Evaluation of mid-term outcomes of partial and intermediate atrioventricular canal defect surgery.

BACKGROUND: The risk factors for reoperation and mortality after partial and intermediate atrioventricular canal defect repair are unclear. This study assessed the mid-term outcomes and risk factors for reoperation and mortality after partial and intermediate atrioventricular canal defect surgery. METHODS: Ninety-seven patients who underwent primary repair of intermediate (n = 45) or partial (n = 52) atrioventricular canal defect between 2005 and 2019 were included in this single-centre study. RESULTS: The median age was 5 years (2.7-8.9 years). The median follow-up time was 32 months (1.6-90.8 months). The estimated freedom from reoperation at 1, 5, and 10 years was 97%, 91%, and 73%, respectively.In multivariable analyses, post-operative left atrioventricular valve regurgitation of grade II or higher (odds ratio [OR]: 5.3, 95% confidence interval [CI]: 1.8-15.5, p = 0.01) and post-operative residual intracardiac shunt (OR: 11.6, 95% CI: 1.6-85.8, p = 0.02) were risk factors for reoperation.In multivariable analyses, perioperative reoperation (OR: 93.4, 95% CI: 3.9-218.7, p = 0.01) and the need for right atrioventricular valve repair (OR: 11.2, 95% CI: 1.0 - 123.3, p = 0.04) were risk factors for mortality. Mortality was higher in patients under 2.6 years of age. CONCLUSION: For patients undergoing repair of partial or intermediate atrioventricular canal defect, those with post-operative left atrioventricular valve regurgitation of grade II or higher and post-operative residual intracardiac shunt have an increased reoperation risk. Higher mortality can be expected after a perioperative reoperation, and in patients requiring right atrioventricular valve repair during the index procedure.

Surgical repair of the anomalous aortic origin of the coronary arteries: a single-center experience.

OBJECTIVE: Anomalous aortic origin of the coronary artery is a rare congenital cardiac anomaly. The aim of this study was to present our experience with patients who underwent surgery for the anomalous aortic origin of the coronary artery. METHODS: This was a retrospective review of our experience with patients who had anomalous aortic origin of the coronary artery from 2019 to 2022. RESULTS: Seven patients were managed for anomalous aortic origin of the coronary artery, including five males and two females. The median age of the patients were 16 years (IQR, 14.5-26 years). Five patients had anomalous aortic origin of the right coronary artery and two patients had anomalous aortic origin of the left coronary artery. Five patients were treated surgically, one patient refused surgical treatment despite myocardial ischaemia symptoms, and the other one was not operated because she had no symptoms. Two patients underwent pulmonary root anterior translocation and left main coronary artery unroofing procedure, one patient underwent right coronary artery unroofing procedure, one patient underwent pulmonary artery lateral translocation procedure, and the last patient underwent right coronary artery osteal translocation procedure. The post-operative mortality or myocardial infarction was not observed in any patient. Patients were followed for a median of 10 months (IQR, 6.75-20.5 months) after repair. CONCLUSION: The data suggest that surgical repair of anomalous aortic origin of the coronary artery can be performed confidently and can be very effective for relieving myocardial ischaemia symptoms. Different surgical techniques can be used in anomalous aortic origin of the coronary artery according to the course and origin of the coronary arteries. To the our knowledge, pulmonary root anterior translocation and coronary artery unroofing procedure were performed for the first time in the literature.

Evaluation of midterm outcomes after Shone's complex surgery: Analysis of reoperation and mortality risk factors.

