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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.
RESUMEN
Abstract Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.