Ann Thorac Surg 2007;83:1876-1878
© 2007 The Society of Thoracic Surgeons
Case Reports
Takayasu’s Arteritis Mimicking Acute Aortic Dissection
Sanjay Theodore, MCh*,
Karthik Vaidyanathan, MS,
Byalal Raghavendrarao Jagannath, DNB,
MadhuSankar Nainar, DNB,
Jaishankar Krishnamoorthy, DM,
Kotturathu Mammen Cherian, FRACS
Department of Thoracic and Cardiovascular Surgery, Frontier Lifeline, Dr KM Cherian Heart Foundation, Mogappair, Chennai, India
Accepted for publication November 13, 2006.
* Address correspondence to Dr Theodore, Frontier Lifeline Ltd, Dr KM Cherian Heart Foundation, R-30C Ambattur Industrial Estate Rd, Mogappair, Chennai 600 101 India (Email: sanjayawake{at}gmail.com).
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Abstract
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A 17-year-old girl presented with a history of dyspnea on exertion and fever of 1-week duration. She was evaluated elsewhere with transesophageal echocardiography and helical computed tomographic scan, and she had been diagnosed with an acute type I dissection of the aorta. She had also been diagnosed with severe aortic regurgitation and a suspected aortic root abscess. On the operating table, we found no evidence of dissection, but we did find that her aorta was severely thickened and inflamed. The patient’s aortic valve was replaced. In view of the left main stem ostial stenosis, we harvested and grafted the left internal thoracic artery to the left anterior descending artery. During the operation it is of paramount importance to rule out dissections involving the arch and coronary ostial narrowing.
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Introduction
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Takayasu’s arteritis (TA) is a nonspecific aorto-arteritis involving the aorta and its branches. The clinical presentation of Takayasu’s arteritis is varied. Aortic dissection as a manifestation of this entity has been rarely reported [1]. We report the case of a 17-year-old girl who was mistakenly diagnosed as having an acute aortic dissection with severe aortic regurgitation, but on the operating table it was found that she actually had aorto-arteritis.
A 17-year-old girl presented with a history of dyspnea on exertion of recent onset and a fever of 1-week duration. She was evaluated elsewhere with transesophageal echocardiography and a helical computed tomographic scan, and she was diagnosed with acute type I dissection of the aorta. She was also diagnosed with severe aortic regurgitation and a suspected aortic root abscess.
On examination she had a Marfanoid habitus. She was febrile, her blood pressure was 140/50 in the right upper limb, and there was no pressure gradient between the upper limbs, but a 50 mm Hg gradient between the upper and lower limb blood pressures was found. The precordium was hyperactive with a 3/6 early diastolic murmur in the right upper sternal border.
We performed an operation on the patient through a midline sternotomy. The ascending aorta and main pulmonary artery looked edematous with thickening and punctuate hemorrhage. Femoral artery and right atrial cannulation was performed and the patient was placed on cardiopulmonary bypass. Retrograde cardioplegia was given to arrest the heart, a left ventricular vent was inserted, and the aorta was clamped and opened. The aortic wall was thickened to about 1 cm. There was no dissecting flap in the ascending aorta. In a steep head down position, circulation was arrested for a brief period of time, and the clamp was removed to evaluate the arch that was also found to be free of dissection. The aortic valve was thickened and non-coapting. The left main ostium was pinpointed. Her aortic valve was replaced with a 23-mm mechanical tilting disc valve, and the aortotomy was directly closed. In view of the left main stem ostial stenosis we harvested and grafted the left internal thoracic artery to the left anterior descending artery.
The patient made an uneventful postoperative recovery. She was put on an injectable steroid in the immediate postoperative period. She was discharged with oral steroids. At her 6-month follow-up her erythrocyte sedimentation rate was normal and she was asymptomatic.
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Comment
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Takayasu’s arteritis is characterized by occlusive or aneurysmal disease of the aorta and its branches. Rarely the inflamed aorta of Takayasu’s arteritis can be a source of dissection, but more commonly the varied symptoms and signs associated with Takayasu’s arteritis can mimic an aortic dissection [2].
Our patient presented with symptoms suggestive of aortic dissection and this was investigated with echocardiography, which showed features of aortic dissection involving the aortic root, and the ascending aorta and arch with severe aortic regurgitation. Helical computed tomography confirmed the presence of type A dissection involving the descending thoracic aorta (Fig 1A). There also seemed to be a dissection involving the main pulmonary artery (Fig 1B).
