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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.

The aim of this study is to present our experience regarding the creation of an aortopulmonary window as the initial palliative procedure.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.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.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.

Background Malnutrition is a common problem in children with congenital heart disease, and it increases the risk of adverse outcomes in the postoperative period. Objectives We aimed to assess the association between malnutrition and cardiac surgery outcomes in paediatric patients aged 0–36 months. Methods This prospective cohort study was performed in a hospital specialising in paediatric cardiothoracic surgery. Children aged 0–36 months admitted to the paediatric cardiac intensive care unit after elective cardiac surgery between January 2018 and July 2018 were included in the study. We evaluated the patients' demographics and clinical variables, nutritional status, adverse outcomes, and 30-day mortality rates. Results A total of 124 cases met the inclusion criteria. Results showed that the Risk Adjustment for Congenital Heart Surgery score ≥5, underweight status (weight-for-age Z score ≤−2), and stunting (length-for-age Z score ≤−2) were all indicators for increased mortality following congenital heart surgery. Underweight children also spent a prolonged stay in the intensive care unit. Stunting (length-for-age Z score ≤−2) was the most strongly associated variable with mortality. Conclusion The results confirm the impact of malnutrition on mortality, postoperative infection, and length of hospitalisation in children undergoing surgery for congenital heart disease.

Background: in this study, we aimed to analyze the predictors and risk factors of mortality in patients who underwent Norwood I procedure with the diagnosis of hypoplastic left heart syndrome. Methods: between January 2009 and December 2020, a total of 139 patients (95 males, 44 females) who underwent Norwood I procedure with the diagnosis of hypoplastic left heart syndrome in our center were retrospectively analyzed. Results: the median birth weight was 3,200 (range, 3,000 to 3,350) g and the median age at the time of operation was seven (range, 5 to 10) days. Pulmonary flow was achieved with a Sano shunt in the majority (72%) of patients. Survival rate was 41% after the first stage. Reoperation for bleeding (p=0.017), reoperation for residual lesion (p=0.011), and postoperative peak lactate level (p=0.029), were associated with in-hospital mortality. Nineteen (33%) of 57 patients died before the second stage. Thirty-three (58%) patients underwent second stage, and survival after the second stage was 94%. Thirteen patients underwent third stage, and survival after the third stage was 85%. Estimated probability of survival at six months, and one, two, three, and four years were 33%, 33%, 25%, 25%, and 22% respectively. Conclusion: hospital and inter-stage mortality rates are still high and this seems to be the most challenging period in term of survival efforts of the patients with hypoplastic left heart syndrome. Early recognition and reintervention of anatomical residual defects, close follow-up in the inter-stage period, and the accumulation of multidisciplinary experience may help to improve the results to acceptable limits. / Amaç: bu çalışmada, hipoplastik sol kalp sendromu tanısıyla Norwood I işlemi uygulanan hastalarda mortalitenin öngörücüleri ve risk faktörleri incelendi. Ça¬lış¬ma pla¬nı: Ocak 2009 ve Aralık 2020 tarihleri arasında merkezimizde hipoplastik sol kalp sendromu tanısıyla Norwood I işlemi uygulanan toplam 139 hasta (95 erkek, 44 kadın) retrospektif olarak incelendi. Bulgular: medyan doğum ağırlığı 3200 (dağılım, 3000-3350) g ve ameliyat sırasında medyan yaş yedi (dağılım, 5-10) gün idi. Hastaların büyük kısmında (%72) pulmoner akım, Sano şant ile sağlandı. İlk aşama sonrası sağkalım %41 idi. Kanama nedeniyle tekrar ameliyat (p=0.017), rezidü anatomik lezyon nedeniyle tekrar ameliyat (p=0.011) ve ameliyat sonrası birinci gün pik laktat seviyesi (p=0.029) hastane mortalitesi ile ilişkili idi. Taburcu edilen 57 hastanın 19’u (%33) ikinci aşamaya ulaşamadan kaybedildi. Otuz üç (%58) hastaya ikinci aşama ameliyatı uygulandı ve ikinci aşama sonrası sağkalım %94 idi. On üç hastaya üçüncü aşama ameliyatı uygulandı ve üçüncü aşama sonrası sağkalım %85 idi. Altı ay ve birinci, ikinci, üçüncü ve dördüncü yılda tahmini sağkalım olasılığı sırasıyla %33, %33, %25, %25 ve %22 idi. So¬nuç: hastane ve aşamalar arası mortalite oranları halen yüksek olup, hipoplastik sol kalp sendromu tanılı hastaların sağkalma çabalarındaki en zorlayıcı zaman dilimi olarak görünmektedir. Anatomik rezidü defektlerin erken tanılanması ve erken yeniden girişim yapılması, aşamalar arası dönemde yakın takip ve multidisipliner birikim ve tecrübe sonuçların kabul edilebilir seviyelere ulaşmasına yardım edebilir., NA