OBJECTIVE: The midterm results of patients who underwent biventricular repair surgery for Shone's complex were examined, and mortality and reoperation risk factors were evaluated. METHODS: This retrospective study included 34 patients with Shone's complex who underwent mitral valve (MV) surgery between 2005 and 2020. RESULTS: A total of 19 patients (56%) had coarctation, 10 (29%) patients had subaortic stenosis, 9 (26.5%) patients had a hypoplastic aortic arch (AA), and 9 (26.5%) patients had aortic valve (AV) stenosis. Twenty-four (70.6%) patients had bileaflet AV. Associated left-sided in-flow stenotic lesions included parachute MV in 19 (56%) patients and supramitral ring in 18 (53%) patients. The estimated freedom from reoperation rate on the 6th month, 1 year and 2 years after surgery was 84.4%, 79.5%, and 71.5%, respectively. The overall mortality rate was 20.6% (seven patients) with a median follow-up of 10 months (0-41). The estimated survival rate on the 6th month, 1 year, and 3 years after surgery was 83.8%, 79.4%, and 79.4 respectively. Bicuspid aortic valve (p = .017) (HR (95% CI) = 0.130 (0.025-0.695) and hammock mitral valve (p = .038) (HR (95% CI) = 11,008 (1,146->100) were associated with mortality. CONCLUSION: The presence of a bicuspid aortic valve hammock mitral valve might have an effect on negative effect on the outcome.

Unifocalization with pericardial roll technique in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

OBJECTIVE: This study aims to compare both the pericardial roll technique with the patch augmentation technique of the unifocalization, and single-stage complete repair with the unifocalization and shunt for the repair of the ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. METHODS: This was a retrospective review of the 48 patients undergoing unifocalization of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries from a single center. Our cohort had two surgical pathways, including single-stage midline unifocalization (n = 40), unifocalization after pulmonary artery rehabilitation by creating an aortopulmonary window or central shunt (n = 8). There were two surgical techniques in single-stage midline unifocalizaton, including widening of the pulmonary arteries with a patch (n = 30), and connecting pulmonary arteries with a pericardial roll (n = 10). RESULTS: A total of 14 (29.2%) of 48 patients underwent single-stage complete repair, 26 patients underwent shunt palliation with unifocalization. Combined early and late mortality was seen in seven patients in those who underwent shunt palliation with unifocalization, while it was seen in one patient in those who underwent a single-stage complete repair (mortality ratio 26.8% vs. 7.1%, p = .22). There was no statistically significant difference between the pericardial roll and patch augmentation techniques in terms of pulmonary artery reintervention (p = .65). Although all pulmonary artery reinterventions were for unilateral pulmonary artery in the roll technique group, 41.7% of reinterventions were for bilateral pulmonary arteries in the pericardial augmentation group. CONCLUSION: Single-stage complete repair of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries has better results than unifocalization with a shunt. In terms of nonvaluable raw material, the use of the pericardial roll technique is a considerable alternative for unifocalization.

COVID-19 and ECMO support after neonatal congenital heart surgery: a case report.

Coronavirus disease 2019 causes respiratory and systemic disease and has led to a sudden epidemic affecting people of all ages. Patients with congenital heart disease represent a high-risk population. In this article, we present a newborn who required extracorporeal membrane oxygenation support for acute respiratory failure in the early postoperative period due to exposure to severe acute respiratory syndrome coronavirus 2 after aortic arch repair and ventricular septal defect closure. To the best of our knowledge, this patient represents the first neonatal case of severe acute respiratory syndrome coronavirus 2 infection after congenital heart surgery and is the youngest patient to need extracorporeal membrane oxygenation support.

The choice of palliative arterial switch operation as an alternative for selected cases in a single center: Experience and mid term results.

INTRODUCTION AND OBJECTIVE: There are various management options for newborns with single ventricle physiology, ventriculoarterial discordance and subaortic stenosis, classically involving the early pulmonary banding and aortic arch repair, the restricted bulboventriculer foramen enlargement or the Norwood and the Damus-Kaye-Stansel procedures. The aim of this study is to evaluate our preferred technique and comment on the midterm results of our clinical experience with palliative arterial switch operation (pASO) for a certain subset of patients. METHOD: We hereby retrospectively evaluate the charts of patients who went through pASO, as initial palliation through Fontan pathway, starting from 2014 till today. RESULTS: Ten patients underwent an initial palliative arterial switch procedure. Eight of 10 patients survived the operation and discharged. Seven of 10 patients completed Stage II and 1 patient reached the Fontan completion stage and the other six of ten (6/10) patients are doing well and waiting for the next stage of palliation. There are two mortalities in the series (2/10) and one patient lost to follow-up (1/10). CONCLUSION: In our opinion, the pASO can be considered as an alternative palliation option for patients with single ventricle physiology, transposition of the great arteries and systemic outflow tract obstruction despite longer cross clamp times compared to other methods, but It not only preserves systolic and diastolic ventricular function, but also provides a superior anatomic arrangement for following stages.

