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Introduction <p>Turner syndrome is the most common established cause of aortic dissection in young women. These patients usually have associated Stanford type A aortic dissection. We present herein a case of coarctation of the aorta complicated by acute type B aortic dissection in a patient with Turner syndrome—an exceptionally rare pathological combination.</p> Case presentation <p>A 40-year-old woman with Turner syndrome experienced sudden back pain. Enhanced computed tomography revealed coarctation of the aorta, type B aortic dissection extending to the terminal aorta, and a descending aortic aneurysm with a size of 33&#xa0;mm. The dissecting aneurysm rapidly expanded; therefore, we performed descending aorta replacement with coarctectomy through a left posterolateral thoracotomy. Arterial cannulas were placed on the ascending aorta and the right femoral artery to provide sufficient cerebral perfusion. To prevent paraplegia, we performed open proximal and distal aortic anastomosis in deep hypothermic cardiac arrest. The postoperative course was uneventful.</p> Conclusions <p>We report a rare case of acute type B aortic dissection in a patient with Turner syndrome and coarctation of the aorta, treated with descending aortic replacement and coarctectomy under deep hypothermic circulatory arrest. Proximal aortic obstruction limited cerebral perfusion via femoral access, requiring careful cannulation planning. Clear visualization is key to achieving hemostasis of well-developed collateral vessels and to the safe performance of complex procedures. This case underscores the importance of individualized surgical strategies and lifelong aortic surveillance in this high-risk population.</p>

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Rapidly expanding Stanford type B aortic dissection associated with coarctation of the aorta in Turner syndrome

  • 期刊论文

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  • 发表日期：

    2026年2月24日

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  • Shuhei Sakaguchi,
  • Koji Furukawa,
  • Masanori Nishimura,
  • Hirohito Ishii,
  • Kosuke Mori,
  • Ayaka Iwasaki

摘要

Introduction

Turner syndrome is the most common established cause of aortic dissection in young women. These patients usually have associated Stanford type A aortic dissection. We present herein a case of coarctation of the aorta complicated by acute type B aortic dissection in a patient with Turner syndrome—an exceptionally rare pathological combination.

Case presentation

A 40-year-old woman with Turner syndrome experienced sudden back pain. Enhanced computed tomography revealed coarctation of the aorta, type B aortic dissection extending to the terminal aorta, and a descending aortic aneurysm with a size of 33 mm. The dissecting aneurysm rapidly expanded; therefore, we performed descending aorta replacement with coarctectomy through a left posterolateral thoracotomy. Arterial cannulas were placed on the ascending aorta and the right femoral artery to provide sufficient cerebral perfusion. To prevent paraplegia, we performed open proximal and distal aortic anastomosis in deep hypothermic cardiac arrest. The postoperative course was uneventful.

Conclusions

We report a rare case of acute type B aortic dissection in a patient with Turner syndrome and coarctation of the aorta, treated with descending aortic replacement and coarctectomy under deep hypothermic circulatory arrest. Proximal aortic obstruction limited cerebral perfusion via femoral access, requiring careful cannulation planning. Clear visualization is key to achieving hemostasis of well-developed collateral vessels and to the safe performance of complex procedures. This case underscores the importance of individualized surgical strategies and lifelong aortic surveillance in this high-risk population.