Ann Thorac Surg 2002;74:908-910
© 2002 The Society of Thoracic Surgeons
Case report
Successful treatment in a patient with Takayasu’s arteritis and Marfan syndrome
Kyung-Hwan Kim, MD, PhD*a,b,
Cheul Lee, MDa,b,
Hyuk Ahn, MD, PhDa,b
a Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea
b Clinical Research Institute, Seoul National University Hospital, Seoul, South Korea
Accepted for publication April 1, 2002.
* Address reprint requests to Dr Kim, Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, 28 Yongon-dong, Chongno-gu, Seoul 110-744, South Korea
e-mail: kh726{at}snu.ac.kr
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Abstract
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We report an unusual case of concomitant Takayasu’s arteritis and Marfan syndrome manifesting left main coronary ostial obstruction and annuloaortic ectasia. Simultaneous surgical treatment consisting of left coronary ostium endarterectomy, coronary artery bypass grafting, and Bentall operation was performed. This case is unique in that the cardiovascular manifestations of Takayasu’s arteritis and Marfan syndrome were both simultaneously presented and surgically treated.
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Introduction
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We report the first case of left main coronary ostial obstruction caused by Takayasu’s arteritis and annuloaortic ectasia caused by concomitant Marfan syndrome. The young female patient successfully underwent coronary ostial endarterectomy, coronary artery bypass grafting (CABG), and Bentall operation. Histopathological examination of the ascending aorta revealed findings suggestive of Marfan syndrome.
A 22-year-old woman, on regular follow-up under the diagnosis of Takayasu’s arteritis and Marfan syndrome, was admitted to our hospital complaining of of exertional chest pain. She had a past history of undergoing aortic-to-left carotid artery bypass surgery with an 8-mm GoreTex vascular graft (W. L. Gore & Associates, Elkton, MD) due to involvement of the arch vessels by Takayasu’s arteritis. Her mother was also a Marfan syndrome patient and had undergone an operation for aortic dissection at our institution. On physical examination, there was no palpable pulse in the upper extremities. The blood pressure in the right leg was 145/80 mm Hg. There was a grade 3/6 diastolic murmur over the aortic area, and left carotid bruit was audible. Electrocardiography revealed normal sinus rhythm and nonspecific ST segment changes. The patient’s erythrocyte sedimentation rate was 14 mm/h. The chest roentgenography showed calcification and focal luminal narrowing of the proximal descending thoracic aorta with poststenotic dilatation. Echocardiography showed akinesia of the basal inferior and inferolateral wall with moderate aortic regurgitation. Coronary angiography revealed a totally occluded left main coronary artery at the ostium. However, during selective right coronary angiography, both the left anterior descending and the left circumflex coronary arteries were perfused via rich right to left collaterals (Figure 1).
Aortography revealed annuloaortic ectasia and stenosis of the proximal descending thoracic aorta with poststenotic dilatation. The arch vessels were not visualized except the left common carotid artery, which was perfused via the GoreTex vascular graft (Figure 2).
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Fig 1. Preoperative coronary angiogram showing total occlusion of the left main coronary artery at the ostium. (RCA = right coronary artery.)
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Fig 2. Preoperative aortogram showing annuloaortic ectasia and stenosis of the proximal descending thoracic aorta with poststenotic dilatation. (G = GoreTex vascular graft; LCCA = left common carotid artery.)
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In surgery, exposure was obtained through a redo median sternotomy. The ascending aorta showed a marked dilatation with a maximal diameter of 6 cm at the sinotubular junction. The diameter of the distal portion was about 3 cm. An arterial cannula was inserted into the left femoral artery and cardiopulmonary bypass was started. Retrograde cardioplegia was used for myocardial protection. After resection of the dilated ascending aorta, examination of the aortic valve revealed a dilated aortic annulus and normal aortic leaflets. The left coronary ostium was obstructed by a thick plaque. This plaque was removed and anastomosis between the left main coronary artery and 6-mm GoreTex vascular graft was performed, followed by Bentall operation with an On-X 25-mm valved 28-mm Hemashield vascular graft. Weaning from cardiopulmonary bypass was uneventful, and the postoperative course was normal. Postoperative coronary angiography showed a patent bypass graft (Figure 3).
