Asian Cardiovasc Thorac Ann 2002;10:184-185
© 2002 Asia Publishing EXchange Pte Ltd
Successful Repair of Ascending Aortic Aneurysm Due to Takayasu's Arteritis
Emin Tireli, MD,
A Kubilay Korkut, MD,
Eylül Kafali, MD,
Murat Basaran, MD
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Department of Cardiovascular Surgery Istanbul Medical Faculty Istanbul University Istanbul, Turkey
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Emin Tireli, MD Tel: 90 212 635 2921 Fax: 90 212 534 2232 email: dr_murat_basaran{at}hotmail.com Department of Cardiovascular Surgery, Istanbul Medical Faculty, Istanbul University, Millet Caddesi, Çapa, Istanbul 34390, Turkey.
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ABSTRACT
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A 2-year-old girl with Takayasu's arteritis and an ascending aortic aneurysm underwent successful graft replacement. Although aneurysms of the great vessels are rarely encountered, this disease should be considered in the differential diagnosis of ascending aortic aneurysms in children.
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INTRODUCTION
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Takayasu's arteritis is an inflammatory disease of the arterial system, which involves mainly the aorta, the large brachiocephalic arteries derived from the aortic arch, and the pulmonary arteries. Its primary manifesta-tions are due to stenotic occlusive lesions of the involved vessels.1 However, the disease can also cause aneurysmal dilatations, usually combined with stenotic lesions or infrequently, as isolated lesions.
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CASE REPORT
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A 2-year-old girl with a diagnosis of Takayasu's arteritis was referred with a history of increasing shortness of breath, coughing, and tachycardia. She was hemo-dynamically stable and her blood pressure was 100/60 mm Hg. There was a grade 1/6 systolic murmur on auscultation. Chest radiography showed an enlarged central opacity. She had an elevated erythrocyte sedimenta-tion rate of 61 mm•h–1. Transthoracic echocardiography showed an ascending aortic aneurysm (AAA) with a diameter of 4.8 cm, extending from the sinotubular junction (Figure 1
). The aortic valve annulus was normal in size and there was no aortic insufficiency. Further diagnostic evaluation with computed tomography confirmed the initial diagnosis and showed a pear-shaped dilatation of the ascending aorta. The patient was initially placed on immunosuppressive therapy with prednisone and methotrexate, and surgery was deferred until resolution of the acute exacerbation of the disease.
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Figure 1. Transthoracic echocardiography showing a pear-shaped dilatation of the ascending aorta, extending from the sinotubular junction. AO = aorta, LA = left atrium, LV = left ventricle, RV = right ventricle.
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The operation was performed through a median ster-notomy. The patient was placed on cardiopulmonary bypass with distal ascending aortic and bicaval cannulation, and cooled to 28°C. A vent was inserted into the left ventricle through the right superior pulmonary vein. The ascending aorta was rigid on palpation, and a pear-shaped dilatation was observed (Figure 2). The sinotubular ridge was preserved and the aortic annulus was not dilated. The aortic diameter was normal just beyond the origin of the innominate artery. The aorta was clamped just proximal to the innominate artery. After aortotomy, cold blood cardioplegia was infused directly through both coronary ostia. The leaflets of the aortic valve were inspected. The free edges of the leaflets were thin and mobile. The ascending aorta was completely transected immediately above the sinotubular junction and just below the aortic crossclamp. A 22-mm Dacron tube graft was cut to an appropriate length and sutured to the proximal aorta with 5/0 polypropylene suture. The distal end of the tube graft was beveled to accommodate the proximal arch anasto-mosis just below the aortic crossclamp, and sutured to the proximal portion of the aortic arch using 5/0 polypropylene suture. The crossclamp time was 28 minutes, and weaning from cardiopulmonary bypass did not require inotropic support. The postoperative course was uneventful and the child was discharged after 9 days. Histology of the specimen removed from the ascending aorta confirmed the initial diagnosis (Figure 3).
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Figure 3. Histological examination of the specimen confirmed the diagnosis of Takayasu's arteritis (Elastica von Gieson stain, original magnification x200).
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DISCUSSION
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Although the incidence of aneurysms in Takayasu's arteritis varies from 2% to 26.7%, isolated AAAs are very rare.2 We believe this is the youngest patient reported to have an AAA. The choice of procedure in patients with aneurysms of the ascending aorta is based mainly on the preoperative studies, although certain intraoperative findings may require a change in the operative strategy. Two principles guide the operative approach to proximal and distal anastomoses: whether the sinotubular ridge is preserved and whether most of the aortic arch is aneurysmal. In this case, since the sinotubular ridge was preserved, the tube graft was sutured above the aortic valve commissures. Preservation of the aortic root and the absence of annular dilatation can be explained by the young age of the patient. Although most of the aortic arch was not aneurysmal, the slight dilatation noticed at the origin of the innominate artery forced us to perform a beveled anastomosis incorporating the proximal portion of the lesser curvature to the anastomotic site.
Patients with a definite clinical and pathological diagnosis of Takayasu's arteritis should be completely evaluated by echocardiography, computed tomography, or magnetic resonance imaging. We recommend early surgery as the sole treatment in order to prevent the potentially catastrophic outcome of aortic involvement. Therefore, the age of the patient should not be considered as a contraindication to surgical intervention. Further, aneu-rysmal aortitis in Takayasu's disease must be distinguished from other forms of aortitis associated with aneurysm formation. Syphilis usually occurs in adults with positive serology, and it affects the ascending aorta and aortic arch.3 In giant cell arteritis which is also seen in older patients, small and medium-sized arteries are commonly involved.4 Tuberculosis of the aorta usually causes false or true aneurysm, but it is completely different from Takayasu's arteritis clinically and histologically.1 Takayasu's arteritis should be considered as the possible cause of an AAA, especially in children.5 In patients with major vessel involvement, definitive diagnosis is critically important. After instituting medical treatment to control the acute phase of the disease, appropriate surgical intervention should be performed as soon as possible.
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REFERENCES
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Kumar V, Cotran RS, Robbins SL. Disease of blood vessels. In: Kumar V, Cotran RS, Robbins SL. Basic pathology. 5th ed. Philadelphia: Saunders, 1992:277–304.
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Gilmor JR. Giant-cell chronic arteritis. J Pathol Bacteriol
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Giordano JM, Hoffman GS. Takayasu's disease: nonspecific aortoarteritis. In: Rutherford RB. Vascular surgery. 5th ed. Philadelphia: Saunders, 2000:364–73.
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ABSTRACT
INTRODUCTION
