[Aortic dissection associated with Turner's syndrome]

Kyobu Geka. 1992 May;45(5):411-4.
[Article in Japanese]

Abstract

We report two cases of Turner's syndrome who had had coarctation repair and who ultimately died from complications believed to be associated with Turner's stigmata. Case 1. The repair of coarctation of the aorta (Co/Ao) at age 5 was associated with the tear of the aortic wall and an Asc. Ao-Desc. Ao bypass together with the closure of the Ao proximal to the Co/Ao was performed. At age 10, she developed a dissecting aneurysm of the Asc. Ao seemingly unrelated to the previous surgery and the Asc. Ao was replaced by a prosthetic tube graft. Pathological diagnosis was cystic medionecrosis. The aortic valve was bicuspid. Subsequently, the patient showed progressive worsening of the aortic regurgitation. The aortic valve replacement at age 14 was complicated by significant bleeding and the patient died on the fifth postoperative day. Case 2. Co/Ao was repaired at age 8. During the operation, the aortic wall appeared to be thin and friable, and there was some tear of the posterior suture line which necessitated reanastomosis with resultant stenosis. At age 9, stenotic area was enlarged by an onlay-patch of a piece of woven Dacron arterial graft. There was gradual swelling of that area on routine x-ray examinations and an aortography at age 16 showed definite aneurysm formation. At age 18, she suddenly developed hemoptysis. Rupture of the aneurysm into the pleural cavity and the left lung was suspected. An emergency surgery to stop bleeding under hypothermic cardiopulmonary bypass was unsuccessful and the patient died in OR.(ABSTRACT TRUNCATED AT 250 WORDS)

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Aneurysm, Dissecting / complications
  • Aneurysm, Dissecting / surgery*
  • Aorta, Thoracic / surgery
  • Aortic Aneurysm / complications
  • Aortic Aneurysm / surgery*
  • Aortic Coarctation / complications
  • Aortic Coarctation / surgery
  • Child
  • Child, Preschool
  • Humans
  • Turner Syndrome / complications*