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This study evaluates a novel surgical technique inspired by the hybrid Norwood procedure, which involves ductal graft placement and bilateral pulmonary artery banding. This technique is designed to avoid the high-risk classical Norwood stage 1 operation and to avoid the need for first stage invasive catheter interventions and interstage catheter interventions when ductal stent placement is not feasible in developing countries with limited resources. Between June 2015 and December 2022, 14 patients with hypoplastic left heart syndrome and variants were treated using this procedure at our center. Polytetrafluoroethylene graft was used for ductal graft procedure and polytetrafluoroethylene bands were used for bilateral pulmonary banding. Atrial septectomy was done selectively. Patients with adequate left heart growth underwent biventricular repair and pulmonary debanding. Patients without left heart growth underwent cavopulmonary anastomosis or necessary intermediate surgical revisions. Seven patients out of 14 reached the second stage. Two received biventricular repair, three underwent bidirectional cavopulmonary anastomosis, and two required repeat palliations other than bidirectional cavopulmonary anastomosis. Modifications of the Norwood procedure is an ongoing process due to the complex nature of hypoplastic left heart syndrome and variants. As experience grows, the mortality associated with ductal graft and bilateral pulmonary banding procedure may decrease, making this technique an alternative for centers where classical Norwood operation or hybrid palliation is not possible, especially in developing countries with limited resources. We might predict promising outcomes, especially for patients with hypoplastic left heart complex where biventricular repair is expected.

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Background: This second harvest of the Congenital Heart Surgery Database intended to compare current results with international databases. Methods: This retrospective study examined a total of 4007 congenital heart surgery procedures from 15 centers in the Congenital Heart Surgery Database between January 2018 and January 2023. International diagnostic and procedural codes were used for data entry. STAT (Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery) mortality scores and categories were used for comparison of the data. Surgical priority status was modified from American Society of Anesthesiologist guidelines. Centers that sent more than 5 cases to the database were included to the study. Results: Cardiopulmonary bypass and cardioplegic arrest were performed in 2,983 (74.4%) procedures. General risk factors were present in 22.6% of the patients, such as genetic anomaly, syndrome, or prematurity. Overall, 18.9% of the patients had preoperative risk factors (e.g., mechanical ventilation, renal failure, and sepsis). Of the procedures, 610 (15.2%) were performed on neonates, 1,450 (36.2%) on infants, 1,803 (45%) on children, and 144 (3.6%) on adults. The operative timing was elective in 56.5% of the patients, 34.4% were urgent, 8% were emergent, and 1.1% were rescue procedures. Extracorporeal membrane oxygenation support was used in 163 (4%) patients, with a 34.3% survival rate. Overall mortality in this series was 6.7% (n=271). Risk for mortality was higher in patients with general risk factors, such as prematurity, low birth weight neonates, and heterotaxy syndrome. Mortality for patients with preoperative mechanical ventilation was 17.5%. Pulmonary hypertension and preoperative circulatory shock had 11.6% and 10% mortality rates, respectively. Mortality for patients who had no preoperative risk factor was 3.9%. Neonates had the highest mortality rate (20.5%). Intensive care unit and hospital stay time for neonates (median of 17.8 days and 24.8 days, respectively) were also higher than the other age groups. Infants had 6.2% mortality. Hospital mortality was 2.8% for children and 3.5% for adults. Mortality rate was 2.8% for elective cases. Observed mortality rates were higher than expected in the fourth and fifth categories of the STAT system (observed, 14.8% and 51.9%; expected, 9.9% and 23.1%; respectively). Conclusion: For the first time, outcomes of congenital heart surgery in Türkiye could be compared to the current world experience with this multicenter database study. Increased mortality rate of neonatal and complex heart operations could be delineated as areas that need improvement. The Congenital Heart Surgery Database has great potential for quality improvement of congenital heart surgery in Türkiye. In the long term, participation of more centers in the database may allow more accurate risk adjustment.

