Kết quả phẫu thuật sửa chữa bệnh gián đoạn quai động mạch chủ kèm thông liên thất tại Bệnh viện Nhi Trung ương

Interrupted aortic arch (IAA) is a complex congenital heart disease that presents early in the neonatal period with high overall mortality and reoperation rate for the aortic arch and LVOT. Our cohort study aimed to evaluate the results and risk factors of death, LVOT reoperation, and arch reintervention after the biventricular repair of the interrupted aortic arch with ventricular septum defect. All 68 patients in our study underwent surgery to repair the aortic arch. The median age at surgery was 23 days old (interquartile range-IQR:14 days- 42.5 days), and males accounted for 66.2%. The median weight at surgery was 3.3kg (IQR: 3.0-3.6kg). In our study, 57 patients underwent primary surgery, 7 underwent LVOT bypass surgery, and 4 underwent aortic arch repair with banding of the pulmonary artery. The mean follow-up time of the study group was 35.5±29.5 days, Early death after surgery had 10 patients (14.7%) and 01 patient died late. The overall actuarial survival after surgery at hospital discharge, 1 year, 3 years, 5 years, and 10 years were 92.6 %, 84.5%, 82.9%, and 82.9%, respectively. Risk factors for time-related death include preoperative culture blood positive (p= 0.002), prolonged postoperative mechanical ventilation (p<0.001), and IAA type B with remote DORV (p=0.002). 9 patients had resected the fibrous/muscle of the subaortic region and 2 patients were catheter-based dilated for arch restenosis. During the follow-up period, the proportion of patients who did not need to reoperate resulting from LVOT obstruction at the time points of 1 year, 5 years, and 10 years, respectively was 97,6%, 81,7%, 73%, and 73%. The result of biventricular repair of the IAA with VSD in newborns and infants in the National Hospital of Pediatrics is satisfactory. Patients with IAA will require ongoing surveillance with cardiology.