Rupture of the Aortic Arch and Descending Aortic Aneurysm in a 24-Year-Old Man with Systemic Lupus Erythematosus

(Department of Cardiovascular Surgery, Sunagawa Medical Center, Sunagawa, Japan)

Akihiko Sasaki Akira Fujii Masahiro Miyajima
A 24-year-old man with systemic lupus erythematosus (SLE) had received long term steroid therapy 10 years prior to this admission. He presented with sudden-onset chest pain. Enhanced CT scan showed the presence of an aortic arch aneurysm 63mm in maximum diameter and a hematoma surrounding the anterior mediastinum. The diameter of the descending thoracic aorta was also dilated to 5cm. We performed ascending and total arch replacement on December 8, 2005. From the 11th postoperative day, he developed fever, indicating mediastinitis. Open drainage was carried out for one week resulting in gradual lysis of fever and the levels of WBC and CRP returned to normal values. The omentum was transplanted to close the defect in the mediastinum. The rest of the postoperative course was uneventful. He was discharged from the hospital last January 25, 2006. Although close medical follow-up was implemented, he had severe chest pain in the morning on June 9, 2006. Enhanced CT showed an expanding descending aortic aneurysm 60mm in diameter. Since antihypertensive therapy was effective, we considered an elective operation. On the 3rd hospital day, he complained of a severe back pain wherein he rapidly progressed into a state of shock. He died due to rupture of the descending aortic aneurysm. We needed emergency operation or endovascular stent graft therapy because of the risk of rupture.
@Jpn. J. Cardiovasc. Surg. 37: 17-20 (2008)