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Department of Radiology
Seth G.S. Medical College and K.E.M. Hospital, Mumbai , India
Interventional Case Record
Interventional Case Record
Percutaneous transluminal angioplasty of the brachiocephalic artery in a patient with Takayasu’s arteritis.
Percutaneous transluminal angioplasty of the brachiocephalic artery in a patient with Takayasu’s arteritis.
Contributed by : Nikhil Parvatkar
Contributed by : Nikhil Parvatkar
Introduction:
Introduction:
Aortoarteritis is recognized as a non-specific inflammation of the aorta and its main branches, and is frequently associated with reactive intimal proliferation.(1) Aortoarteritis leads to stenosis, occlusion, dilatation and aneurysm formation of the affected vessels. This disease is also known as Aortitis syndrome, Takayasu’s arteritis, Middle aortic syndrome, pulseless disease, atypical coarctation of aorta, and aortic arch syndrome. It most commonly affects young population in the Orient although it occurs worldwide. (2) It mainly affects young adults in the second and third decades of life. The reported female : male ratio has varied from 9:1 to 1.3:1. (3)
Aortoarteritis is recognized as a non-specific inflammation of the aorta and its main branches, and is frequently associated with reactive intimal proliferation.(1) Aortoarteritis leads to stenosis, occlusion, dilatation and aneurysm formation of the affected vessels. This disease is also known as Aortitis syndrome, Takayasu’s arteritis, Middle aortic syndrome, pulseless disease, atypical coarctation of aorta, and aortic arch syndrome. It most commonly affects young population in the Orient although it occurs worldwide. (2) It mainly affects young adults in the second and third decades of life. The reported female : male ratio has varied from 9:1 to 1.3:1. (3)
Here we report on a young woman with Takayasu’s arteritis with complete occlusion of the left common carotid artery (CCA) and the left subclavian artery and severe (upto 80 %) stenosis of the brachiocephalic artery (BCA), managed successfully by balloon angioplasty of the BCA.
Here we report on a young woman with Takayasu’s arteritis with complete occlusion of the left common carotid artery (CCA) and the left subclavian artery and severe (upto 80 %) stenosis of the brachiocephalic artery (BCA), managed successfully by balloon angioplasty of the BCA.
Case Presentation:
Case Presentation:
A 28 year old married woman, presented with vertigo, tremors , blurring of vision, dyspnoea on exertion , presyncope and syncopal attacks since six months. She had complete loss of vision in her left eye since three months, and blurry vision in the right eye since one month. She is known to have of Takayasu’s arteritis since three years. On examination there was a significant difference in upper and lower limb blood pressures. The right upper limb was : 72/46 mm Hg, the left upper limb was not recordable. The right lower limb was 128/91 mm Hg, the left lower limb : 159/100 mm Hg.
A 28 year old married woman, presented with vertigo, tremors , blurring of vision, dyspnoea on exertion , presyncope and syncopal attacks since six months. She had complete loss of vision in her left eye since three months, and blurry vision in the right eye since one month. She is known to have of Takayasu’s arteritis since three years. On examination there was a significant difference in upper and lower limb blood pressures. The right upper limb was : 72/46 mm Hg, the left upper limb was not recordable. The right lower limb was 128/91 mm Hg, the left lower limb : 159/100 mm Hg.
On examination the patient had tachypnea . Her lab investigations were as follows Hb- 8.7 gm% , Platelets- 2.8 lakhs, total leukocyte count - 7,600 per microliter, prothrombin time and INR- 15/1.1, S.Creat : 1.0. ESR : 30 mm (raised, normal 0-15).
On examination the patient had tachypnea . Her lab investigations were as follows Hb- 8.7 gm% , Platelets- 2.8 lakhs, total leukocyte count - 7,600 per microliter, prothrombin time and INR- 15/1.1, S.Creat : 1.0. ESR : 30 mm (raised, normal 0-15).
Medical management with Tab . Methotrexate 5 mg OD, Tab Folic acid 5mg OD, Tab Metoprolol 50 mg OD, Tab Wysolone 10 mg OD, (Tab Aspirin 75 mg + Tab Atorvastatin 10 mg + Tab Clopidogrel 75 mg) OD at present.
Medical management with Tab . Methotrexate 5 mg OD, Tab Folic acid 5mg OD, Tab Metoprolol 50 mg OD, Tab Wysolone 10 mg OD, (Tab Aspirin 75 mg + Tab Atorvastatin 10 mg + Tab Clopidogrel 75 mg) OD at present.
On Coronary angiography, there was chronic thrombotic occlusion (CTO) of the left main coronary artery, and 60 % ostial stenosis in the dominant right coronary artery.
On Coronary angiography, there was chronic thrombotic occlusion (CTO) of the left main coronary artery, and 60 % ostial stenosis in the dominant right coronary artery.