Anomalous left coronary artery from the pulmonary artery repair outcomes: Preoperative mitral regurgitation persists in the follow-up.

OBJECTIVE: This study aims to present the midterm outcomes of surgical correction of the anomalous left coronary artery from the pulmonary artery (ALCAPA) with a focus on mitral regurgitation (MR). METHODS: Medical records of 36 ALCAPA patients who underwent surgery in a single center were retrospectively reviewed. RESULTS: There were one (2.7%) neonate, 19 (52.7%) infants, 15 (41.6%) children, and 1 (2.7%) adult patient operated. Coronary reimplantation, tube reconstruction, and coronary artery bypass surgery techniques were performed in 29 (80.5%), 6 (16.6%), and 1 (2.7%) patient, respectively. Sixteen (44.4%) patients had a low ejection fraction and 13 (36.1%) patients had at least moderate MR preoperatively. None of our patients underwent a concomitant mitral surgery during the initial repair. Three and two patients died in the early and late postoperative period, respectively. Two patients underwent reoperation due to MR and pulmonary stenosis, separately. Preoperative MR was the only factor associated with at least moderate MR at the final follow-up (p < .01) and the presence of preoperative moderate or over-moderate MR estimated at least moderate MR at the final follow-up with 100% sensitivity and 80% specificity. CONCLUSION: Although the mitral valve was not repaired in the first operation, the reoperation rate is low. However, a moderate or higher preoperative MR predicted MR at the last follow-up. Performing annuloplasty in such patients can be a strategy to be researched.

High incidence of fever in patients after biointegral pulmonic valved conduit implantation.

OBJECTIVE: To describe our short- and medium-term outcomes using the BioIntegral pulmonic conduit. METHODS: Between August 2018 and September 2019, the BioIntegral pulmonic valved conduit was used for right ventricular outflow tract reconstruction in 48 patients. The data were retrospectively retrieved from the patient charts. RESULTS: The median age at surgery was 36 months (interquartile range [IQR] = 18-62 months). The diagnoses were pulmonary atresia-ventricular septal defect in 28 patients, absent pulmonary valve in four patients, truncus arteriosus in six patients, TGA-VSD-PS in five patients, conduit stenosis in three patients, and left venticular outflow tract obstruction requiring a Ross operation in two patients. In the postoperative short-term follow-up, 15 patients out of 48 had a high fever. Of these, 12 patients had concomitantly elevated C-reactive protein levels. There were no patients with blood culture positivity. The median postoperative length of hospital stay was 14 days (IQR = 8-21 days). The overall mortality was 4% in two patients, one died of right ventricular failure and multiple organ failure and one died of pulmonary embolism. The two patients who died were not among the 15 patients with fever. However, four patients with fever underwent balloon angioplasty for conduit restenosis in their medium-term follow-up. CONCLUSION: There was a high incidence of fever and adverse outcomes in the short-term postoperative follow-up of patients in whom the BioIntegral pulmonic valved conduit was implanted. Caution is advisable in using these conduits until there is convincing evidence about the sterilization and storage standards of these grafts.

Comparison of Doty and McGoon techniques for surgical reconstruction of congenital supravalvular aortic stenosis.

