Abstract
Background: Progressive dilation of the ascending aorta is prone to lead to aortic dissection in patients with pulmonary artery atresia and ventricular septal defect. However, reports of long-term complications such as aortic aneurysm or aortic dissection in adults with pulmonary artery atresia are relatively rare.
Case presentation: Case 1: A 38-year-old male patient diagnosed with "pulmonary artery atresia, ventricular septal defect, bicuspid aortic valve deformity" underwent ventricular septal defect repair, aortic valve replacement, and correction of pulmonary artery atresia at the age of 21. Seventeen years postoperatively, he developed aortic dissection and underwent Bentall surgery.
Case 2: A 33-year-old male patient diagnosed with "pulmonary artery atresia, ventricular septal defect" underwent Blalock-Taussig shunt surgery at the age of 17. Sixteen years postoperatively, he developed aortic root aneurysm and ascending aortic dilation.
Case 3: A 42-year-old female patient diagnosed with "pulmonary artery atresia, ventricular septal defect" underwent pulmonary artery atresia corrective surgery and ventricular septal defect repair at the age of 14. Twenty-eight years postoperatively, she developed aortic root aneurysm and ascending aortic dilation.
Conclusions: Following corrective surgery for pulmonary artery atresia with ventricular septal defect, long-term follow-up monitoring is necessary. Progressive dilation of the aorta should be promptly intervened to prevent the occurrence of aortic dissection.