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The article presents a case study of two-and-a-half-female patient with cardiac arrest (CA) during an operation for inguinal hernia (IH) and syndactyly. Topics discussed include diagnosis of Timothy syndrome (TS) by electrocardiography (ECG)-Holter findings, implantation of an epicardial dual-chamber implantable cardioverter defibrillator (EDCICD), and starting the treatment with beta-blocker propranolol.

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., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2024, Turkish Society of Cardiovascular Surgery.)

Background: This study aims to investigate the role of ultrasonography in the postoperative evaluation of diaphragm function in patients with congenital heart defect., Methods: This prospective study included a total of 360 patients (176 males, 184 females; mean age 2 years; range, 1 month to 8 years) who underwent congenital heart surgery and 44 patients (22 males, 22 females; mean age 1 years; range, 1 month to 4 years) who underwent diaphragm ultrasonography between September 2018 and March 2019. Ultrasonography was performed for the patients who had difficulty in weaning from mechanical ventilation or who were thought to have diaphragm dysfunction due to pathological findings on postoperative chest X-rays. The findings were interpreted as normal, paresis, or paralysis., Results: Diaphragm dysfunction was demonstrated in 23 patients (6.3%), paralysis in 11 patients (3%), and paresis in 12 patients (3.3%). A median sternotomy was performed in 21 patients (91%), and seven of them (30%) were redo cases. Five patients (21%) had single ventricle physiology. Six patients (1.6%) needed an intervention due to diaphragm dysfunction. The interventional procedures were diaphragm plication in three patients (0.8%) and tracheotomy in three patients (0.8%). Three of these patients had a single ventricle and three had biventricular physiology. The median time after surgery for these procedures was 36 days. One patient (0.2%) died in the intensive care unit. The mean length of stay in the intensive care unit and hospital was 36±12 and 48±21 days, respectively., Conclusion: Diaphragm dysfunction should be kept in mind in patients undergoing congenital heart surgery and in those who need prolonged intubation during the postoperative period. Ultrasonography is a non-invasive diagnostic tool which can be used to identify diaphragm dysfunction and the best course of management of this clinical condition., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2020, Turkish Society of Cardiovascular Surgery.)