Case Study

Giant-cell aortitis: an unusual case of Bentall operation

Asian Cardiovascular & Thoracic Annals 2014, Vol. 22(3) 342–344 ß The Author(s) 2012 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492312470669 aan.sagepub.com

Anaı¨s Lemaire1, Fabio Cuttone1, Sabino Caprio1, Massimo Massetti1 and Franc¸oise Galateau-Salle2

Abstract Noninfectious ascending aortitis is a very rare cause of ascending aortic aneurysm. We report a case of the truly fortuitous finding of this rare condition in a 67-year-old man operated on for an ascending aortic aneurysm associated with dystrophic aortic valve regurgitation. Intraoperative inspection revealed dissection of the aorta just above the left main coronary artery. A modified Bentall operation was performed. The pathological diagnosis was giant cell arteritis.

Keywords Aortic aneurysm, thoracic, aortitis, aneurysm, dissecting, giant cell arteritis, incidental findings

Introduction Giant cell arteritis (GCA) is an inflammatory vasculopathy affecting large and medium-sized arteries, and its incidence has been reported as 18.8 per 100,000 per year.1 Aortic involvement has been estimated to occur in 10% to 15% of patients with GCA, but it is most often asymptomatic.2 Clinical giant cell aortitis commonly presents as an ascending aortic aneurysm involving the ascending aorta at and above the sinotubular junction, frequently extending into the aortic arch;3 but in some cases, aortitis is an incidental finding at the time of histopathological examination after surgery for aortic aneurysm.1 The relative risk of developing an aortic dissection or aneurysm is 17.3.4 We describe the truly fortuitous finding of this rare condition in a patient with ascending aortic aneurysm associated with dystrophic aortic valve regurgitation.

Case report A 67-year-old man was referred to our department with a large ascending aortic aneurysm. His past history was marked by a stroke 9 years earlier, without sequelae, and hepatic hemangioma. He had no history suggesting temporal arteritis, polymyalgia rheumatica, tuberculosis, or systemic inflammatory response syndrome.

He was asymptomatic except for moderate effort dyspnea (New York Heart Association functional class II). The surgical indication for this progressively increasing aneurysm was raised after 5 years of follow-up. Preoperative computed tomography (Figure 1) showed dilatation of the aortic root (45 mm in diameter) with loss of the sinotubular junction and an ascending aorta aneurysm (52 mm in diameter). This was consistent with transthoracic echocardiography measurements. The aortic valve was tricuspid and dystrophic with moderate valve regurgitation. Preoperative coronary angiography demonstrated the absence of coronaropathy (Figure 2). After a full median sternotomy, the pericardium was opened longitudinally. The ascending aorta was found to be dilated. The patient was cannulated in the standard fashion. The ascending aorta was circularly opened. Intraoperative inspection 1 Department of Cardiac Surgery, University of Caen Basse-Normandie and University Hospital of Caen, France 2 Department of Pathology, University of Caen Basse Normandie and University Hospital of Caen, France

Corresponding author: Anaı¨s Lemaire, Department of Cardiac Surgery, University Hospital of Caen, Avenue de la Coˆte de Nacre, 14033 Caen Cedex 9, France. Email: [email protected]

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Figure 1. Preoperative 3-dimensional computed tomography reconstruction: (a) axial and (b) frontal views showing dilatation of the aortic root with loss of the sinotubular junction and an ascending aortic aneurysm. The aortic dissection above the left main coronary artery was not visible in these images.

Discussion

Figure 2. Coronary angiography was also unable to detect the coronary artery lesion. Note the normal appearance of the left main coronary artery (white arrow).

revealed chronic dissection just above the left main coronary artery, associated with an intramural hematoma. The patient underwent a modified Bentall procedure (button technique) with fixation of the proximal intimal flap on the left main coronary artery. The aortic crossclamp time was 120 min, and cardiopulmonary bypass time was 147 min. All resected fragments were referred to a pathologist for analysis. This revealed an abundance of inflammatory cells including giant multinucleated cells, which indicated active GCA. The elastic lamina was disrupted by medial necrosis (Figure 3). The postoperative course was uneventful, and the patient was discharged from the hospital on the 8th postoperative day.