BACKGROUND: We aimed to compare the results of two surgical methods for the treatment of congenital supravalvular aortic stenosis. METHODS: From May 2004 to January 2020, 29 patients underwent surgical repair for supravalvular aportic stenosis in a single centre. The perioperative evaluation of the patients was retrospectively reviewed. RESULTS: Fifteen (51.7%) and 14 (48.2%) patients were treated with the Doty and the McGoon methods, respectively. The median age of our cohort was 4.5 (3.0-9.9) years. Ten (34.5%) patients had Williams-Beuren syndrome, and pulmonary stenosis was observed in 12 (41.3%) patients. The median follow-up time was 2.5 (0.7-7.3) years. On follow-up, five patients had residual stenosis with the McGoon technique and one with the Doty technique (p = 0.05). One patient died early in the post-operative period in the Doty group, and three patients were re-operated on due to restenosis in the McGoon group. Freedom from re-operation in the Doty group at 1, 3, 5, and 10 years was 100%. In the McGoon group, freedom from re-operation rates at the 1-, 3-, and 7-year follow-up were 100, 88.9, and 44.4%, respectively (p = 0.08). CONCLUSION: Our results with both surgical techniques suggest that supravalvular aortic stenosis can be treated with good results. The Doty method provided better relief for the supravalvular aortic segment, considering the residual stenosis and the re-operation rates.

Ligation of patent ductus arteriosus through anterior thoracotomy in preterm infants: a 10-year experience.

OBJECTIVE: The aim of this study is to report on the short-term and mid-term outcomes of preterm infants who underwent patent ductus arteriosus ligation through anterior mini-thoracotomy. METHODS: Data for 103 preterm infants who underwent patent ductus arteriosus clipping through an anterior mini-thoracotomy at the 2nd intercostal space between 2009 and 2019 were retrospectively reviewed. The patients were divided into two groups according to their weight at the time of surgery. The complications, morbidity, and mortality rates of each group were compared at postoperative day 30 and at the end of 1 year after surgery. RESULTS: During the operation, the median weight of the patients was 900 g (IQR 800-1125 g), the median age was 21 days (IQR 14.5-29 days). The lowest body weight was 460 g. In three patients (3%), there was intraoperative bleeding from the patent ductus arteriosus that required transition to median sternotomy. In one patient (1%) a residual patent ductus arteriosus that required reoperation was observed. Twelve patients (12%) died in the first 30 days postoperatively. Six patients (6%) died between the postoperative day 30 and 1 year. There was no statistically significant difference in the rates of mortality, morbidity, and complication between the groups. CONCLUSIONS: Based on our observations of over a hundred preterm infants with patent ductus arteriosus over a decade, ligation through anterior mini-thoracotomy is the main surgical procedure of choice for this patient group in our clinic. Our findings demonstrate the safety of this approach and we believe that it can be successfully replicated in other institutions.

The impact of COVID-19 pandemic on congenital heart surgery practice: An alarming change in demographics.

BACKGROUND: The aim of this study is to investigate the effect of COVID-19 outbreak on congenital cardiac surgery practice in a single center. METHODS: The first case of COVID-19 in our country was seen on March 11th, 2020. The patients operated between March 11th, 2019-and March 10th, 2020 were taken as the pre-COVID group, and those operated between March 11th and May 11th, 2020 were taken as the COVID group. The data was retrospectively collected, and the two periods were compared. RESULTS: Monthly average number of cases which was 52 patients/month (626 patients in 12 months) before COVID decreased to 35 patients/month (70 patients in 2 months) during COVID periods (P < .01). During the pre-COVID period the median postoperative length of hospital stay was 3 (IQR: 1-5) days. During the COVID period, this decreased to 1 (IQR: 1-3) day (P < .01). During the pre-COVID period, the hospital expenses of 17% (8/47) of the foreign nationals were covered by their homeland. The remaining 83% (39/47) were paid from the asylum seekers' fund. The proportion of foreign nationals operated significantly decreased during the COVID period ([7%; 47/632 vs 1%; 1/70]; P = .04). No significant difference was observed in terms of STAT mortality scores and categories and postoperative results of the operations performed between the two periods. CONCLUSIONS: Congenital cardiac surgery practice can be safely maintained with restricted case volume during the pandemic period. It is alarming that patients in the deprived areas cannot access pediatric cardiac surgery and possibly other health services because of closure of the borders between countries.

Early outcomes of usage of cryoFORM® probe for cryomaze procedure in congenital heart surgery.