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Fig 1. (A) Sagittal cut showing diffuse disease up to the diaphragm. (B) Axial cut showing an artifact indicating a pulmonary artery dissection (arrow).
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There have been previous reports in which mistaken diagnosis of aortic dissection was made with both echocardiography and computed tomography due to artifacts [3]. The sensitivity of computed tomography and transthoracic echocardiography were 98.3% and 59.3%, respectively, and the specificity of these were only 83% and 76.9%, respectively, in the diagnosis of aortic dissection in one large study [4]. The relatively low specificity of echocardiography for diagnosis of dissection has been attributed to linear artifacts [5]. Pitfalls in computed tomographic diagnosis include technical factors such as improper timing of contrast material, streak artifacts generated by high attenuation material, high contrast interface, cardiac motion, and periaortic structures [6].
If the correct diagnosis had been made on this patient prior to surgery, an operation could have been deferred to allow for resolution of aortitis. Surgery even could have been avoided, because 60% of patients respond to steroids alone with resolution of aortitis, stenosis, and aneurysms [7]. The impact of steroid therapy for aortic regurgitation could have been studied, because the regurgitation was due to aortitis and not valvular pathology per se.
During surgery we found a thickened and inflamed aortic root and a diagnosis of aortitis was made on table. We confirmed there was no dissection flap in the arch of the aorta. Left main coronary ostia were pinpointed and were not admitting the cardioplegia cannula. Takayasu’s arteritis commonly affects the coronary ostia and vein grafts implanted into the diseased aortic root and are prone to ostial narrowing [8]. We elected to graft the left internal thoracic artery to the left anterior descending artery, because there was no left subclavian disease. On opening the left pleura it was found that the descending aorta was inflamed with multiple adhesions.
It is important to begin these patients on steroid therapy in the postoperative period [5]. Our patient had remarkable improvement in constitutional symptoms and a decline in erythrocyte sedimentation rate. A postoperative 64-slice computed tomographic scan did not show any dissection, and multiple areas of disease were found in the ascending, arch and descending aorta with a functioning internal mammary artery graft (Fig 2A). Left main coronary artery was also found to have 50% stenosis (Fig 2B).
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Fig 2. (A) Reconstructed 64-slice computed tomographic scan shows a functioning internal thoracic artery graft. (B) Reconstructed 64-slice computed tomographic scan shows left main stenosis.
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In conclusion it is possible that a patient with Takayasu’s arteritis may be operated on for a mistaken diagnosis of dissection due to falsely positive investigations. During surgery it is of paramount importance to rule out dissections involving the arch and coronary ostial narrowing. Surgical correction should be followed with steroid therapy and long-term follow-up.
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References
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- Ando M, Okita Y, Tagusari O, Kitamura S, Matsuo H. A surgically treated case of Takayasu’s arteritis complicated by aortic dissections localized in the ascending and abdominal aortae J Vasc Surg 2000;31:1042-1045.[Medline]
- Reichman EF, Weber JM. Undiagnosed Takayasu’s arteritis mimicking an acute aortic dissection J Emerg Med 2004;27:139-142.[Medline]
- Shanmugam G, McKeown J, Bayfield M, Hendel N, Hughes C. False positive computed tomography findings in aortic dissection Heart Lung Circ 2004;13:184-187.[Medline]
- Nienaber CA, von Kodolitsch Y, Nicolas V, et al. The diagnosis of thoracic aortic dissection by noninvasive imaging procedures NEJM 1993;328:1-9.[Abstract/Free Full Text]
- Vignon P, Spencer KT, Rambaud G, et al. Differential transesophageal echocardiographic diagnosis between linear artifacts and intraluminal flap of aortic dissection or disruption Chest 2001;119:1778-1790.[Medline]
- Batra P, Bigoni B, Manning J, et al. Pitfalls in the diagnosis of thoracic aortic dissection at CT angiography Radiographics 2000;20:309-320.[Abstract/Free Full Text]
- Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: a review J Clin Pathol 2002;55:481-486.[Abstract/Free Full Text]
- Endo M, Tomizawa Y, Nishida H, et al. Angiographic findings and surgical treatments of coronary artery involvement in Takayasu arteritis J Thorac Cardiovasc Surg 2003;125:570-577.[Abstract/Free Full Text]
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Abstract
Introduction
