Neonatal Outcomes of Critical Congenital Heart Defects: A Multicenter Epidemiological Study of Turkish Neonatal Society : Neonatal Outcomes of CCHD.

Critical congenital heart disease (CCHD) is one of the leading causes of neonatal and infant mortality. We aimed to elucidate the epidemiology, spectrum, and outcome of neonatal CCHD in Türkiye. This was a multicenter epidemiological study of neonates with CCHD conducted from October 2021 to November 2022 at national tertiary health centers. Data from 488 neonatal CCHD patients from nine centers were entered into the Trials-Network online registry system during the study period. Transposition of great arteria was the most common neonatal CHD, accounting for 19.5% of all cases. Sixty-three (12.9%) patients had extra-cardiac congenital anomalies. A total of 325 patients underwent cardiac surgery. Aortic arch repair (29.5%), arterial switch (25.5%), and modified Blalock-Taussig shunt (13.2%). Overall, in-hospital mortality was 20.1% with postoperative mortality of 19.6%. Multivariate analysis showed that the need of prostaglandin E1 before intervention, higher VIS (> 17.5), the presence of major postoperative complications, and the need for early postoperative extracorporeal membrane oxygenation were the main risk factors for mortality. The mortality rate of CCHD in our country remains high, although it varies by health center. Further research needs to be conducted to determine long-term outcomes for this vulnerable population.

Non-Conduit Repair of Truncus Arteriosus.

INTRODUCTION: The procedure of choice for treatment of truncus arteriosus is one-stage repair within the first few months of life. Establishing right ventricle-pulmonary artery direct continuity without conduit can be a good alternative in the absence of valved conduits in developing centers. METHODS: Between January 2021 and June 2021, a total of five patients (three males, two females) underwent definitive repair of truncus arteriosus without an extracardiac conduit. We used the Barbero-Marcial technique to allow age-related growth, eliminate the risk of conduit-related complications, and to avoid forcing a conduit to place in a very small mediastinal space. RESULTS: The patients' mean age was 31.2 days (11-54 days). Their mean bodyweight was 3.2 kg (2.7-3.8kg). Mean postoperative intensive care unit stay was 39.6 days (7-99 days). There were two mortalities in the intensive care unit on postoperative days 12 and 61 due to lung-related problems. The remaining three cases' mean ventilation time was 15.6 days (8-22 days). CONCLUSION: Having access to a valved conduit is still challenging for some centers, and the non-conduit repair technique defined by Barbero-Marcial can be a successful, life-saving alternative easy for young surgeons to perform in newly based centers.

Non-Conduit Repair of Truncus Arteriosus

ABSTRACT Introduction: The procedure of choice for treatment of truncus arteriosus is one-stage repair within the first few months of life. Establishing right ventricle-pulmonary artery direct continuity without conduit can be a good alternative in the absence of valved conduits in developing centers. Methods: Between January 2021 and June 2021, a total of five patients (three males, two females) underwent definitive repair of truncus arteriosus without an extracardiac conduit. We used the Barbero-Marcial technique to allow age-related growth, eliminate the risk of conduit-related complications, and to avoid forcing a conduit to place in a very small mediastinal space. Results: The patients' mean age was 31.2 days (11-54 days). Their mean bodyweight was 3.2 kg (2.7-3.8kg). Mean postoperative intensive care unit stay was 39.6 days (7-99 days). There were two mortalities in the intensive care unit on postoperative days 12 and 61 due to lung-related problems. The remaining three cases' mean ventilation time was 15.6 days (8-22 days). Conclusion: Having access to a valved conduit is still challenging for some centers, and the non-conduit repair technique defined by Barbero-Marcial can be a successful, life-saving alternative easy for young surgeons to perform in newly based centers.

A Better Way for Ductal Stenting in Patients with Duct-Dependent Pulmonary Flow and Vertical and Tortuous Patent Ductus Arteriosus.

