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Total Aorta Replacement in Takayasu Arteritis Presenting as Mega Aorta
CASE REPORT
Case Report
Total Aorta Replacement in Takayasu Arteritis Presenting as Mega Aorta Manju Gupta, MCh ∗ , Anil Bhan, MCh, Sandeep Khanzode, MS, Shambhu Das, MD and Ruma Ray, MD Departments of Cardiothoracic & Vascular Surgery, Cardiac Anaesthesia & Cardiac Pathology, All India Institute of Medical Sciences, New Delhi, India
We report a unique case of successful two-stage total aortic replacement from ascending aorta to aortic bifurcation that was done for extensive aneurysmal involvement of the aorta in Takayasu arteritis. (Heart, Lung and Circulation 2007;16:382–384) © 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved. Keywords. Aortic aneurysm; Takayasu arteritis
Introduction
T
akayasu’s arteritis is an inflammatory arteriopathy that progresses to obliteration of multiple large arteries and the aneurysmal dilatation of the aorta is a rare occurrence.1 To the best of our knowledge, total aortic replacement for aneurysm of aorta from ascending aorta to aortic bifurcation in Takayasu disease has not been reported in the English literature. We report a unique case of successful two-stage total aortic replacement from ascending aorta to aortic bifurcation.
Case Report A 25-year-old male presented with history of abdominal pain and low-grade fever. On clinical examination there was a lump in left lower abdomen and monoparesis of left lower limb with fixed flexion deformity involving the left hip joint. Laboratory data showed the erythrocyte sedimentation rate as 54 mm/h. Ultrasound abdomen exhibited an abdominal aortic aneurysm. Chest X-ray revealed widening of mediastinum suggestive of aneurysm of ascending arch and descending thoracic aorta. Echocardiography showed an aneurysm of ascending arch and descending aorta with trivial aortic regurgitation. Contrast enhanced computed tomography scan of the chest and abdomen confirmed the presence of an extensive aortic aneurysm with maximum dilatation of 6–10 cm. The aortic wall was diffusely thickened with an intramural thrombus in the descending thoracic aorta with compression and displacement of adjacent Available online 6 March 2007 ∗
Corresponding author. Tel.: +919868301060. E-mail address: [email protected] (M. Gupta).
structures in the thorax and abdomen and no involvement of origin of great vessels. CT angiography revealed extensive aortic aneurysm with intramural thrombus and a pseudoaneurysm of the left common iliac artery (Fig. 1). In the first stage, the supra-coronary ascending aortic replacement, hemiarch replacement and an elephant trunk procedure was done from midsternotomy approach under deep hypothermic circulatory arrest with retrograde cerebral perfusion. The arterial inflow was through transaxillary route with bicaval venous drainage for cardiopulmonary bypass circuit. Two weeks later, completion of elephant trunk procedure along with replacement of total aorta upto aortic bifurcation was done through left thoracoabdominal approach. The celiac trunk, superior mesenteric and right renal arteries were implanted on the aortic graft as a patch. The left renal artery was accidentally transected requiring a saphenous vein graft for reconstruction. The pseudoaneurysm of the left common iliac artery was repaired using a patch. The postoperative period was uneventful and the patient was discharged on fortieth postoperative day. At four-month follow-up, there was relief of abdominal and lower limb pain with palpable lower limb pulses. There was improvement in flexion deformity and monoparesis with physiotherapy. The CT angiography revealed a good functional graft with adequate perfusion of all the visceral vessels (Figs. 2 and 3).
Histology On the gross appearance, the aortic wall was irregularly thick (thickness 3–8 mm) with glossy myxoid appearance of the intima. Microscopic examination revealed panaortitis with thickening and neomyointimal prolif-
© 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.
1443-9506/04/$30.00 doi:10.1016/j.hlc.2006.11.009
Gupta et al. Total aorta replacement in Takayasu arteritis
383
eration of the intima, widespread destruction with loss of elastic tissue of the media with focal giant-cell rich granulomatous reaction and adventitial fibrosis with thickening of the vasa vasora suggestive of Takayasu arteritis.
Comment
Figure 1. Preoperative CT angiography revealed extensive aortic aneurysm with intramural thrombus and a pseudoaneurysm of the left common iliac artery.
Figure 2. Postoperative picture of the patient showing the surgical approaches used for the first and the second stage of the procedure.
