|Year : 2014 | Volume
| Issue : 3 | Page : 86-87
Giant cell arteritis causing annuloaortic ectasia
Shetty Kailkere Padma1, Mundayat Gopalakrishnan2, K Sajitha2, BD Impana2
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 Publication||5-Sep-2014|
Shetty Kailkere Padma
Department of Pathology, K S Hegde Medical Academy of Nitte University, Deralakatte, Mangalore - 575 018, Karnataka
Source of Support: None, Conflict of Interest: None
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
| Introduction|| |
Giant cell arteritis (GCA) is one of the most common vasculitides in the population above the age of 50 years.  Also called Horton's disease, it is a systemic vasculitis that primarily affects the extracranial arteries of head and neck in the elderly.  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|| |
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.
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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| Discussion|| |
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.  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.  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.  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.  GCA should be suspected when cardiologists come across aortic aneurysm, especially in older women. 
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]