OBJECTIVES: This study aimed to evaluate the early outcomes of patients who underwent a concomitant therapeutic maze procedure for congenital heart surgery. MATERIALS AND METHODS: Between 2019 and 2020, eight patients underwent surgical cryoablation by using the same type of cryoablation probe. RESULTS: Three patients had atrial flutter, two had Wolf-Parkinson-White syndrome, two intra-atrial reentrant tachycardia, and one had atrial fibrillation. Four patients underwent electrophysiological study. Preoperatively, one patient was on 3, two were on 2, five were on 1 antiarrhythmic drug. Six patients underwent right atrial maze and two underwent bilateral atrial maze. Five out of six right atrial maze patients underwent right atrial reduction. Nine different lesion sets were used. Some of the lesions were combined and applied as one lesion. In Ebstein's anomaly patients, the lesion from coronary sinus to displaced tricuspid annulus was delicately performed. The single ventricle patient with heterotaxy had junctional rhythm at the time of discharge and was the only patient who experienced atrial extrasystoles 2 months after discharge. Seven of the eight patients were on sinus rhythm. No patient needed permanent pacemaker placement. CONCLUSION: Cryomaze procedure can be applied in congenital heart diseases with acceptable arrhythmia-free rates by selecting the appropriate materials and suitable lesion sets. The application of cryomaze in heterotaxy patients can be challenging due to differences in the conduction system and complex anatomy. Consensus with the electrophysiology team about the choice of the right-left or biatrial maze procedure is mandatory for operational success.

Aortic translocation procedure: Early outcomes from a single center.

BACKGROUND/AIM: The data on the Nikaidoh procedure is limited in the literature. This study presents our experiential findings with aortic translocation (Nikaidoh) procedure and its modifications. METHODS: We retrospectively reviewed the data of all the patients who underwent aortic translocation in our institution. RESULTS: Between September 2014 and November 2018, eight patients underwent aortic translocation surgery. The median age was 3.2 years (14 months and 9 years). The diagnosis was transposition of the great arteries (TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction (LVOTO) in five patients (63%); double-outlet right ventricle (DORV), VSD, and LVOTO in two patients (25%) and DORV and remote VSD in one patient (12%). Additionally, two patients had tricuspid straddling. In terms of surgical procedures, six patients underwent standard Nikaidoh procedures, while one patient underwent double root translocation and one patient underwent a half-turned truncal switch operation. Hospital mortality was recorded for one patient (12.5%). Median intensive care and hospital stay duration was 3 days (interquartile range [IQR]: 2-5 days) and 11 days (IQR: 8-17 days). Median follow-up duration was 10 months (IQR: 10-24 months). One of the patients, who underwent standard Nikaidoh procedure, died in the late follow-up. CONCLUSIONS: Aortic translocation procedure is an option for TGA, VSD, and LVOTO patients with a distal VSD or tricuspid straddling and DORV patients with a small VSD. Avoiding the use of a conduit through modifications, such as double root translocation and half-turned truncal switch operation, may decrease the risk of long-term recurrent interventions.

Surgical Treatment of Pediatric Refractory Ventricular Tachycardia Originating From a Left Ventricular Rhabdomyoma.

Cardiac tumors are very rare in children, and echocardiography is very important in their detection. The clinical presentation can vary greatly depending on arrhythmia or obstruction. One of the most important factors determining the surgical approach is the clinical process. In this case report, we report the surgical treatment of a rhabdomyoma that caused refractory ventricular tachycardia.

Aortic valve neocuspidization with the Ozaki procedure in congenital aortic valve disease: Early results.

