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Year : 2014  |  Volume : 2  |  Issue : 3  |  Page : 86-87

Giant cell arteritis causing annuloaortic ectasia

1 Department of Pathology, K S Hegde Medical Academy of Nitte University, Deralakatte, Mangalore, Karnataka, India
2 Department of Cardiothoracic and Vascular Surgery, K S Hegde Medical Academy of Nitte University, Deralakatte, Mangalore, Karnataka, India

Date of Web Publication5-Sep-2014

Correspondence Address:
Shetty Kailkere Padma
Department of Pathology, K S Hegde Medical Academy of Nitte University, Deralakatte, Mangalore - 575 018, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2321-449x.140234

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Giant cell arteritis (GCA) is a rare cause of ascending aortic aneurysm. We present here a 50-year-old patient who was clinically diagnosed with annuloaortic ectasia. She was successfully treated by Bentall's operation. Histopathologically, the findings were of GCA. GCA should be suspected when older women presented with aortic aneurysm and continued surveillance of remaining aorta is necessary.

Keywords: Annuloaortic ectasia, aortic regurgitation, giant cell arteritis

How to cite this article:
Padma SK, Gopalakrishnan M, Sajitha K, Impana B D. Giant cell arteritis causing annuloaortic ectasia. Heart India 2014;2:86-7

How to cite this URL:
Padma SK, Gopalakrishnan M, Sajitha K, Impana B D. Giant cell arteritis causing annuloaortic ectasia. Heart India [serial online] 2014 [cited 2022 Jan 21];2:86-7. Available from: https://www.heartindia.net/text.asp?2014/2/3/86/140234

  Introduction Top

Giant cell arteritis (GCA) is one of the most common vasculitides in the population above the age of 50 years. [1] Also called Horton's disease, it is a systemic vasculitis that primarily affects the extracranial arteries of head and neck in the elderly. [2] However, the aorta with any of its primary and secondary branches may be affected. Aortic aneurysm and aortic dissection have been described in GCA. Annuloaortic ectasia is a common cause of ascending aortic aneurysm. GCA causing annuloaortic ectasia is a very rare entity. We report a case of annuloaortic ectasia in which the histological diagnosis was GCA.

  Case report Top

A 50-year-old woman was referred to our hospital for dyspnea on exertion, Class II of 1 month duration. Her general examination was unremarkable. Blood pressure was within normal limits. Her cardiovascular examination revealed an early diastolic murmur at the aortic area. Chest x-ray showed dilatation of ascending aorta.[Figure 1] Echocardiography showed dilated ascending aorta with moderate aortic regurgitation. Her coronary angiogram revealed normal coronaries. Computerized tomography aortogram revealed aneurysmal ascending aorta with a size of about 5 cm. Arch vessels and arch were normal. There were no features of Marfan's syndrome.
Figure 1: Chest X-ray-showing dilatation of ascending aorta

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She underwent elective Bentall's operation. At surgery patient was found to have dilated aortic annulus, thinned out leaflets with lax cusps. Aortic root was thinned out and showed calcification. Two-thirds of ascending aorta showed aneurysmal dilation of >5 cm diameter. The aortic valve and ascending aorta were replaced with 21 mm St. Jude composite graft. [Figure 2] Postoperatively patient recovered completely with remarkable symptomatic improvement.

Histopathological examination of the aortic wall showed; dense connective that tended to hyalinization. There was perivascular infiltration of lymphocytes, plasma cells, and giant cells. Some of the multinucleated giant cells were of Langhans type.[Figure 3] and [Figure 4] The pathological findings were suggestive of GCA
Figure 2: Postoperative echocardiogram-showing the composite graft in position

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Figure 3: Granulomatous inflammation with Langhan's giant cells, plasma cells and lymphocytes with central hyalinized connective tissue (H and E ×100)

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Figure 4: H and E ×400-higher power view of the granulomatous lesion

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  Discussion Top

Despite GCA being a common cause of vasculitis, it is a rare cause of annuloaortic ectasia. Annuloaortic ectasia is a common finding in connective tissue diseases such as Marfan's syndrome. [3] There is an abnormal dilation of the aortic root with noncoaptation of the aortic leaflets. The most common histopathological finding in Marfan's syndrome is cystic medial necrosis because of abnormal fibrillin in the vascular walls. This patient did not have any of the clinical features of Marfan's syndrome. Except for age, she did not fulfill any of the other features as per the American college of Rheumatology criteria for clinical diagnosis of GCA. [4] Our patient presented with a short history of breathlessness and was clinically diagnosed as annuloaortic ectasia. Bentall's operation with replacement of ascending aorta with aortic valve is a standard procedure for this condition, which was performed in our patient. Alternate procedure called David's operation in which aortic valve is conserved; replacing only the aortic root is being performed increasingly in some centers.

Histopathologically, this patient had the classical picture of granulomatous inflammation with Langhans giant cells, plasma cells and lymphocytes along with hyalinized connective tissue.

Patients with GCA were 17.3 times more likely to develop a thoracic aortic aneurysm and 2.4 times more likely to develop abdominal aortic aneurysm compared to the general population. [5] Frequent surveillance of the remaining aorta is mandatory in patients with GCA since aneurysms of aorta and other great vessels occur in nearly half of the patients. [6] GCA should be suspected when cardiologists come across aortic aneurysm, especially in older women. [7]

  References Top

1.Stvrtina S, Rovensky J. Histopathology of giant cell (Temporal) arteritis changes in aorta. Scr Med (BRNO) 2006;79:221-36.  Back to cited text no. 1
2.Nesi G, Anichini C, Pedemonte E, Tozzini S, Calamai G, Montesi GF, et al. Giant cell arteritis presenting with annuloaortic ectasia. Chest 2002;121:1365-7.  Back to cited text no. 2
3.Cozijnsen L, Braam RL, Waalewijn RA, Schepens MA, Loeys BL, van Oosterhout MF, et al. What is new in dilatation of the ascending aorta? Review of current literature and practical advice for the cardiologist. Circulation 2011;123:924-8.  Back to cited text no. 3
4.Ponte E, Cafagna D. Horton's giant cell arteritis. Minerva Med 1997;88:245-55.  Back to cited text no. 4
5.Evans JM, O'Fallon WM, Hunder GG. Increased incidence of aortic aneurysm and dissection in giant cell (temporal) arteritis. A population-based study. Ann Intern Med 1995;122:502-7.  Back to cited text no. 5
6.Zehr KJ, Mathur A, Orszulak TA, Mullany CJ, Schaff HV. Surgical treatment of ascending aortic aneurysms in patients with giant cell aortitis. Ann Thorac Surg 2005;79:1512-7.  Back to cited text no. 6
7.Gelsomino S, Romagnoli S, Gori F, Nesi G, Anichini C, Sorbara C, et al. Annuloaortic ectasia and giant cell arteritis. Ann Thorac Surg 2005;80:101-5.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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