Introduction: There is limited data on totally anomalous pulmonary venous connection (TAPVC) repair results in our country. The aim of this study is to evaluate the early postoperative results of a large series of TAPVC patients operated in our clinic. Patients and Methods: The data of the patients who underwent TAPVC repair in our clinic between May 2005 and May 2021 were retrospectively reviewed using hospital records. Results: A total of 150 TAPVC patients aged between 0 days and 39 years (median 3 months, IQR: 18 days-9 months), 92 males (61%) and 58 females (39%) underwent surgical intervention. The median length of hospi- tal stay was 12 (IQR: 7-16) days. Overall mortality was 14% (21 patients). Seventeen out of 32 patients with additional cardiac anomalies (53%) and 4 out of 118 patients with isolated TAPVC (3%) died. The presence of additional cardiac anomalies was associated with mortality (p< 0.00001). Mortality rate in univentricular patients was 59% (10 patients). This rate was higher in comparison to the mortality rate of biventricular patients (8%; p< 0.00001). Three patients were reoperated due to postoperative pulmonary venous stenosis. Conclusion: The TAPVC outcomes in isolated and biventricular patients were favorable with a low mortality and postoperative pulmonary venous stenosis in this cohort. Patients with a single ventricle physiology and/or heterotaxia had a significantly increased risk of mortality, which might be due to the intrinsic challenges of the univentricular physiology regarding the balance between the pulmonary and systemic circulations. Tendency for increased mortality in the cases with preoperative pulmonary venous obstruction is a potential target for improvement.

OBJECTIVES This study aims to compare the early- and long-term outcomes of patients who undergo owl’s eye pulmonary artery (PA) reconstruction to those of patients who undergo conventional PA reconstruction. METHODS From January 2016 to January 2017, 64 consecutive patients underwent an arterial switch operation. The patients were divided into 2 groups in terms of neo-PA reconstruction method: 30 patients who underwent neo-PA reconstruction by owl’s eye technique were defined as group 1 and 34 patients who underwent neo-PA reconstruction by the conventional approach were defined as group 2. In the final model, after propensity matching, 23 patients from each group with similar propensity scores were included in the study. RESULTS There was no significant difference between the groups regarding patient characteristics and operative findings. In the early period, the duration of intensive care unit and hospital stays and the rate of mild neo-pulmonary stenosis (neo-PS) were significantly higher in the owl’s eye group (P = 0.04, 0.04 and 0.03). In the late period, the rate of severe neo-PS and reintervention was significantly higher in the owl’s eye group (P = 0.02 and 0.04). Furthermore, the rates of 3-year freedom from pulmonary reintervention and freedom from moderate–severe neo-PS were significantly lower in group 1 (P = 0.04). In addition, the owl’s eye reconstruction was the only factor independently related to moderate–severe neo-PS in the long term (hazard ratios = 11.2, P = 0.02). CONCLUSIONS We have abandoned the owl’s eye method for neo-PA reconstruction of the neo-PA because of serious complications. According to our series and the literature, reconstruction of the neo-PA with an oversized, pantaloon-shaped fresh autologous pericardial patch is still superior to the other techniques.