Background: In this study, we present our early results with the Ozaki procedure in the treatment of congenital aortic valve disease. Methods: Between July 2021 and July 2023, a total of 14 patients (10 males, 4 females; median age: 13.9 years; range, 8.5 to 15 years) who underwent neoaortic valve reconstruction of three leaflets using Ozaki procedure were retrospectively analyzed. Preoperative, postoperative, and follow-up echocardiogram images were evaluated. Results: Preoperative indications were aortic regurgitation (n=3) or combined aortic stenosis and regurgitation (n=11). The median aortic annular diameter was 23 (range, 19.5 to 25) mm on preoperative echocardiography. The median preoperative peak systolic aortic valve gradient for patients with aortic stenosis was 60 (range, 45 to 93) mmHg. The median preoperative aortic valve regurgitation grade was 4 (range, 3 to 4). Autologous pericardium and bovine pericardium were used in 12 and two patients, respectively. There was no conversion to valve replacement, myocardial infarction, or mortality in the early postoperative period. The median follow-up time was 8.5 (range, 6 to 19) months. One patient who performed the Ozaki procedure with bovine pericardium underwent valve replacement eight months later. Conclusion: The Ozaki procedure can be performed safely and effectively in congenital aortic valve stenosis and insufficiency with promising early results.

Risk factors and early outcomes of chylothorax following congenital cardiac surgery: A single-center experience.

Background: This study aims to investigate the incidence and risk factors for chylothorax and to evaluate the effect of chylothorax on the early postoperative outcomes following congenital cardiac surgery. Methods: A total of 1,053 patients (606 males, 447 females; median age: 12 months; range, 3 days to 48 years) who underwent surgery for congenital heart disease at our institute between January 2018 and December 2019 were retrospectively analyzed. Patients with chylothorax were identified and the data of this cohort was compared with the entire study population. Following the diagnosis of chylothorax, a standardized management protocol was applied to all patients. Results: Of 1,053 patients operated, 78 (7.4%) were diagnosed with chylothorax. In the univariate analysis, younger age, peritoneal dialysis, preoperative need for mechanical ventilation, surgical complexity, delayed sternal closure, high vasoactive inotrope score in the first 24 h after operation, residual or additional cardiac lesions which required reoperations were found to be the risk factors for chylothorax (p<0.05). In the multivariate analysis, the correlation persisted with only younger age, infections, and peritoneal dialysis requirement (p<0.05). In the chylothorax group, ventilation times were longer, and re-intubation and infection rates were higher (p<0.05). Although the length of intensive care unit and hospital stay was significantly longer in this patient group, there was no significant association between the development of chylothorax and in-hospital mortality (p>0.05). Conclusion: Chylothorax following congenital cardiac surgery is a significant problem which prolongs the length of hospital stay and increases the infection rates. Complex cardiac pathologies which require surgery at early ages and re-operations are risk factors for chylothorax. Although there is no consensus on the most optimal therapeutic strategy, standardizing the management protocol may improve the results.

Successful surgical repair of critical ascending and descending aortic aneurysms at nine-year intervals in Wiskott-Aldrich syndrome: A case report.

Wiskott-Aldrich syndrome is an uncommon X-linked inherited disorder related to primary immunodeficiency, infections, eczema, and thrombocytopenia. A 21-year-old male patient with this syndrome underwent descending aortic aneurysm repair at the age of 12. The patient had ascending aortic aneurysm with aortic valve regurgitation and surgical aortic root replacement was performed. To the best of our knowledge, this is the first case with Wiskott-Aldrich syndrome operated due to aneurysms development in different segments of the thoracic aorta in both childhood and young adult periods.

Comparison of cases with and without additional lower body perfusion in newborns undergoing aortic arch reconstruction with antegrade selective cerebral perfusion method.

Background: The aim of this study was to analyze the effect of additional lower body perfusion, compared to antegrade selective cerebral perfusion, on early postoperative outcomes after aortic arch repair in neonates with biventricular morphology. Methods: Between January 2017 and April 2020, a total of 46 neonates (34 males, 12 females; median age: 10 days; range, 7 to 14 days) with biventricular morphology underwent an aortic arch reconstruction were retrospectively analyzed. The effects of antegrade selective cerebral perfusion and additional lower body perfusion techniques on vital organ preservation and mortality were evaluated in these patients who underwent arch reconstruction. Results: In the univariate analysis of the whole cohort, postoperative creatinine level was lower in the additional lower body perfusion group, while there was no significant difference between the other parameters. In the multivariate analysis, intraoperative highest lactate level (odds ratio: 1.7; 95% confidence interval: 1.07-2.68; p=0.02) and postoperative 4th t o 6 th h lactate levels (odds ratio: 2.34; 95% confidence interval: 1.08-5.09; p=0.03) were independent predictors of early mortality. Mortality rate was higher in the antegrade selective cerebral perfusion group (22% vs. 7%), although it did not reach statistical significance. In the receiver operating characteristic curve analysis, the cut-off value for intraoperative lactate was 6.2 mmol/L (sensitivity: 85.7%, specificity: 71.1%) and the cut-off value for the lactate level at the postoperative 4th to 6th h was 4.9 mmol/L (sensitivity: 85.7%, specificity: 73.7%). Above these lactate levels were found to be associated with mortality. Conclusion: Additional lower body perfusion may have a role in vital organ protection in aortic arch repair of neonates, compared to antegrade selective cerebral perfusion.

