Isolated Necrotizing Aortitis Presenting as Incidental Thoracoabdominal Aortic Aneurysm: A Case Report

ABSTRACT

Noninfectious aortitis is frequently asymptomatic yet often leads to the development of ascending aortic aneurysms requiring repair. We present the case of a 64-year-old Caucasian woman who presented to our medical center with an incidentally discovered thoracoabdominal aneurysm. She had previously been in good health and had not complained of chest pain or been otherwise symptomatic. At presentation to our clinic, her ascending aorta measured 5 cm at the sinotubular junction (STJ) with dilation of her descending thoracic aorta to 6 cm. Her coronary angiogram was normal. Cross-sectional imaging was notable for thickening of the aortic wall along its length. The entity of isolated noninfectious aortitis is increasingly being recognized as an identifiable factor leading to ascending aneurysmal disease. In reviewing this case, we outline current understanding and guidelines for management and follow-up of this pathology. Operative repair of the ascending arch was conducted using an interpositional graft. The aortic valve apparatus was normal and the proximal anastomosis was created at the STJ. Intraoperatively, an inflammatory obliteration of the aortopulmonary window was noted. It was not possible to completely excise the posterior aortic wall. Our patient had an uneventful recovery and final pathology revealed necrotizing aortitis (NA). She is currently undergoing routine surveillance of her descending thoracoabdominal aneurysm. Recent case series indicate that NA is a histologically distinct process, which is associated commonly with development of ascending aneurysm, and although it is most commonly an isolated finding, it may be associated with other vascular abnormalities including stenoses of branch vessels. There is a growing body of literature suggesting that NA represents a distinct clinical entity, associated with the development of ascending aortic aneurysms. Further research is required to determine the optimal follow-up and value of medical therapies.

KEYWORDS

Aorta - surgery - etiology - aortic aneurysms

REFERENCES

• 1 Gornik H L, Creager M A. Aortitis.  Circulation. 2008;  117 3039-3051
• 2 Merkel P A. Noninfectious ascending aortitis: staying ahead of the curve.  J Rheumatol. 2009;  36 2137-2140
• 3 Burke A P, Tavora F, Narula N, Tomaszewski J E, Virmani R. Aortitis and ascending aortic aneurysm: description of 52 cases and proposal of a histologic classification.  Hum Pathol. 2008;  39 514-526
• 4 Miller D V, Isotalo P A, Weyand C M, Edwards W D, Aubry M C, Tazelaar H D. Surgical pathology of noninfectious ascending aortitis: a study of 45 cases with emphasis on an isolated variant.  Am J Surg Pathol. 2006;  30 1150-1158
• 5 Mennander A A, Miller D V, Liang K P et al.. Surgical management of ascending aortic aneurysm due to non-infectious aortitis.  Scand Cardiovasc J. 2008;  42 417-424
• 6 Theodore S, Madhusankar N, Jagannath B R, Cherian K M. Importance of ostial coronary stenosis in non infectious aortitis.  Asian Cardiovasc Thorac Ann. 2007;  15 82
• 7 Chau E M, Wang E, Chiu C S, Chow W H. Non-infectious aortitis: an important cause of severe aortic regurgitation.  Asian Cardiovasc Thorac Ann. 2006;  14 177-182
• 8 Rojo-Leyva F, Ratliff N B, Cosgrove III D M, Hoffman G S. Study of 52 patients with idiopathic aortitis from a cohort of 1,204 surgical cases.  Arthritis Rheum. 2000;  43 901-907