Background: The aim of this study was to evaluate the predictability of postoperative pulmonary artery pressure (PAP) using intraoperative flow study in patients undergoing bidirectional Glenn operation. Methods: Patients who underwent Glenn operation under cardiopulmonary bypass (CPB) were included in the study. During the operation, after the completion of additional procedures under CPB, an intraoperative flow study was performed prior to Glenn anastomosis. After the completion of bidirectional Glenn, the patient was separated from the CPB and PAP was measured. The relationship between this pressure and flow study measurement was analyzed. Results: Nine patients who underwent bidirectional Glenn operation with additional procedures under CPB between July 2018 and January 2019 were included in the study. The median PAP was 9 mm Hg (interquartile range [IQR]: 7-10 mm Hg) in the flow study and 10 mm Hg (IQR: 8-11 mm Hg) after CPB, and the median difference between these pressures was 1 mm Hg (IQR: 1-3 mm Hg). There was a strong correlation between these two measurements ( r = 0.732; P = .025). Conclusion: The results of this study show that PAP after the Glenn procedure can be estimated using an intraoperative flow study. We believe that this method may be useful in intraoperative decision-making for Glenn operation in single ventricular patients who require extensive pulmonary artery (PA) reconstruction due to limited PA development, branch PA stenosis, or nonconfluent PAs. Also, this method can be used as a sort of intraoperative pulmonary resistance reversibility study in patients with high preoperative pulmonary vascular resistance due to surgically correctable pulmonary venous hypertension.

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.

Pulmonary atresia with ventricular septal defect (PA-VSD) is a congenital complex cardiac anomaly. The blood supply to the lungs can be from patent ductus arteriosus (PDA), major aortopulmonary collateral artery (MAPCA) or in very rare cases from coronary artery-pulmonary artery fistula (CAPAF). We had coronary artery-pulmonary artery fistula (CAPAF) in 2 patients which had surgical intervention. In our first patient which was operated 10 years ago, the main pulmonary artery had a source from LMCA. In second patient, the right pulmonary artery derived from LMCA and advanced to the right lung from the posterior of the aorta and the left pulmonary artery was coming out of the PDA.

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.

Objective This study aims to present our experience with single-stage complete unifocalization and intraoperative flow study for the repair of ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. Methods This study was conducted through retrospective chart review of all the patients who underwent complete single-stage midline unifocalization in a single tertiary-care institution. Results Twenty-two patients underwent midline single-stage unifocalization. The median age was 11 months (IQR: 5-21 months). The number of collateral arteries unifocalized was between one and three (median two). In-hospital mortality was 5%. Follow-up was complete; and the median follow-up regarding survival was 20 months (IQR: 10-28 months). There were three late deaths, and the estimated survival rate was 80% at 10 months and on. Out of 22 patients, ventricular septal defect was closed in the first surgery in three patients (14%) and the second surgery in four patients (19%). Total seven patients underwent surgical total repair (32%). Additionally, one out of four patients whose ventricular septal defects were closed with a fenestrated patch is under follow-up with a small ventricular septal defect, while two are waiting for ventricular septal defect closure. Therefore, total eight patients (36%) have reached total correction. Conclusion Single-stage unifocalization is a feasible treatment option in ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. This cohort had unfavorable results regarding the rate of complete repair. The pitfalls encountered were related to problems with meticulous surgical technique, complete unifocalization, and correct implementation of the flow study.

Objective The Ross procedure has been cited as the procedure of choice for young patients requiring aortic valve replacement. However, potential for reintervention requirement in both left and right ventricular outflow tracts can be a source of concern. The aim of the present study was to describe our experience with this procedure. Methods A retrospective chart review of all the patients who underwent the Ross procedure in a single institution was performed. National death registry records were used for late mortality. Results Eighteen Ross procedures between May 2003 and May 2018 were performed. The median age of the cohort was 15 [interquartile range (IQR): 12-18] years. The pulmonic conduit was a homograft in 11 patients, Labcor in 5 patients, Contegra in 1 patient, and Medtronic Freestyle Valve in 1 patient. There were three early deaths. The median follow-up of 15 hospital survivors was 11 (IQR: 3-14) years. Any late mortality was not observed. In the two surviving patients with infective endocarditis, there was no recurrent infective endocarditis. Freedom from reintervention was 80% at 8 years and onward. Any risk factors associated with reintervention could not be identified. However, freedom from autograft dilatation at 10 years was 45%. Conclusion Autograft failure is a potential problem in the long-term follow-up of Ross patients. Freedom from reintervention was satisfactory, and the type of pulmonic conduit did not affect the mid-term outcomes. In patients with infective endocarditis, the Ross procedure has a low recurrence rate, but it might have an increased risk of mortality.