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BACKGROUND: Campotodactyly-artrhropathy-coxa vara-pericarditis (CACP) syndrome is a very rare autosomal recessive genetic disorder. It is characterized by flexion contracture of the fifth finger (camptodactyly); noninflammatory arthropathy; decreased angle between the shaft and the head of the femur (coxa vara) and pericarditis. Its association with mitral stenosis has not yet been reported. Hereby we report this unique association with CACP syndrome. CASE: An eleven-year-old girl presented with non-productive cough, dyspnea, and orthopnea. She was diagnosed CACP syndrome at the age of seven and a biallelic frameshift mutation in the PRG4 gene was determined. The physical examination revealed pectus excavatum, camptodactyly, genu valgum, tachypnea and orthopnea. The functional capacity was NYHA III-IV. She had 2/6 soft pansystolic murmur at 4th left intercostal space and a rumbling diastolic murmur at apex. Echocardiography revealed an enlarged left atrium, severe stenotic mitral valve with a mean diastolic transmitral gradient of 22.5 mmHg, mild mitral regurgitation and mild apical pericardial effusion. The patient had mitral comissurotomy and partial pericardiectomy operation. Her post-operative transmitral gradient decreased to 6.9 mmHg and the pulmonary pressure was 30 mmHg. Her functional capacity increased to NYHA I-II. CONCLUSIONS: The main defect is the proteoglycan 4 protein which acts like a lubricant in articular and visceral surfaces. Therefore, the leading clinical feature is arthropathy. Cardiac involvement other than clinically mild pericarditis is not usually expected. Three types of proteoglycans (decorin, biglycan, and versican) are present in the mitral valve. This could be the reason of mitral valve involvement in rare cases as like ours. It is important that these patients undergo echocardiographic examination regularly.

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BACKGROUND AND AIM OF THE STUDY: After congenital cardiac surgery, the duration of mechanical ventilation (MV) is related to the clinical status, type of operation, and the sedative-analgesic agents used postoperatively. This study aims to examine the effects of dexmedetomidine and morphine on the fast-track extubation (FTE) and ultra-fast-track extubation (UFTE) protocol after congenital cardiac surgery. METHODS: A total of 251 pediatric patients were divided into two groups: 118 patients in the morphine group (Group M) and 133 patients in the dexmedetomidine group (Group D). We retrospectively reviewed medical data including hemodynamic parameters, duration of MV and cardiovascular intensive care unit (CICU), additional sedative/analgesic requirement, adverse events, the need for reintubation, and noninvasive MV, sedation, and pain scores. RESULTS: The mean mechanical ventilation duration of Group D was significantly shorter than Group M (3.74 ± 0.83 h in Group D, 5.72 ± 1.54 h in Group M, respectively) (p = .001; p < .05). In Group D, the success rate of FTE was 92.5% (n = 123) and UFTE was 7.5% (n = 10) (p = .001). In Group M, the success rate of FTE was 72.9% (n = 86) and UFTE was 0% (n = 0) (p > .05). CONCLUSIONS: Dexmedetomidine and morphine have clinical benefits which encourage their use for FTE protocol. Dexmedetomidine has more benefits compared to morphine. It can be used in UFTE protocol, besides its use in FTE protocol with fewer side effects.

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BACKGROUND AND AIM OF THE STUDY: Transfusion-associated hyperpotassemia is a serious complication of packed red blood cell (PRBC) transfusion after congenital cardiac surgery. Our study aimed to identify risk factors and potential preventive measures of transfusion-associated hyperpotassemia in neonates and infants after congenital cardiac surgery. METHODS: Pediatric patients who underwent congenital cardiac surgery and need transfusion were enrolled in this prospective study. The potassium concentration of PRBC was checked from the sample taken from the segment. The volume of transfusion, age of PRBC, potassium concentration of unit were recorded. The estimated increment of potassium level in patients after PRBC transfusion was calculated. RESULTS: Seventy-four individual patients, 95 distinct transfusions, 112 blood products were evaluated. The mean age of the blood unit was 3.8 ± 1.4 days. The mean potassium concentration in the PRBCs was 9.9 ± 2.4 mmol/L. A weak correlation was observed between the potassium value of the PRBC and the age of PRBC (p = 0.049, r = 0.2, y = 0.24 × x + -0.68). There was a weak correlation between the potassium value of PRBCs and the age of the unit (p < 0.001, r = 0.37, y = 2.8 × x + -3.6). CONCLUSIONS: Before transfusion, even PRBC is fresh, measuring the potassium level of PRBC and the potassium that will be given to the pediatric patient with transfusion can prevent transfusion-related hyperpotassemia and related complications. Otherwise, high potassium levels, which may be overlooked despite being fresh, may cause serious complications, even cardiac arrest, especially in neonates and infants.