BACKGROUND: In this study, we aimed to compare the femoral route and the carotid artery route in terms of procedural success of ductal stent implantation in patients with ductdependent pulmonary blood flow. METHODS: The study included 51 patients with duct-dependent pulmonary circulation who underwent ductal stent implantation upon their admission to our clinic between July 2017 and March 2021. In total, 23 patients (group I) underwent ductal stent implantation via the femoral route, while the remaining 28 (group II) underwent the procedure via the carotid artery. The groups were compared in terms of procedural success, time, post-procedural blood pH, lactate levels, and complications. RESULTS: Duct morphology was observed in group 1 as follows: type 1 in 12 patients, type 3 in 8, type 2 in 2, and type 6 in 1 patient. In group 2, 26 patients had type 3, 1 had type 2, and 1 had type 6. The tortuosity index of the patients in group 1 was 1 in 8 patients, 2 in 8 patients, and 3 in 7 patients, while in group 2, it was 1 in 5 patients, 2 in 15 patients, and 3 in 8 patients. The success rate was 69.6% (16/26) in group I and 93.5% (29/31) in group II (P=.030). The cumulative success rate was 88.2% (45/51). The procedural durations were 78.2 ± 34.1 and 52.1 ± 22.0 minutes in group I and group II, respectively (P=.002). The mean blood pH values upon the completion of the procedure were 7.26 ± 0.1 and 7.33 ± 0.0 in group I and group II, respectively (P=.038). The mean post-procedural lactate levels were 2.8 mmol/L and 2.3 mmol/L in group I and group II, respectively (P=.038). The 2 groups did not show any differences in terms of procedural complications. CONCLUSION: The carotid artery route can be preferred, especially in vertical and tortuous ductus arteriosus, as it is associated with a high success rate and a short procedural time, as well as a better metabolic condition after the procedure.

Ligation of patent ductus arteriosus through left anterior mini-thoracotomy in preterm infants.

OBJECTIVE: Patent ductus arteriosus is an important cause of morbidity and mortality, especially in very low birth weight infants. The aim of the study is to report our single-centre short-term results of preterm patients who underwent ligation through left anterior mini-thoracotomy . METHODS: Data of 27 preterm infants operated by the same surgeon who underwent Patent ductus arteriosus (PDA) closure with left anterior mini-thoracotomy technique between November 2020 and January 2022 at a single institution were reviewed. The patients were divided into two groups according to their weight at the time of surgery. Data on early postoperative outcomes and survival rates after discharge were collected. RESULTS: Twenty-seven patients with a mean (±SD) gestational age of 25.8 (±2.0) weeks and a mean birth weight of 1027 (±423) g were operated using left anterior mini-thoracotomy technique. The lowest body weight was 480 g. Complications such as bleeding, abnormal healing of incision, or pneumothorax were not seen. There were 8 mortalities after the operation (29,6 %). The causes of the deaths were sepsis, necrotising enterocolitis, hydrops fetalis, hepatoblastoma, and intracranial bleeding. There was no statistically significant difference in the rates of complication between the groups. CONCLUSIONS: Left anterior mini-thoracotomy technique can be performed as the first choice when transcatheter intervention cannot be applied in preterm infants. It provides easy access to the PDA, a good exposure, minimal contact with the lungs, good cosmetic results in early and mid-term and shortens the operation time, especially in very low birth weight preterm babies. However, early ligation may be helpful to minimise the complications related to PDA.

Is arterial switch operation possible with neonatology-focused intensive care unit modality.

BACKGROUND: In this study, we aimed to examine the feasibility of arterial switch operation and its perioperative management with neonatology-focused intensive care modality in a region of Turkey where the birth rate and the number of asylum seekers who had to leave their country due to regional conflicts are high. METHODS: Between December 2017 and June 2020, a total of 57 patients (48 males, 9 females; median age: 12.2 days; range, 2 to 50 days) who were diagnosed with transposition of the great arteries in our clinic and underwent arterial switch operation were retrospectively analyzed. All patients were followed by the neonatologist in the neonatal intensive care unit during the preoperative and postoperative period. RESULTS: Thirty-eight (66.7%) patients had intact ventricular septum, 16 (28.1%) had ventricular septal defect, two (3.5%) had coarctation of the aorta, and one (1.7%) had Taussig-Bing anomaly. Coronary artery anomaly was present in 14 (24.5%) patients. The most common complications in the intensive care unit were renal failure requiring peritoneal dialysis in seven (12.3%) patients, supraventricular tachyarrhythmia in six (10.5%) patients, and eight (14%) patients left their chests open. The median length of stay in intensive care unit was 13.8 (range, 9 to 25) days and the median length of hospital stay was 24.5 (range, 16 to 47) days. The overall mortality rate for all patients was 12.3% (n=7). The median follow-up was 8.2 months. A pulmonary valve peak Doppler gradient of ≥36 mmHg was detected in five patients (8.7%) who were followed, and these patients were monitored by providing medical treatment. None of the patients needed reoperation or reintervention. CONCLUSION: We believe that arterial switch operation, one of the complex neonatal cardiac surgery, can be performed with an acceptable mortality and morbidity rate with the use of neonatology-focused intensive care modality, which is supported by pediatric cardiology and pediatric cardiac surgery.

