{"title":"特纳综合征患者急性B型主动脉夹层和主动脉根部动脉瘤的两阶段手术:1例报告。","authors":"Kazunori Sakaguchi, Hidekazu Nakai, Takanori Tsujimoto, Atsunori Kono, Yojiro Koda, Katsuhiro Yamanaka, Kenji Okada","doi":"10.1186/s44215-025-00202-9","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Case presentation: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":520286,"journal":{"name":"General Thoracic and Cardiovascular Surgery Cases","volume":"4 1","pages":"23"},"PeriodicalIF":0.0000,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12046644/pdf/","citationCount":"0","resultStr":"{\"title\":\"Two-stage surgery for acute type B aortic dissection and aortic root aneurysm in a patient with Turner syndrome: a case report.\",\"authors\":\"Kazunori Sakaguchi, Hidekazu Nakai, Takanori Tsujimoto, Atsunori Kono, Yojiro Koda, Katsuhiro Yamanaka, Kenji Okada\",\"doi\":\"10.1186/s44215-025-00202-9\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>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.</p><p><strong>Case presentation: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>\",\"PeriodicalId\":520286,\"journal\":{\"name\":\"General Thoracic and Cardiovascular Surgery Cases\",\"volume\":\"4 1\",\"pages\":\"23\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-05-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12046644/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"General Thoracic and Cardiovascular Surgery Cases\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1186/s44215-025-00202-9\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"General Thoracic and Cardiovascular Surgery Cases","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1186/s44215-025-00202-9","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Two-stage surgery for acute type B aortic dissection and aortic root aneurysm in a patient with Turner syndrome: a case report.
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.