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OBJECTIVES: A new congenital heart surgery database (CKCV) with real-time online reporting function was recently developed in Turkey. All standard international parameters were used, but Aristotle Comprehensive Complexity score was modified. In this study, the first analysis of the CKCV Database is reported. METHODS: The CKCV Database included 2307 procedures from 12 centers between January 2018 and March 2020. All parameters, including 10 real-time online reports, which represent the number of centers, number and mortality rates of all procedures, number of extracorporeal membrane oxygenation (ECMO) and results, details of postoperative complications, age-group statistics, analysis for priority status, mean intensive care and hospital stay durations of the procedures, results of Aristotle Basic, Modified Aristotle Comprehensive (MACC) and Society of Thoracic Surgeons-European Association (STAT) Score Categories, comparison of centers were analyzed. RESULTS: Most common 10 procedures were ventricular septal defect (VSD) repair (n = 273), tetralogy of Fallot (TOF) repair (n = 243), atrial septal defect (ASD) repair (n = 181), complete AVSD repair (n = 95), cavopulmonary anastomosis (n = 81), systemic to pulmonary shunt (n = 79), modified Fontan (n = 71), subaortic resection, (n = 66) PA banding (n = 66), and arterial switch operation (n = 66). Cardiopulmonary bypass was used in 84.6% of the procedures. Overall mortality rate was 6.0%. A total of 618 major and 570 minor complications were observed in 333 and 412 patients, respectively. According to six MACC categories, number of the patients and mortality rates were I (293; 0.3%); II (713; 1.4%); III (601; 3.3%); IV (607; 12%); V (84; 35.7%); and VI (9; 55.6%), respectively. Analysis of five STAT Categories showed 0.7, 3.8, 5.4, 14.9, and 54.7% mortality rates. CONCLUSIONS: CKCV Database has a great potential for nationwide quality improvement studies. Users could instantly analyze and compare their results to national and international aggregate data using a real-time online reporting function. This is the first multicenter congenital database study in Turkey.

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BACKGROUND: Limited data exist regarding the coronary revascularization procedures needed during the repair of several congenital and pediatric cardiac malformations. We aimed to determine risk factors for in-hospital mortality and long-term outcomes of various pediatric coronary revascularization procedures. METHODS: We retrospectively reviewed the records of 32 consecutive pediatric patients who underwent coronary revascularization procedures at our institution between May 1995 and June 2020. In-hospital mortality, risk factors, surgical indications, revascularization patency, and mid- and long-term follow-up data were investigated. Patients were categorized into the coronary artery bypass grafting (n = 11) and other coronary artery procedure (n = 21) groups. RESULTS: The median age and weight of patients at the time of surgery were 9 months and 4.8 kg, respectively. There were five in-hospital deaths (5/32, 15.6%). The mortality rates were 27.2% (3/11) in the coronary artery bypass grafting group and 9.5% (2/21) in the other coronary artery procedure group (p = .206; 95% confidence interval: 0.496-25.563). The mortality rates for planned and rescue procedures were 8.3% (2/24) and 37.5% (3/8) (p = .06), respectively. The median follow-up time was 12.5 years. Control imaging studies for coronary patency were performed in 70.3% (19/27) of surviving patients. The overall coronary patency rate was 94.7% (18/19). CONCLUSIONS: Pediatric coronary revascularization procedures with elective-planned indications can be performed with good outcomes. Young age and rescue and emergency procedures may carry an increased risk of in-hospital mortality, although not found to be statistically significant. Surviving patients require lifelong follow-up regarding the patency of reperfused coronary arteries.