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.

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.

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.

BACKGROUND The aim of this study is to describe the short-term and mid-term outcomes of the preterm infants who underwent patent ductus arteriosus (PDA) ligation in by anterior mini-thoracotomy. METHODS Data of 103 preterm infants who underwent PDA ligation by anterior mini-thoracotomy between 2009 and 2019 were retrospectively reviewed. PDA was clipped through an anterior mini-thoracotomy at the 2nd intercostal space. Outcomes were defined according to complications, morbidity and mortality rates within the postoperative 30 days and one year. RESULTS The median weight of the patients during the operation was 900 (IQR800–1125 g) grams and the lowest body weight was 460 grams. The median age at the operation was 21 (IQR14,5–29 days) days. In three patients (3%) there was intraoperative bleeding from the PDA that required transition to median sternotomy. In one patient (1%) residual PDA was seen and this patient was reoperated on the 4th postoperative day. One patient (1%) underwent surgical revision for chylothorax and chylomediastinum causing late cardiac tamponade on the 26th postoperative day. Twelve patients (12%) died in the first 30 days postoperatively. Six patients (6%) died between the thirtieth day and a year. CONCLUSIONS After a decade and over a hundred patients with PDA ligation through anterior mini-thoracotomy in preterm infants is still the main procedure of choice in this patient group in our clinic. Our outcomes demonstrate the safety of this approach and we believe that it can be reproducible.

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

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

Objective: To compare the early and long-term results of patients in whom was performed modified closed coronary transfer with the results of patients in whom was performed trap-door transfer techniques by utilizing propensity-matching analysis to provide optimal identical patient matching for the groups. Methods: From August 2015 to December 2017, 127 consecutive patients underwent arterial switch operation due to simple and complex transposition of the great arteries, with or without additional arch and complex coronary pattern, by a single surgical team included into the study. Of these, in 70 patients it was performed modified closed coronary transfer technique and in 57 patients it was performed trap-door style coronary transfer technique. The patients were divided into two groups in terms of coronary transfer method. In the final model, after propensity matching, 47 patients from each group having similar propensity score were included into the study. Results: There was no significant difference between the groups regarding patient characteristics. Cross-clamp time and operation time were significantly lower in the modified technique group compared with the other group (P=0.03 and P=0.05, respectively). When compared the early and late postoperative outcomes, there was no significant difference between the groups. Postoperative echocardiographic findings were mostly similar between the groups. Conclusion: The patients in whom was performed our modified technique demonstrate overall good outcomes and the current technique ensures shorter arterial cross-clamp and operation times. It may be an alternative method to the trap-door technique for the coronary transfer during the arterial switch operation.

An eight-year-old boy with tricuspid atresia was found to have atretic coronary sinus ostium during cardiac catheterization. Single-stage extracardiac fenestrated Fontan operation was performed with surgical unroofing of the coronary sinus into the left atrium to avoid the risk of cardiac congestion.

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.