Histopathological examination of the aortic wall revealed cystic medial myxoid degeneration with medial tear and dystrophic calcification. These findings were suggestive of Marfan syndrome. Histopathological examination of the plaque revealed myxoid degeneration with dystrophic calcification. During the follow-up period, the disease activity of Takayasu’s arteritis is being well controlled by use of corticosteroid and methotrexate. The erythrocyte sedimentation rates at 6 and 12 months after operation were 19 and 24 mm/h, respectively.
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Fig 3. Postoperative coronary angiogram showing a patent bypass graft, well-visualized left anterior descending, and left circumflex coronary arteries.
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Comment
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Takayasu’s arteritis is a nonspecific inflammatory disorder of unknown etiology involving the aorta, its main branches, and the pulmonary arteries. Coronary artery involvement occurs in about 10% of cases [1], and the presence of a coronary arterial lesion is potentially lethal in Takayasu’s arteritis. The ostia or the proximal parts of the coronary arteries are predominantly involved. The surgical treatment options for coronary ostial stenosis due to Takayasu’s arteritis have been reported to be CABG [2, 3] and ostium endarterectomy [4]. When utilizing the saphenous vein for CABG, the proximal anastomotic portion of the ascending aorta should be stenosed or occluded if inflammation is extended. Saphenous vein graft failure can occur due to intimal hyperplasia of the saphenous vein graft itself. Arterial grafts, such as those of the internal mammary artery and the gastroepiploic artery, cannot be used when the inflammation extends to the aortic arch or to the descending aorta.
Marfan syndrome is an autosomal dominant disorder of connective tissue involving the skeletal, ocular, and cardiovascular systems [5]. Aortic aneurysm and dissection are the life-threatening cardiovascular complications of Marfan syndrome. Aortic histology in Marfan syndrome shows separation and fragmentation of elastic fibers, termed cystic medial necrosis. The treatment options for aortic root aneurysm in patients with Marfan syndrome have been reported to be composite replacement of the aortic valve and ascending aorta [6] and aortic valve-sparing operations [7].
In our case, the patient had concomitant Takayasu’s arteritis and Marfan syndrome, the former involving the left coronary ostium and causing complete obstruction, the latter causing annuloaortic ectasia with aortic regurgitation. Takayasu’s arteritis can also cause aortic aneurysm and aortic regurgitation [8], but the aortic histopathology of the patient was suggestive of Marfan syndrome. We successfully performed simultaneous left coronary ostium endarterectomy, CABG, and Bentall operation in this very rare case. We used a GoreTex vascular graft for CABG due to concerns of saphenous vein graft failure. To our knowledge, this is the first case in which a patient with concomitant Takayasu’s arteritis and Marfan syndrome has undergone combined operation for complications of these two diseases.
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References
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- Lupi-Herrera E., Sanchez-Torres G., Marcushamer J., et al. Takayasu’s arteritis: clinical study of 107 cases. Am Heart J 1977;93:94-102.[Medline]
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- Kuwahara K., Ohteki H., Itoh T., Naitoh K., Furukawa K., Natsuaki M. Successful combined operation for Takayasu’s arteritis. Ann Thorac Surg 1991;52:549-551.[Abstract]
- Pyeritz R.E., McKusick V.A. The Marfan syndrome: diagnosis and management. N Engl J Med 1979;300:772-777.[Medline]
- Gott V.L., Green P.S., Alejo D.E., et al. Replacement of the aortic root in patients with Marfan’s syndrome. N Engl J Med 1999;340:1307-1313.[Abstract/Free Full Text]
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Abstract
Introduction
