Objective To analyze the preoperative diagnosis of aberrant subclavian artery (ASA) by echocardiography and CTA, and to evaluate the postoperative follow-up results. Methods Data of 192 childrens with ASA confirmed by cardiothoracic surgery were retrospectively analyzed. Results In 192 childrens, 189 cases were right aortic arch with aberrant left subclavian artery (RAA-ALCA). 3 cases were left aortic arch with aberrant right subclavian artery (LAA-ARLA). 132 cases combine with other common cardiac structural malformations. The common malformations included 96 cases of Kommerell diverticulum, 89 cases of atrial septal defect/patent foramen ovale, 51 cases of ventricular septal defect,20 cases of left superior vena cava remaining, 17 cases of patent ductus arteriosus. In 192 cases, echocardiography accurately diagnosed ASA in 124 cases (64.6%); 68 cases (35.4%) were misdiagnosed. A total of 174 cases (90.6%) were accurately diagnosed by CTA, and 18 cases (9.4%) were missed and misdiagnosed. In 132 cases complicated with other cardiac structural malformations, Echocardiography missed diagnosis of patent foramen ovale in 2 cases, 47 cases of atrial septal defect/patent foramen ovale and small ventricular septal defect were missed by CTA diagnosis. In the follow-up of 96 children who underwent vagal left subclavian artery transplantation or Kommerell diverticulectomy, 81 children who underwent vagal subclavian artery transplantation and Kommerell diverticulectomy had no obvious anastomotic obstruction, and 14 patients underwent CTA. Evaluated tracheal compression was less than preoperative.Conclusions Echocardiography and CTA in the preoperative diagnosis of ASA each have advantages and disadvantages, can respectively in the process of postoperative follow-up evaluation anastomotic and airway pressure situation, therefore, the joint multimodal inspections on echocardiography and CTA, can be more accurate diagnosis of ASA and merge heart abnormalities, as well as a more comprehensive evaluation of postoperative recovery effect.