left ventricle outflow obstruction after Ross operation, pulmonary atresia, and isolated dysplastic pulmonary valve stenosis. Thirty eight (78%) patients had previously implanted conduits in the pulmonary position, the rest had either RVOT patch reconstruction (n = 6; 13%) or biological valve implantation (n = 2; 4%). They presented primarily with pulmonary stenosis (n = 18; 39%) or regurgitation (n = 28; 60%). RESULTS: All procedures were successful - 44 Melody and 2 Edwards-Sapien valves were implanted. Before each procedure exclusion of potential coronary compression and RVOT prestenting was performed. Significant RVOT systolic gradient reduction (from 35.3 ± 19.5 to 13.5 ± 7.1 mm Hg; p < 0.001) and decrease of right to left ventricle systolic pressure ratio from 0.58 ± 0.18 to mean 0.37 ± 0.1 (p < 0.001) was achieved. Also, in every patient PA-RVOT competence was restored, with minor in¬competence in only a few patients. Post procedure follow-up ranged from 2 to 86 (mean 35.2) months. Follow-up fluoroscopy or chest X-ray revealed 6 stent fractures (2 stent defragmentation - with only 1 significant valve stenosis). CONCLUSIONS: Transcutaneous pulmonary valve replacement is a safe procedure with encouraging results, it also enables deferring surgical reintervention in the majority of patients.

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.

Initial experience with live three-dimensional image overlay for ductal stenting in hypoplastic left heart syndrome.

AIMS: Precise visualisation of ductal morphology and adjacent vessels is crucial for accurate stent choice and placement during hybrid palliation of hypoplastic left heart syndrome (HLHS). We aimed to describe our initial experience with live three-dimensional reconstruction overlay derived from rotational angiography (RA) for ductal stenting in HLHS. METHODS AND RESULTS: We carried out a retrospective review of ductal stenting in 18 newborns with HLHS, including six patients with 3D reconstruction overlay used to guide the intervention. The median age at the intervention was 20 days (range 13-31 days) and the median weight was 3.25 kg (range 3-4 kg). Eleven RA runs were performed, pre and post stent implantation in five patients and before the intervention in one patient. 3D reconstructions from all RA runs had sufficient image quality to allow stent placement without additional contrast injections. Comparison with 2D angiography-guided ductal stenting showed similar contrast usage, with the 2D angiography patients receiving a higher radiation dose. CONCLUSIONS: Three-dimensional rotational angiography provides accurate visualisation of the ductal morphology and nearby structures. Three-dimensional reconstruction overlay with clear landing points enabled precise stent implantation with no additional contrast injections and lower radiation doses than conventional angiography in our patients.

Severe Congenital Obstruction of the Left Main Coronary Artery Coexisting With Supravalvular Aortic Stenosis in Williams Syndrome: A Dangerous Association.

Congenital obstruction of the left main coronary artery is a complicating feature of supravalvular aortic stenosis. We describe an eight-month-old female patient with Williams syndrome, supravalvular aortic stenosis, and branch pulmonary artery stenosis, with concomitant anomaly of severe obstruction of the left coronary artery orifice.

Intra-uterine growth restriction as a risk factor for hypertension in children six to 10 years old.

INTRODUCTION: Intra-uterine growth restriction (IUGR) is present in about 3-10% of live-born newborns and it is as high as 20-30% in developing countries. Since the 1990s, it has been known that abnormalities during foetal growth may result in cardiovascular disease, including hypertension in adulthood. METHODS: This study evaluated blood pressure parameters (using ambulatory blood pressure monitoring) in children aged six to 10 years old, born as small for gestational age (SGA), and compared them to their healthy peers born as appropriate for gestational age (AGA). RESULTS: In the SGA group, an abnormal blood pressure level (prehypertension or hypertension) was present significantly more often than in the AGA group (50 vs 16%, p < 0.01). This relationship also occurred in association with the type of IUGR (asymmetric p < 0.01, symmetric p < 0.05). CONCLUSIONS: In SGA children, abnormal blood pressure values occurred more frequently than in AGA children.