Background This study aimed to describe the learning curve of the extracardiac Fontan procedure in a single center and to analyze the changes in clinical applications and outcomes. Methods A retrospective chart review of all extracardiac Fontan patients in a single tertiary care center was undertaken. Patients with a diagnosis of hypoplastic left heart syndrome and those who had undergone a lateral tunnel modification, intra/extracardiac Fontan, Kawashima procedure, or inferior vena cava-to-azygous vein connection were excluded from the analysis. Results Between May 2004 and February 2018, data of 159 extracardiac Fontan patients were analyzed. The median age was 5.5 years (range 4.5–8.2 years). Based on a cumulative sum analysis, a hinge point was determined to divide the cohort into 2 phases. Phase 1 ( n = 70) represented the first learning phase and phase 2 ( n = 89) represented the later phase. Mortality decreased in phase 2 (2/89; 2%) compared to phase 1 (10/70; 14%; p = 0.004). Two (3%) patients had extracorporeal membrane oxygenation in phase 1, and 5 (6%) in phase 2 ( p = 0.47). More patients in phase 2 underwent a prior bidirectional Glenn procedure (83/89 vs. 57/70; p = 0.02), fenestration (80/89 vs. 9/70; p Conclusions This study shows that increased use of extracorporeal membrane oxygenation, strict implementation of the three-stage management plan, routine fenestration, and a low threshold for pulmonary artery reconstruction may be associated with decreased mortality in the extracardiac Fontan procedure.

THE INCIDENCE of congenital heart defects is approximately 7 to 10 per 1,000 live births. With advancing technology and improved diagnostic, surgical, anesthetic, and postoperative management protocols, the tendency today is to perform the complete repair of defects early in infancy. Infancy is defined as the period from birth until age 1. The management of cardiopulmonary bypass (CPB) in infancy has many challenges and increased risk of morbidity and mortality compared with that of the adult population. Infants are more prone to complications because of the immaturity of their organ systems. There is a need for a more complete understanding of the anatomic, metabolic, and physiologic differences between infants and adults, and patient care must be individualized considering the degree of hemodilution and hypothermia, acidbase strategies, flow rates, circuit designs, priming the pump, and choice of cannulae. This review article outlines the differences between infants and adults regarding their responses to CPB and reviews the main subjects like hypothermia, hemodilution, acid-base strategies, inflammatory response, extracorporeal circuit, and CPB protocols for infants. Since the first successful application of extracorporeal circulation in the 1950s, CPB has evolved and still is evolving, but basic concepts have remained the same: Oxygenation and carbon dioxide elimination, perfusion, systemic cooling and rewarming, and removal of blood from the heart to provide a blood-free surgical field. On the other hand, despite clear understanding of the basic concepts and many improvements, CPB management in infants still remains challenging and unique.

Amac: Bu calismada yeni gelistirilen bir enflamatuvar parametre olan notrofil/lenfosit oraninin koroner arter baypas greft (KABG) cerrahisi sonrasi erken donem mortalite prediktoru olarak etkisi arastirildi. Ca­lis­ma­pla­ni:­Aralik 2011 Nisan 2012 tarihleri arasinda klinigimizde izole KABG cerrahisi yapilan ardisik 210 hasta prospektif olarak incelendi. Hastalarin baslangic ozellikleri ve ameliyat oncesi notrofil/lenfosit oranlari belirlendi. Ameliyat sonrasi takip suresi 86.1±38.9 gun idi. Primer sonlanim noktasi, tum-nedenlere bagli mortalite olarak belirlendi. Bul gu lar: Dort hastada ameliyat sonrasi ilk 30 gunde olmak uzere, toplam sekiz hastada (%3.8) mortalite goruldu. Tek degiskenli analizlerde ameliyat oncesi notrofil/lenfosit orani, mortalite gelisen ve gelismeyen gruplar arasinda anlamli derecede farkli olarak saptandi (p=0.037). Alici islem karakteristikleri (ROC) egrisinde notrofil/lenfosit orani icin esik degeri 2.81 olarak belirlendi (AUC=0.72, duyarlilik: %75, ozgulluk: %67). Iki grup arasinda anlamli farka sahip olan degiskenler ile yapilan lojistik regresyon analizinde ise, notrofil/lenfosit orani icin esik degerinin uzerinde olmasi mortalite icin bagimsiz bir prediktor olarak saptandi (OR 6.47, %95 CI 1.18-35.38, p=0.031). So­nuc:­Kolay bir sekilde hesaplanabilen notrofil/lenfosit orani KABG cerrahisi sonrasi erken donem mortaliteyi ongormede bagimsiz bir faktor olarak kullanilabilir. Anah tar soz cuk ler: Koroner arter baypas greftleme; lenfosit; mortalite; notrofil. Background:­ In this study, we aimed to investigate the newly introduced inflammatory biomarker, neutrophil/ lymphocyte ratio, as a mortality predictor following coronary artery bypass graft (CABG) surgery. Methods: Between December 2011 and April 2012, 210 consecutive patients who underwent isolated CABG surgery in our clinic were prospectively analyzed. The baseline characteristics of the patients and preoperative neutrophil/ lymphocyte ratio were determined. The postoperative follow-up was 86.1±38.9 days. The primary endpoint was all-cause mortality. Results:­Eight patients (3.8%) died of whom four deaths occurred during the first 30 days of follow-up. Univariate analyses revealed a significant difference in the preoperative neutrophil/lymphocyte ratio between the groups in which mortality was seen and the group in which no mortality was observed (p=0.037). The Receiver operating characteristic (ROC) curve showed a threshold value of 2.81 for neutrophil/lymphocyte ratio (AUC=0.72, sensitivity: 75%, specificity: 67%). Logistic regression analysis of the variables with significant differences between two groups revealed that the neutrophil/lymphocyte ratio over its threshold value was an independent predictor for mortality (OR 6.47, 95% CI 1.18-35.38, p=0.031). Conclusion:­ Neutrophil/lymphocyte ratio, which can be easily calculated, can be used as an independent factor in predicting early mortality following CABG surgery.

