Current Nationwide Landscape of Cardiac Surgery for Children With Trisomy 18 in Japan

Despite the increasing number of retrospective cohorts describing successful cardiac surgery for children with trisomy 18, no consensus has been reached among healthcare providers regarding cardiac surgery in Japan. This study provided a benchmark to facilitate consensus building by assessing a nationwide surgical database in Japan. A total of 78,179 cardiothoracic surgeries performed for children with congenital heart disease were registered in the Japan Cardiovascular Surgery Database Congenital Section from 2013 to 2021. Of these, 789 (1.0%) surgeries were performed for 502 children with trisomy 18 with increasing yearly trends. Of these, 490 cases (96.1%) of mild-to-moderate cardiac lesions underwent simple surgery, whereas 600 closed-heart surgeries accounted for 76.0%. Approximately 19.4% of the cases were associated with noncardiac lesions that led to postoperative complications at a frequency of 35.3%, whereas cardiac-related postoperative complications accounted for 30.5%. Severe infections occurred postoperatively in approximately 9% of the cases. Forty-two (8.4%) patients died in-hospital, of which the most common cause was heart-related, followed by respiratory-related and infection. This nationwide database analysis may provide valuable information that may serve as a benchmark and enable us to better define the patient attributes that will maximize the effectiveness of cardiac surgery for the vulnerable children.