The reported incidence of aortic aneurysm in Takayasu arteritis or pulseless disease is 10–48%.2 The most common site is the descending thoracic and thoraco-abdominal aorta. The involvement of ascending aorta, arch and infrarenal aorta are less frequent. Rupture of aortic aneurysm is recognised as a potentially fatal complication of Takayasu disease and the presence of an aneurysm is considered an unfavourable prognostic sign. Surgical intervention is necessary for large, complicated, symptomatic or for rapidly expanding aneurysm. Though corticosteroid therapy may have a role in management of obstructive lesions of Takayasu disease in case of aneurysmal lesions it increases the risk of rupture as it weakens the wall of the aneurysm.1,2 The inflammatory nature and variable pattern of involvement of the aorta by the disease makes the arterial reconstruction surgery complex and demanding with frequent graft occlusions, significant operative mortality and postoperative haemorrhage and anastomotic aneurysm.1,3 Surgical technique includes prosthetic graft replacement with anastomotic sites away from areas of active aortitis or inactive areas to which the inflammatory process may progress, reinforcement of suture lines to reduce the risk of anastomotic aneurysm formation and creation of a ‘ventral aorta,’ a bypass from the ascending aorta down to the aortic bifurcation, and revascularisation of both kidneys.3,4 However, based on our experience in this case, we support Kieffer’s view that the upper and lower limit of the aneurysm is well defined and the anastomoses can be made in a macroscopically healthy zone.2 There are reports of surgical repair for aneurysm of ascending aorta, arch of aorta, descending thoracic aorta and thoracoabdominal in Takayasu disease, but there is not a single case reported of total aorta replacement as in our patient.1–4
Conclusions We report a case of successful total aortic replacement from ascending aorta to aortic bifurcation with separate revascularisation of visceral arteries and repair of pseudoaneurysm of left common iliac artery with no false aneurysm formation at the anastomotic sites at four months after surgery on CT angiography.
References
Figure 3. Postoperative CT angiography showing a good functional graft with adequate perfusion of all the visceral vessels.
1. Kieffer E, Chiche L, Bertal A, Koskas F, Bahnim A, Bletry O, Cacoub P, Prette JC, Thomas D. Descending Thoracic and Thoracoabdominal Aortic Aneurysm in patients with Takayasu disease. Ann Vasc Surg 2004;18:505–13.
CASE REPORT
Heart, Lung and Circulation 2007;16:382–384
384
Gupta et al. Total aorta replacement in Takayasu arteritis
CASE REPORT
2. Weaver FA, Yellin AE, Campen DH. Surgical procedures in the management of Takayasu arteritis. J Vasc Surg 1990;12: 429–39. 3. Iwai T, Inoue Y, Matsukura I, Sugano N, Numano F. Surgical technique for management of Takayasu’s arteritis. Int J Cardiol 2000;75:S135–40.
Heart, Lung and Circulation 2007;16:382–384
4. Leitao MJ, da Gama AD. Surgical management of Takayasu’s arteritis with extensive vascular involvement and bilateral renal artery stenosis. Rev Port Cir Cardiotorac Vasc 2004;11:35–9 [Article in Portuguese].
Case Report
Total Aorta Replacement in Takayasu Arteritis Presenting as Mega Aorta Manju Gupta, MCh ∗ , Anil Bhan, MCh, Sandeep Khanzode, MS, Shambhu Das, MD and Ruma Ray, MD Departments of Cardiothoracic & Vascular Surgery, Cardiac Anaesthesia & Cardiac Pathology, All India Institute of Medical Sciences, New Delhi, India
We report a unique case of successful two-stage total aortic replacement from ascending aorta to aortic bifurcation that was done for extensive aneurysmal involvement of the aorta in Takayasu arteritis. (Heart, Lung and Circulation 2007;16:382–384) © 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved. Keywords. Aortic aneurysm; Takayasu arteritis
Introduction
T
akayasu’s arteritis is an inflammatory arteriopathy that progresses to obliteration of multiple large arteries and the aneurysmal dilatation of the aorta is a rare occurrence.1 To the best of our knowledge, total aortic replacement for aneurysm of aorta from ascending aorta to aortic bifurcation in Takayasu disease has not been reported in the English literature. We report a unique case of successful two-stage total aortic replacement from ascending aorta to aortic bifurcation.
Case Report A 25-year-old male presented with history of abdominal pain and low-grade fever. On clinical examination there was a lump in left lower abdomen and monoparesis of left lower limb with fixed flexion deformity involving the left hip joint. Laboratory data showed the erythrocyte sedimentation rate as 54 mm/h. Ultrasound abdomen exhibited an abdominal aortic aneurysm. Chest X-ray revealed widening of mediastinum suggestive of aneurysm of ascending arch and descending thoracic aorta. Echocardiography showed an aneurysm of ascending arch and descending aorta with trivial aortic regurgitation. Contrast enhanced computed tomography scan of the chest and abdomen confirmed the presence of an extensive aortic aneurysm with maximum dilatation of 6–10 cm. The aortic wall was diffusely thickened with an intramural thrombus in the descending thoracic aorta with compression and displacement of adjacent Available online 6 March 2007 ∗
Corresponding author. Tel.: +919868301060. E-mail address: [email protected] (M. Gupta).