Background The aim of this study was to determine whether prophylactic use of calcium dobesilate (CD) can improve venous function after saphenous vein harvest in coronary artery bypass graft (CABG). Materials and Methods A total of 100 patients who underwent elective CABG were divided into four equal groups. In Group A, the greater saphenous vein (GSV) was harvested below the knee and, in Group B, through the knee till the groin. These patients remained untreated. Group C received CD in a dosage of 1500 mg po daily after the GSV was harvested below the knee. Group D received same dosage of CD after the GSV was harvested through the knee till the groin. Venous function of ipsilateral leg was evaluated clinically and by Doppler ultrasonography in the postoperative first week and second month. Results Clinical findings of venous insufficiency were observed with a similar rate between groups at both early and late periods. In Groups A and B, after 2 months, flow velocities decreased and reflux periods increased significantly. In groups C and D, treatment with CD for 2 months after saphenectomy resulted in a significant increase in flow velocities and a significant decrease in reflux periods. Patients in Groups B and D have significantly more impaired venous functions. Conclusion Saphenectomy results in ipsilateral leg venous dysfunction, which seems to be unrelated to leg swelling and to be more prominent in patients with high-level saphenectomy. In addition, to be careful about the restriction of the saphenectomy procedure into the most appropriate level, prophylactic use of CD can prevent this deterioration when it was added to varice socks.

Ocular infections after a heart transplant are rare; but when present, they generally appear during the first year after surgery. Ocular infections may cause significant loss of vision and morbidity if not diagnosed early. For that reason, heart transplant patients should undergo a routine visual examination during follow-up. We report our experience regarding the followup and treatment of a case of toxoplasma retinitis diagnosed in one of our heart transplant recipients.

Objective: Repair of congenital heart defects involving the right ventricular outflow tract (RVOT) may require pulmonary valve replacement at the time of primary repair or reoperation. This study compares the outcomes of bovine jugular vein grafts (BJV, Contegra, Medtronic Inc.) with cryopreserved pulmonary homografts (PHs) in patients with RVOT obstruction at a single institution. Methods: We reviewed the outcomes of all BJVs and PHs implanted for RVOT reconstruction from 1999 to 2010. Echocardiographic data were reviewed to evaluate valve performance. Graft dysfunction is defined as RVOT obstruction with peak echo-Doppler gradient >40 mm Hg and/or grade III/IV conduit valve regurgitation. Graft failure is defined as need for conduit replacement or need for catheter or surgical reintervention. Results: A total of 216 patients who received BJVs (n = 153) and PHs (n = 63) were studied. There was no significant difference between the groups with respect to mean age, body surface area, conduit indication, or conduit diameter, though mean follow-up duration was longer in patients that received homografts. Conduit dysfunction and conduit failure and need for explantation were worse for homografts, albeit at longer follow-up interval. Distal stenosis and actuarial survival were similar. Conclusions: In the first ten years after pulmonary implantation of BJVs and PHs, survival and freedom from distal stenosis are statistically similar, but freedom from failure, dysfunction, and explantation are significantly better for BJV conduits. The BJV conduit is a good alternative in patients who require RVOT reconstruction.