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Background and aim: In patients undergoing congenital cardiac surgery, it is crucial to maintain oxygen demand-consumption balance. Central venous oxygen saturation (ScvO2) is a useful indicator of oxygen demand and consumption balance which is an invasive method. Near-infrared spectroscopy (NIRS) is a noninvasive, continuous monitoring technique that measures regional tissue oxygenation. NIRS that is placed over the internal jugular vein cutaneous area (NIRSijv) has the potential to show ScvO2 indirectly. In this study, we aimed to determine the correlation between ScvO2 with NIRSijv in pediatric patients undergoing congenital cardiac surgery. Materials and methods: Fifty children participated in the study. Four patients were excluded for the inability of internal jugular vein (IJV) catheterization due to technical difficulties. After anesthesia induction, NIRS probes were placed on the IJV site with ultrasound guidance for the measurement of continuous transcutaneous oxygen saturation. The catheter insertion was also done through the IJV from the other side using ultrasound guidance. Cerebral oxygenation monitoring was done using NIRS with a single pediatric probe placed on the right forehead. Values of NIRSijv, cerebral NIRS (NIRSc) and ScvO2, were recorded at certain times until postoperative 24th hour. Results: Data were collected at 8 different time points. There was a significant correlation between ScvO2 and NIRSijv in all measurement time points (r = 0.91), (P = 0.001). The mean bias between ScvO2 and NIRSijv was 2.92% and the limits of agreement were from 11% to ­5.2%. There was a moderate correlation between ScvO2 and NIRSc (r = 0.45), (P = 0.001). The mean bias between ScvO2 and NIRSc was 2.7% and the limits of agreement were from +26% to ­20%. Conclusions: In this study, we found a strong correlation between ScvO2 and NIRS measurements taken from the internal jugular vein site. Accordingly, continuous noninvasive monitoring with transcutaneous NIRSijv can be an alternative method as a trend monitor for the central venous oxygen saturation in pediatric cardiac patients undergoing congenital cardiac surgery.

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Left ventricular pseudoaneurysm (LVPA) is a rare but lethal complication of mitral valve replacement (MVR) or myocardial infarction. Early correction is necessary for patients with extensive and expanding LVPA. We report a transcatheter closure of LVPA after MVR. A 63-year-old woman presented with an LVPA 2 months after MVR. The repeated computed tomographic scan and transthoracic echocardiography showed enhancement of pseudoaneurysm. The LVPA was closed successfully with Amplatzer Vascular Plug using a transcatheter approach.

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INTRODUCTION: Compromise of tissue oxygenation during surgery is associated with increased mortality and morbidity in the postoperative period in patients with congenital cardiac disorders. It may be monitored with near-infrared spectroscopy (NIRS). We aimed to evaluate the tissue oxygenation and factors which may affect it by bilateral cerebral and somatic NIRS levels during cardiopulmonary bypass and to compare the NIRS values of cyanotic and acyanotic patient groups. MATERIAL AND METHODS: Two groups of patients with cyanotic and acyanotic congenital heart diseases were included in the study. Each group consisted of 15 patients between 0 and 5 years of age. All data were collected following anesthesia induction (T1), the 10th (T2) and 30th min (T3) of cardiopulmonary bypass (CPB), every 30 min during CPB (T4, T5, T6) and 1 h after (TS). Bilateral and somatic NIRS, blood gases, mean arterial pressure, and temperatures were recorded. RESULTS: Left and right somatic NIRS values in groups at all measurements did not differ significantly. Left and right cerebral NIRS values at T2 and T3 in cyanotic patients were significantly higher than in acyanotic patients. Mean arterial pressure and lactate levels at T1 and T3 measurements were responsible for left cerebral NIRS changes and mean arterial pressure on right cerebral NIRS values. CONCLUSIONS: Monitorization of tissue perfusion has critical importance during CPB of patients with congenital heart defects. Oxygenation may easily and reliably be measured with NIRS. Cerebral and somatic NIRS are more pronounced in cyanotic patients and cerebral NIRS is strongly associated with mean arterial pressure and circulating lactate levels.