A novel Fontan Y-graft for interrupted inferior vena cava and azygos continuation.

OBJECTIVES: To evaluate the hemodynamicdynamic advantage of a new Fontan surgical template that is intended for complex single-ventricle patients with interrupted inferior vena cava-azygos and hemi-azygos continuation. The new technique has emerged from a comprehensive pre-surgical simulation campaign conducted to facilitate a balanced hepatic flow and somatic Fontan pathway growth after Kawashima procedure. METHODS: For 9 patients, aged 2 to18 years, majority having poor preoperative oxygen saturation, a pre-surgical computational fluid dynamics customization is conducted. Both the traditional Fontan pathways and the proposed novel Y-graft templates are considered. Numerical model was validated against in vivo phase-contrast magnetic resonance imaging data and in vitro experiments. RESULTS: The proposed template is selected and executed for 6 out of the 9 patients based on its predicted superior hemodynamic performance. Pre-surgical simulations performed for this cohort indicated that flow from the hepatic veins (HEP) do not reach to the desired lung. The novel Y-graft template, customized via a right- or left-sided displacement of the total cavopulmonary connection anastomosis location resulted a drastic increase in HEP flow to the desired lung. Orientation of HEP to azygos direct shunt is found to be important as it can alter the flow pattern from 38% in the caudally located direct shunt to 3% in the cranial configuration with significantly reversed flow. The postoperative measurements prove that oxygen saturation increased significantly (P-value = 0.00009) to normal levels in 1 year follow-up. CONCLUSIONS: The new Y-graft template, if customized for the individual patient, is a viable alternative to the traditional surgical pathways. This template addresses the competing hemodynamic design factors of low physiological venous pressure, high postoperative oxygen saturation, low energy loss and balanced hepatic growth factor distribution possibly assuring adequate lung development. DATE AND NUMBER OF IRB APPROVAL: 25 October 2019, 280011928-604.01.01.

Single-center outcomes after surgical creation of aortopulmonary window in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

Background: The aim of this study is to present our experience regarding the creation of an aortopulmonary window as the initial palliative procedure. Methods: Between February 2016 and February 2021, a total of eight patients (3 males, 5 females; median age: 2 months; range, 0.7 to 6 months) who underwent aortopulmonary window creation were retrospectively analyzed. Data collection was performed by review from our institution. Results: There was no occurrence of early or late mortality in any patient. The median postoperative duration of mechanical ventilation and length of hospital stay were five and eight days, respectively. No postoperative reperfusion injury or severe pulmonary overcirculation was observed in any of the patients. Four patients achieved complete repair with unifocalization of the major aortopulmonary collateral arteries, one patient had a second procedure, and the remaining three patients waited for complete repair. The median right ventricle-toaortic pressure ratio after complete repair was 0.6 (range, 0.4 to 0.7). The median follow-up after complete repair was 1.4 (range, 0.9 to 2.8) years, and the median follow-up period for all survivors was 2.7 (range, 0.9-5.8) years. Conclusion: Our study results suggest that aortopulmonary window operation can be safely performed in selected patients with good early and mid-term outcomes. Although the central pulmonary arteries are very small, half of the patients underwent complete repair and achieved acceptable right ventricle-to-aortic pressure ratios. Patient selection criteria and early postoperative interventions are of utmost importance to prevent postoperative pulmonary overcirculation.