structures in the thorax and abdomen and no involvement of origin of great vessels. CT angiography revealed extensive aortic aneurysm with intramural thrombus and a pseudoaneurysm of the left common iliac artery (Fig. 1). In the first stage, the supra-coronary ascending aortic replacement, hemiarch replacement and an elephant trunk procedure was done from midsternotomy approach under deep hypothermic circulatory arrest with retrograde cerebral perfusion. The arterial inflow was through transaxillary route with bicaval venous drainage for cardiopulmonary bypass circuit. Two weeks later, completion of elephant trunk procedure along with replacement of total aorta upto aortic bifurcation was done through left thoracoabdominal approach. The celiac trunk, superior mesenteric and right renal arteries were implanted on the aortic graft as a patch. The left renal artery was accidentally transected requiring a saphenous vein graft for reconstruction. The pseudoaneurysm of the left common iliac artery was repaired using a patch. The postoperative period was uneventful and the patient was discharged on fortieth postoperative day. At four-month follow-up, there was relief of abdominal and lower limb pain with palpable lower limb pulses. There was improvement in flexion deformity and monoparesis with physiotherapy. The CT angiography revealed a good functional graft with adequate perfusion of all the visceral vessels (Figs. 2 and 3).
Histology On the gross appearance, the aortic wall was irregularly thick (thickness 3–8 mm) with glossy myxoid appearance of the intima. Microscopic examination revealed panaortitis with thickening and neomyointimal prolif-
© 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.
1443-9506/04/$30.00 doi:10.1016/j.hlc.2006.11.009
Gupta et al. Total aorta replacement in Takayasu arteritis
383
eration of the intima, widespread destruction with loss of elastic tissue of the media with focal giant-cell rich granulomatous reaction and adventitial fibrosis with thickening of the vasa vasora suggestive of Takayasu arteritis.
Comment
Figure 1. Preoperative CT angiography revealed extensive aortic aneurysm with intramural thrombus and a pseudoaneurysm of the left common iliac artery.
Figure 2. Postoperative picture of the patient showing the surgical approaches used for the first and the second stage of the procedure.
The reported incidence of aortic aneurysm in Takayasu arteritis or pulseless disease is 10–48%.2 The most common site is the descending thoracic and thoraco-abdominal aorta. The involvement of ascending aorta, arch and infrarenal aorta are less frequent. Rupture of aortic aneurysm is recognised as a potentially fatal complication of Takayasu disease and the presence of an aneurysm is considered an unfavourable prognostic sign. Surgical intervention is necessary for large, complicated, symptomatic or for rapidly expanding aneurysm. Though corticosteroid therapy may have a role in management of obstructive lesions of Takayasu disease in case of aneurysmal lesions it increases the risk of rupture as it weakens the wall of the aneurysm.1,2 The inflammatory nature and variable pattern of involvement of the aorta by the disease makes the arterial reconstruction surgery complex and demanding with frequent graft occlusions, significant operative mortality and postoperative haemorrhage and anastomotic aneurysm.1,3 Surgical technique includes prosthetic graft replacement with anastomotic sites away from areas of active aortitis or inactive areas to which the inflammatory process may progress, reinforcement of suture lines to reduce the risk of anastomotic aneurysm formation and creation of a ‘ventral aorta,’ a bypass from the ascending aorta down to the aortic bifurcation, and revascularisation of both kidneys.3,4 However, based on our experience in this case, we support Kieffer’s view that the upper and lower limit of the aneurysm is well defined and the anastomoses can be made in a macroscopically healthy zone.2 There are reports of surgical repair for aneurysm of ascending aorta, arch of aorta, descending thoracic aorta and thoracoabdominal in Takayasu disease, but there is not a single case reported of total aorta replacement as in our patient.1–4
Conclusions We report a case of successful total aortic replacement from ascending aorta to aortic bifurcation with separate revascularisation of visceral arteries and repair of pseudoaneurysm of left common iliac artery with no false aneurysm formation at the anastomotic sites at four months after surgery on CT angiography.
References
Figure 3. Postoperative CT angiography showing a good functional graft with adequate perfusion of all the visceral vessels.
1. Kieffer E, Chiche L, Bertal A, Koskas F, Bahnim A, Bletry O, Cacoub P, Prette JC, Thomas D. Descending Thoracic and Thoracoabdominal Aortic Aneurysm in patients with Takayasu disease. Ann Vasc Surg 2004;18:505–13.
CASE REPORT
Heart, Lung and Circulation 2007;16:382–384
384
Gupta et al. Total aorta replacement in Takayasu arteritis
CASE REPORT
2. Weaver FA, Yellin AE, Campen DH. Surgical procedures in the management of Takayasu arteritis. J Vasc Surg 1990;12: 429–39. 3. Iwai T, Inoue Y, Matsukura I, Sugano N, Numano F. Surgical technique for management of Takayasu’s arteritis. Int J Cardiol 2000;75:S135–40.
Heart, Lung and Circulation 2007;16:382–384
4. Leitao MJ, da Gama AD. Surgical management of Takayasu’s arteritis with extensive vascular involvement and bilateral renal artery stenosis. Rev Port Cir Cardiotorac Vasc 2004;11:35–9 [Article in Portuguese].