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BACKGROUND: In this study, we evaluated the efficacy of intercostal nerve block for postoperative pain management in pediatric patients undergoing atrial septal defect closure through a right lateral mini-thoracotomy. METHODS: Between January 2016 and January 2019, a total of 63 pediatric patients (37 males, 26 females; mean age 34.8±26.8 months; range, 2 to 96 months) who underwent corrective congenital heart surgery for atrial septal defect closure through a right lateral mini-thoracotomy were retrospectively reviewed. The patients were divided into two groups as those (Group 1, n=33) receiving intercostal nerve block and general anesthesia and those (Group 2, n=30) receiving general anesthesia alone. Intravenous morphine at a dose of 0.03 mg/kg was applied as rescue analgesia to the patients with a Ramsay Sedation Scale score of >4 and Children"s Hospital of Eastern Ontario Pain Scale score of >7. The total analgesic requirement, adverse effects, duration of mechanical ventilation and length of stay in the intensive care unit were recorded. RESULTS: The mean duration of mechanical ventilation and intensive care unit stay was shorter in Group 1 compared to Group 2 (3.6±1.3 vs. 9.4±2.1 h; 23±2.6 vs. 30±7.2 h, respectively) (p<0.0001). The need for postoperative rescue analgesia was statistically significantly lower in Group 1 compared to Group 2 (0.3±0.5 mg vs. 1.1±0.9 mg, respectively) (p=0.003). The mean total morphine consumption was also lower in Group 1 compared to Group 2 (4.0±2.2 mg vs. 9.0±3.4 mg, respectively) (p<0.0001). CONCLUSION: Intercostal nerve block before thoracotomy closure in pediatric patients undergoing atrial septal defect repair under mini-thoracotomy provides early extubation, shorter mechanical ventilation duration and intensive care unit stay, and reduced analgesic requirements.

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In international experience, performance improvement, patient safety, and quality assurance are mainly based on database studies in the field of pediatric and congenital cardiac surgery. Data collection and analysis of the databases allow the clinician to identify the complexity, to predict possible risks and complications, and to appropriately evaluate the outcomes and performances. Recently, the Children"s Heart Foundation Working Group has developed a database project in Turkey based on the parameters and methodologies of the international databases, namely the Pediatric Heart Surgery National Database. Currently, it is a must for us to put this project into practice to reach the global standards in the pediatric and congenital heart surgery in our country. We believe that all children with congenital heart disease should have an opportunity to benefit from the most recent medical and surgical treatment modalities with the most favorable results.

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Background/aim: Although pulmonary valve replacement (PVR) improves ventricular function and symptoms, the benefit and optimal timing of PVR are controversial. This study aimed to evaluate early response to PVR for right ventricle (RV) dilatation and QRS duration. Materials and methods: Retrospective analysis was performed for 32 patients with repaired tetralogy of Fallot (TOF) between March 2005 and October 2017. The differences between preoperative and postoperative changes in echocardiographic parameters, clinical symptoms, and QRS duration were evaluated. Results: There were no in-hospital or late deaths. Mean age at the time of PVR was 16.57 ± 7.97 years. The interval between TOF repair and PVR was 12.99 ± 7.06 years. Postoperative echocardiographic findings showed significant reduction in indexed RV end-diastolic diameter (RV-EDDI) and the ratio of RV/LV-EDDI (P = 0.001 and P = 0.001, respectively). Higher preoperative RV-EDDI was associated with decreased change in RV-EDDI after PVR (r = 0.63; P = 0.001). Normalization of RV diameters was found to be independent of age at PVR, interval between TOF repair and PVR, preoperative QRS duration, and preoperative RV-EDDI. Conclusion: Significant improvement in RV diameter and symptoms could be obtained with PVR in patients with severe pulmonary regurgitation.