Aplastic Anemia as an Immune-mediated Complication of Thymoma: A Case Report.

Thymomas are the most common masses located in the anterior mediastinum, and they are often associated with autoimmune disorders including myasthenia gravis, polymyositis, and aplastic anemia (AA). Autoreactive T-cell clones generated by the thymoma may lead to autoimmune disorders. We report the case of a 14-year-old boy who was examined for AA, and the underlying cause was determined to be an immune-mediated complication of thymoma. He had no matched sibling donors. He underwent thymectomy, and 3 months later he was treated with immunosuppressive therapy (IST), consisting of antithymocyte globulin and cyclosporine A. The duration of the IST was determined to be a period of 12 months. He has recently been in complete response condition for 6 months since IST stopped. IST is a successful treatment choice for thymomas associated with AA in childhood.

A Complication following the Transcatheter Closure of a Muscular Ventricular Septal Defect.

Today, congenital heart diseases may be treated without surgery through advances in interventional cardiology. However, complications such as infection and thrombus formation may develop due to foreign materials used during these procedures. Surgical intervention may be required for the removal of the device utilized for the procedure. In this case report, we present the surgical treatment of a residual ventricular septal defect (VSD) that had developed in a 6-year-old patient with an apical muscular VSD closed with the Amplatzer muscular VSD device. The patient was admitted to the emergency room with complaints of abdominal pain and high fever 5 days after discharge without any cardiac symptoms. When she arrived at our clinic, she had a heart rate of 95 bpm, blood pressure of 110/70 mmHg, and temperature of 38.5ºC. Examinations of the other systems were normal, except for a 3/6 pan-systolic murmur at the mesocardiac focus on cardiac auscultation. Echocardiography showed a residual VSD, and the total pulmonary blood flow to the total systemic blood flow ratio (Qp/Qs) of the residual VSD was 1.8. In the operating room, the Amplatzer device was removed easily with a blunt dissection. The VSD was closed with an autologous fresh pericardial patch. Following the pulmonary artery debanding procedure, the postoperative period was uneventful. The condition of the patient at the time of discharge and in the first postoperative month's follow-up was good. There was no residual VSD or infection.

Right juxtaposition and a tunnel between the atrial appendages in a patient with atrial septal defect and pulmonary valve stenosis.

A 13-month-old boy with a diagnosis of atrial septal defect and pulmonary valve stenosis was admitted for corrective surgery. Right juxtaposition of the atrial appendages with a tunnel between the atrial appendages was detected during the surgery. The patient was operated successfully and had an uneventful recovery. Once right juxtaposition of the atrial appendages has been identified, the possibility of a "tunnel" communication between the appendages must be considered and ruled out.

Inter-aorta-caval located tumor: a case report.

A paraganglioma is a rare tumor that develops out of extra-adrenal chromaffin cells and pheochromocytomas originating from the adrenal medulla. Early diagnosis and surgical planning are crucial, since the tumor secretes catecholamine and is adjacent to large vessels in the abdomen. Furthermore, since complete resection improves the prognosis, we recommend a meticulous surgical technique. Here, we present a case of paraganglioma in a 32-year-old male patient who initially presented with a stomachache. After conducting the required tests, we resected the tumor that was pressing against the vena cava in the interaortocaval region.

Successful combination of interventional and surgical treatment of left atrium perforation.

Percutaneous interventions are increasingly used in the treatment of cardiac diseases which are resistant to medical treatment. However, the complications caused by these interventions can lead to serious results. In this article, we present a case of a successful combination of interventional and surgical treatment methods, following the development of left atrial perforation during radiofrequency catheter ablation (RFA), in a patient with atrial fibrillation resistant to medical treatment.