Background For children with congenital aortic stenosis (AS) who are selected for biventricular repair, valvuloplasty can be achieved by surgical aortic valvuloplasty (SAV) or by transcatheter balloon aortic dilation (BAD). A retrospective study was undertaken to compare the effectiveness of BAD versus SAV, evaluating the long-term survival, incidence of aortic valve restenosis or aortic insufficiency (AI) or both, and freedom from reoperation for repeated valve repair or replacement. Neonates less than 2 months of age were excluded from this comparison. Methods We reviewed the outcomes of children undergoing repair by SAV (n = 89) and BAD (n = 69) at our institution during a recent 20-year period. Clinical and echocardiographic follow-up were analyzed. The patient groups were compared with regard to the persistence or recurrence of postoperative aortic gradients and valve insufficiency and valve-related reintervention, including aortic valve replacement (AVR). Results There was no significant difference between the groups with respect to mean age, body surface area, valve anatomy, sex, and preoperative gradients. Our data demonstrate that gradient reduction, AI, and the need for reintervention were worse for BAD. Aortic gradients at last follow-up were similar in both cohorts, but return of a significant gradient occurred sooner for patients who had BAD. Aortic gradient at discharge was significantly better for the patients who underwent SAV. Kaplan-Meier analysis showed that at 10 years, comparison of SAV and BAD was as follows: freedom from reintervention, 72% versus 53% (p = 0.02) and freedom from AVR, 80% versus 75% (p = 0.32). Conclusions BAD yields less gradient reduction, more postprocedural AI, and a shorter interval between initial and subsequent reintervention than does SAV. Our results demonstrate that SAV is safe and effective and that residual gradients and degree of AI are low. After SAV, the need for AVR can usually be delayed until the child is significantly older. The long-term functional stability after SAV is excellent. BAD in comparison is associated with an increased frequency and severity of AI and the need for earlier reintervention and valve replacement. SAV should be offered to all patients beyond the newborn period because it gives superior and longer lasting palliation.

We planned a prospective study to evaluate the regression of left ventricular mass one year after surgery in older patients (or=55 years) who have undergone aortic valve replacement with stentless bioprostheses or mechanical valve.Between 1998 and 2000, 54 patients had aortic valve replacement with stentless bioprostheses (14 CryoLife-O'Brien, 40 Medtronic Freestyle), and 62 patients had aortic valve replacement with St. Jude Medical mechanical valve. In preoperative echocardiographic evaluation, the St. Jude group had the highest left ventricular mass (LVM). The difference between groups was significant (O'Brien group: 329+/-75 g, Medtronic group: 382+/-116 g, St. Jude group: 445+/-138 g, p=0.006). The same level of statistical significance was achieved when LVM indexes were compared (LVMI; 192+/-39 g/m2, 217+/-65 g/m2, 255+/-78 g/m2, respectively, p=0.008). All the surviving patients were evaluated echocardiographically one year after surgery.Regression of LVM occurred in all patients after aortic valve replacement: 26% in the O'Brien group, 37% in the Medtronic group and 32% in the St. Jude group. The difference between the groups was not significant. LVM and LVMI's were significantly lower when compared with preoperative values, but the degree of regression was not different between groups one year after surgery.Regression of left ventricular mass after aortic valve replacement is directly related to the preoperative ventricular mass and independent of the valve type in older patients. The choice of prosthesis should be based on patient characteristics.

Through a median sternotomy, we performed a single-stage repair of severe aortic coarctation, ventricular septal defect, patent foramen ovale, and mitral valve insufficiency. The severe aortic coarctation was repaired by interposing a synthetic graft between the distal ascending aorta and the descending aorta. We first repaired the coarctation with the 38-year-old man on cardiopulmonary bypass, before aortic cross-clamping, in order to shorten the cross-clamp time.