Prognosis Analysis of Children with Interrupted Aortic Arch Complicated with Ventricular Septal Defect and Other Associated Intracardiac Defects after One-Stage Radical Surgery.

BACKGROUND: High rates of mortality and aortic arch stenosis have been reported for one-stage radical surgery of interruption of aortic arch (IAA) with ventricular septal defect (VSD) and other associated intracardiac defects, but the sample size of the study is relatively small, and the credibility of the study is not high. The risk factors of death and aortic arch stenosis will be analyzed in a large sample size of infants with IAA, VSD and other associated intracardiac defects after one-stage radical resection.

METHODS: A retrospective analysis was performed on 152 children with IAA, VSD and other associated intracardiac defects from January 2006 to January 2017 who had undergone one-stage radical resection, including 95 cases of type A and 57 cases of type B. January 2006-December 2011 as the early period, and January 2012-January 2017 as the late period. Cox proportional hazards regression model was used to analyze the risk factors for mortality and aortic arch stenosis after surgery, the overall survival rate was analyzed by the Kaplan-Meier method, and the survival curve was drawn by GraphPad Prism 8 software.

RESULTS: 22 cases (14.47%) died, 27 cases (17.76%) developed aortic arch stenosis. The 1-month, 3-month, 6-month, 1-year, 3-year, and 5-year survival rates were 85.53%, 85.53%, 85.53%, 84.21%, 78.95% and 75.66%, respectively. Low age (Hazard Ratio (HR) = 0.551, 95% Confidence Interval (CI): 0.320-0.984, p = 0.004), low body weight (HR = 0.632, 95% CI: 0.313-0.966, p = 0.003), large ratio of VSD diameter/aortic diameter (VSD/AO) (HR = 2.547, 95% CI: 1.095-7.517, p = 0.044), long duration of cardiopulmonary bypass (HR = 1.374, 95% CI: 1.000-3.227, p = 0.038), and left ventricular outflow tract obstruction (LVOTO) (HR = 3.959, 95% CI: 1.123-9.268, p = 0.015) were independent risk factors for postoperative death. The surgical period (January 2006-December 2011) (HR = 0.439, 95% CI: 0.109-0.964, p = 0.046) and the addition of pericardial anastomosis to the anterior aortic wall (HR = 0.398, 95% CI: 0.182-0.870, p = 0.021) were independent risk factors for postoperative aortic arch stenosis.

CONCLUSIONS: Children with low age, low body weight, large ratio of VSD/AO, long duration of cardiopulmonary bypass, LVOTO, the surgical period (January 2006-December 2011) and pericardial anastomosis with anterior aortic wall have poor prognosis.