The originality of our case is that GCA was a truly isolated finding because the patient had no history of rheumatologic symptoms nor signs of temporal arteritis or inflammatory response syndrome. Moreover, we found very uncommon anatomical and histological lesions at the expense of the left main coronary artery, which had yet no clinical relevance and was not seen on preoperative coronary angiography. Histology of GCA often reveals nearcomplete disruption of the elastic medial layer, which predispose to rupture and dissection before the aneurysm achieves the standard size for intervention.3,5,6 The coronary artery dissection in our case perfectly illustrates this susceptibility and the need for rapid surgery in this pathology. Afterwards, those patients incidentally diagnosed with GCA should benefit from frequent surveillance of the remaining aorta because GCA of the ascending aorta is associated with increased frequency of distal aortic events during long-term follow-up, even in the absence of signs or symptoms of systemic vasculitis.3,7 Some reports in the literature suggest that aggressive steroid therapy is effective in GCA and can prevent dilatation of the remaining aorta and great vessels, but this is still unclear.3,8 Giant cell aortitis is one the rare causes of ascending aortic aneurysm, which can also reach the coronary arteries. The possibility of this diagnosis should be suspected and investigated even in the absence of systemic symptoms. On the other hand, patients presenting with diagnosed GCA-related aortic aneurysm should be quickly evaluated by a multidisciplinary team because of the high risk of sudden death due to aortic dissection or rupture. If aortitis is an incidental histopathologic

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Figure 3. Histopathologic analysis of the resected fragments diagnosed giant cell aortitis. (a) Inflammatory infiltrate in the media with medial necrosis and disruption of the elastic lamina. Hematoxylin and eosin stain, original magnification  40. (b) Close-up of the chronic inflammatory infiltrate including giant multinucleated cells. Hematoxylin and eosin stain, original magnification  100.

finding, the patient requires careful and prolonged follow-up of the remaining aorta and great vessels. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Conflicts of interest statement None declared.

References 1. Gornik HL and Creager MA. Aortitis [Review]. Circulation 2008; 117: 3039–3051. 2. Weyand CM and Goronzy JJ. Giant cell arteritis and polymyalgia rheumatic. Ann Intern Med 2003; 139: 505–515. 3. Zehr KJ, Mathur A, Orszulak T, Mullany C and Schaff HV. Surgical treatment of ascending aortic aneurysms in patients with giant cell aortitis. Ann Thorac Surg 2005; 79: 1512–1517.

4. Josselin-Mahr L, El Hessen TA, Toledano C, et al. Inflammatory aortitis in giant cell arteritis. Presse Med 2012 Apr 30. [Epub ahead of print]. 5. Liu G, Shupak R and Chiu BK. Aortic dissection in giantcell arteritis. Semin Arthritis Rheum 1995; 25: 160–171. 6. Lie JT. Aortic and extracranial large vessel giant cell arteritis: a review of 72 cases with histopathologic documentation. Semin Arthritis Rheum 1995; 24: 422–431. 7. Wang H, Smith RN, Spooner AE, et al. Giant cell aortitis of the ascending aorta without signs or symptoms of systemic vasculitis is associated with elevated risk of distal aortic events. Arthritis Rheum 2012; 64: 317–319. 8. Blumstein H, Dubin Kerr L and Fallon JT. Giant cell aortitis with histopathologic and clinical response to steroid therapy: a case report. J Thorac Cardiovasc Surg 2006; 132: 1467–1468.

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Giant-cell aortitis: an unusual case of Bentall operation.

Noninfectious ascending aortitis is a very rare cause of ascending aortic aneurysm. We report a case of the truly fortuitous finding of this rare cond...
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