Two-stage surgery for acute type B aortic dissection and aortic root aneurysm in a patient with Turner syndrome: a case report.

Abstract

Background: Patients with Turner syndrome are at high risk of aortic dissection and are more likely to present with fatal outcomes. Turner syndrome is classified as a heritable thoracic aortic disease in the latest EACTS/STS guidelines. Herein, we present a case of two-staged surgery for acute type B aortic dissection and aortic root aneurysm in a patient with Turner syndrome.

Case presentation: A 29-year-old female patient with Turner syndrome was admitted to our center due to back pain and was diagnosed with acute type B aortic dissection. Echocardiography revealed a dilated aortic root with bicuspid aortic valve. On the 5 th day after admission, the patient presented with a higher level of back pain. Follow-up computed tomography scan revealed changes from two- to three-channel aortic dissection a further aortic dilation. Therefore, descending aorta and partial aortic arch replacement were initially performed. Then, valve-sparing aortic root replacement and residual arch replacement were performed 3 months after the first surgery. Postoperative echocardiography confirmed the absence of aortic regurgitation. The patient was discharged on the 17th postoperative day without any complications.

Conclusions: Two-stage surgery was successfully performed for the thoracic aorta and aortic root aneurysms in a patient with Turner syndrome. The patient recovered for 3 months after the left thoracotomy surgery and underwent a two-stage surgery through a median sternotomy surgery with good surgical results.