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OBJECTIVE: This study aimed to identify the best graft-to-pulmonary artery (PA) anastomosis angle measuring pulmonary blood flow, wall shear stress (WSS), and shunt flow. METHODS: A tetralogy of Fallot with pulmonary atresia computer model was used to study three different modified Blalock-Taussig shunt (mBTS) anastomosis angle configurations with three different PA diameter configurations. Velocity and WSS were analyzed, and the flow rates at the right PA (RPA) and left PA (LPA) were calculated. RESULTS: A 4-mm and 8-mm diameter of RPA and LPA, respectively with vertical shunt angle produces the highest total flow. In the RPA larger diameter than the LPA configutations, the left-leaning shunt produces the lowest total PA flow whereas in the LPA larger diameter than the RPA configuratios, the right-leaning shunt produces the lowest total PA flow. Therefore, the shunt anastomosis should not be leaned through the narrow side of PA to reach best flow. As the flow inside the shunt increased, WSS also increased due to enhanced velocity gradients. CONCLUSION: The anastomosis angle between the conduit and PA affects the flow to PA. Vertical anastomosis configurations increase the total PA flow; thus, these configurations are preferable than the leaned configurations.

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BACKGROUND: We present our clinical experience with coronary artery bypass grafting (CABG) in children. METHODS: Ten children who underwent CABG between July 1995 and August 2017 were retrospectively analyzed. Data including congenital cardiac malformations, previous surgical procedures, age and sex, type of coronary complications, ischemic events preceding surgery, and ventricular function before and after CABG were recorded. RESULTS: The study population consisted of five males and five females with a median age of 2.5 years (range, 88 days to 15 years). Eight internal mammary arteries (IMAs) and two saphenous veins were used for grafting. Indications for bypass grafting were coronary artery (CA) complications related to the post-arterial switch operation in six, CA complications during the Ross procedure in two, and an iatrogenic CA injury during complete repair of tetralogy of Fallot with abnormal CA, crossing the right ventricular outflow tract in two patients. Six of the grafts were performed as rescue procedures. Three patients died during hospitalization. The mean follow-up time was 6.8 years (range, 3 months to 18 years). Anastomoses were evaluated by coronary angiography in four patients, and were all patent. Echocardiography revealed normal myocardial function in all patients. CONCLUSION: Our study suggests that the IMA should be the graft of choice in children due to its growth potential and long-term patency.

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Left atrial aneurysm is an extremely rare anomaly, which can be associated with supraventricular arrhythmia, compression of coronary arteries, intracardiac thrombus, life-threatening systemic embolization, pulmonary venous obstruction, mitral valve insufficiency, and congestive heart failure. Herein, we report a four-year-old boy who had a giant aneurysm of the left atrium and severe mitral regurgitation. The aneurysm and mitral valve cleft causing severe mitral regurgitation were successfully repaired.

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Transcatheter device implantation has become an attractive alternative to surgery in the closure of atrial septal defects in selected patients. However, it can lead to early and late sequelae, some of them life threatening. For example, 79 days before her admission to our emergency department with sudden-onset respiratory distress and respiratory arrest (leading to cardiac tamponade and rupture), a 22-year-old woman had undergone percutaneous closure of an atrial septal defect. We describe the damage and its treatment. Although the adverse effects of transcatheter device implantation are rare, physicians should know that these events can be life threatening. Further data are needed to prevent such sequelae and to design new devices. It is of utmost importance that patients and their family members be informed both of possible sequelae and of life-saving interventions to be administered at early diagnosis.

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One of the options for the management of borderline/failing extracardiac Fontan circulation is surgical creation of an atrial fenestration to decompress the systemic venous compartment and improve cardiac output. Depending on the body surface area of the patient, a 5- to 10-mm polytetrafluoroethylene (PTFE) tube graft can be used. When fenestration is required in a patient with failing Fontan circulation, particularly in redo cases, application of a side-biting clamp may be challenging because of adhesions and a thickened atrial wall. In this article, we present our off-pump technique of atrial-side anastomosis of PTFE graft interposition between an extracardiac Fontan conduit and the atrium without using a side-biting clamp.

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