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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: 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.

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.

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 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 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

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: This study aims to evaluate the surgical results of our clinic according to presumption systems of Risk Adjustment in Congenital Heart Surgery, Aristotle Basic Complexity score, Aristotle Comprehensive Complexity score, and Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories and to compare the efficiency of these systems in predicting morbidity and mortality. METHODS: In the study, classification and the risk scoring were performed with the four different systems for 1,950 patients (1,038 males, 912 females; mean age 5.5 months; range, 1 day to 18 years) who were administered congenital heart surgery between 1 October 2012 and 31 December 2016. The hospital mortality and morbidity were calculated for each category from the four models. The discriminatory ability of the models was determined by calculating the area under the receiver operating characteristic curve and the receiver operating characteristic curves of the four models were compared. RESULTS: Median weight of the patients was 7.2 kg (range, 1.8-80 kg). Among the patients, 53% were males and 47.5% were younger than one year of age. Of totally 1,950 operations, mortality was observed in 149 (7.6%) and morbidity was observed in 541 (27.7%). Areas under the receiver operating characteristic curve for mortality were 0.803, 0.795, 0.729, and 0.712 for the Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Aristotle Comprehensive Complexity, Risk Adjustment in Congenital Heart Surgery, and Aristotle Basic Complexity scores, respectively. Areas under the receiver operating characteristic curve for morbidity were 0.732, 0.731, 0.730, and 0.685 for the Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Risk Adjustment in Congenital Heart Surgery, Aristotle Comprehensive Complexity, and Aristotle Basic Complexity scores, respectively. CONCLUSION: Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Risk Adjustment in Congenital Heart Surgery, Aristotle Basic Complexity, and Aristotle Comprehensive Complexity score systems were effective in predicting the morbidities and mortalities of patients who underwent congenital heart surgery and evaluating the performance of the surgical centers. Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories were on the forefront due to high feasibility and performance. Aristotle Basic Complexity score system had the lowest performance. Combinations of systems will provide the most benefit during evaluation of results.

Non-syndromic congenital supravalvular aortic stenosis (SVAS) leads to ventricular hypertrophy and increased oxygen consumption, and when combined with other factors reduces coronary blood flow, potentially resulting in myocardial ischemia and sudden cardiac death. While the anatomic obstruction of coronary circulation is as common in non-syndromic SVAS as in Williams syndrome, it often remains unacknowledged. Extracorporeal membrane oxygenation (ECMO) is an elective procedure that can be used to support patients with cardiac arrest during diagnosis as a way to reduce cardiopulmonary load in preparation for surgery or further treatment. In this report, we describe the rare case of an infant with severe SVAS and mild valvular pulmonary and left main coronary artery stenosis, as well as breath-holding spells. After multiple cardiac arrests, the infant underwent diagnostic catheter angiography on ECMO and had the pathology surgically corrected.

Background:­This study aims to compare two surgical techniques [right lateral minithoracotomy (RLMT) and median sternotomy (MS)] used to close atrial septal defects (ASD), and measure patient satisfaction, cosmetic results, and patient perception with tests used for evaluating scar and mental status. Methods: A total of 26 ASD patients were included in the study, of which 14 were operated with the MS method (group MS), and 12 were operated with the RLMT method (group RLMT). Patients were divided into two groups according to the incision sites. Patients’ incision scars were assessed objectively according to Vancouver Scar Scale (VSS), and Patient and Observer Scar Assessment (POSAS) criteria. Besides, patients were administered Patient Satisfaction Score (PSS), Body Image Questionnaire (BIQ), and The Hamilton Anxiety Rating Scale (HAM-A) to measure their perception. Results:­ Overall patient satisfaction was greater in the RLMT group than the MS group (p=0.021). There was no significant difference between the two groups in terms of VSS, BIQ, HAM-A, and skin type. Average scar length (p

Peripheral artery cannulation which allows continuous blood pressure monitoring is an easy way for blood sampling. Rarely, peripheral arteries can not be cannulated percutaneously or by cutdown technique. In this article, we describe a six-month-old female infant with a weight of six kilograms whom the left internal mammary artery was cannulated for monitoring arterial blood pressure due to unsuccessful catheterizations of other peripheral arteries. This line continued to function for 10 days postoperatively without any difficulty or complication. The internal mammary artery provides reliable access for blood pressure monitoring and arterial blood sampling in intraand postoperative period in pediatric patients.

OBJECTIVES: To investigate the effects of intravenous iloprost on pulmonary artery hypertension (PAH) in infants undergoing congenital heart surgery. METHODS: In this prospective, randomized study, the study group (n = 15) received a continuous infusion of iloprost (2.0 ng/kg/min) that was delivered immediately after weaning from cardiopulmonary bypass and continued for 72 h postoperatively. Patients in the control group (n = 12) were managed conventionally. The groups were compared in terms of postoperative data, including systolic and mean pulmonary artery (PA) pressures, PA/systemic pressure ratio, lactate level, PAH crisis, ventilation time, reintubation and lengths of intensive care unit (ICU) and hospital stay. Transthoracic echocardiography was used to assess PA pressures at 1 day, 7 days and 30 days after surgery. RESULTS: No mortality occurred. PAH crisis occurred in 2 (16.6\%) patients in the control group and 4 (26.7\%) patients in the study group (P = 0.53). Postoperative PA pressures and PA/systemic pressure ratios were similar between the groups (P > 0.05). The durations of ICU (P = 0.40) and hospital (P = 0.98) stays were similar between the groups. Echocardiographic studies demonstrated a significant decrease in postoperative PA pressures in the control (P = 0.001) and study (P = 0.0001) groups. However, no significant change was observed between the groups (P > 0.05). The Tukey multiple comparison test showed a significant decrease in PA pressures at each follow-up in both groups (P < 0.05). CONCLUSIONS: Intravenous iloprost demonstrated no additional benefit over the conventional management of infants with PAH after repair of intracardiac defects. Clinicians may prefer other alternative agents in infants with a high risk of PAH crisis.