CT Aortogram : There is complete occlusion of left subclavian artery, and left common carotid artery. The left vertebral artery shows reformation possibly from the left internal mammary artery (LIMA). There is severe stenosis (80%) of the brachiocephalic artery. (Fig. 1);
CT Aortogram : There is complete occlusion of left subclavian artery, and left common carotid artery. The left vertebral artery shows reformation possibly from the left internal mammary artery (LIMA). There is severe stenosis (80%) of the brachiocephalic artery. (Fig. 1);
Fig. 1: : CT Aortogram showing severe stenosis (upto 80%) of the brachiocephalic artery.
severe ( > 90 %) stenosis of the right common carotid artery. The right subclavian artery is normal in calibre. There is moderate wall thickening in the aortic arch and descending thoracic aorta till D10 level. (Fig. 2) . The ascending aorta and abdominal aorta are normal. The left Axillary and brachial arteries reformed via collaterals. The abdominal arteries are normal. (Fig. 3, Fig, 4)
severe ( > 90 %) stenosis of the right common carotid artery. The right subclavian artery is normal in calibre. There is moderate wall thickening in the aortic arch and descending thoracic aorta till D10 level. (Fig. 2) . The ascending aorta and abdominal aorta are normal. The left Axillary and brachial arteries reformed via collaterals. The abdominal arteries are normal. (Fig. 3, Fig, 4)
FIG 2 : Moderate wall thickening in the aortic arch and descending thoracic aorta till D10 level.
FIG 3 : MIP images of celiac artery showing normal calibre.
FIG 4 : MIP images of both renal arteries showing normal calibre.
A DSA confirmed the above findings, of complete occlusion of left subclavian and left common carotid arteries, with severe stenosis of brachiocephalic artery (Fig. 5, Video 1) and the right common carotid artery. The right vertebral artery was normal in calibre and was the only major dominant vessel for intracranial supply.
A DSA confirmed the above findings, of complete occlusion of left subclavian and left common carotid arteries, with severe stenosis of brachiocephalic artery (Fig. 5, Video 1) and the right common carotid artery. The right vertebral artery was normal in calibre and was the only major dominant vessel for intracranial supply.
VID 1.mp4Fig. 5: DSA image showing severe stenosis of the right brachiocephalic artery.
Video 1 Angiogram from aortic arch showing severe stenosis at right brachiocephalic artery. Complete occlusion of left common carotid artery and left subclavian artery.
MRI of the brain (not shown) revealed chronic lacunar infarcts with small vessel ischemic changes in both cerebral hemispheres.
MRI of the brain (not shown) revealed chronic lacunar infarcts with small vessel ischemic changes in both cerebral hemispheres.
On detailed evaluation a right brachiocephalic balloon angioplasty was planned. However there was a risk of on table stroke and adverse neurological events,(syncopal attack during balloon inflation) as the brachiocephalic artery was the only dominant artery supplying the intracranial circulation.
On detailed evaluation a right brachiocephalic balloon angioplasty was planned. However there was a risk of on table stroke and adverse neurological events,(syncopal attack during balloon inflation) as the brachiocephalic artery was the only dominant artery supplying the intracranial circulation.
Interventional technique :
Interventional technique :
A 6 Fr short sheath access obtained into right common femoral artery. A 4 Fr pigtail catheter was advanced over Terumo wire into the arch of aorta and aortogram obtained. This showed severe (upto 80 %) short segment stenosis of the brachiocephalic artery with complete occlusion of left common carotid and left subclavian artery.The right subclavian artery was normal in course and calibre.
A 6 Fr short sheath access obtained into right common femoral artery. A 4 Fr pigtail catheter was advanced over Terumo wire into the arch of aorta and aortogram obtained. This showed severe (upto 80 %) short segment stenosis of the brachiocephalic artery with complete occlusion of left common carotid and left subclavian artery.The right subclavian artery was normal in course and calibre.
A 260 cm Extra stiff guide wire was introduced and the short sheath and was exchanged for a 6 Fr x 90 cm long Shuttle sheath. This sheath was placed in the arch of aorta just proximal to the origin of the brachiocephalic artery.
A 260 cm Extra stiff guide wire was introduced and the short sheath and was exchanged for a 6 Fr x 90 cm long Shuttle sheath. This sheath was placed in the arch of aorta just proximal to the origin of the brachiocephalic artery.
A 0.18 “ guidewire (V 18) was used to navigate the stenosis in the right brachiocephalic artery and was advanced into the right subclavian artery. (VID 2)
A 0.18 “ guidewire (V 18) was used to navigate the stenosis in the right brachiocephalic artery and was advanced into the right subclavian artery. (VID 2)
VID 2.mp4Video 2 0.18” (V 18) wire navigated across the stenosis into the right subclavian artery.
6 mm x 40 mm followed by 8 mm x 40 mm balloons were navigated across the stenosis and an angioplasty was performed (Fig. 6, Videos 3,4) . The balloons were inflated upto nominal pressure and immediately deflated. There was no on-table neurodeficit.
6 mm x 40 mm followed by 8 mm x 40 mm balloons were navigated across the stenosis and an angioplasty was performed (Fig. 6, Videos 3,4) . The balloons were inflated upto nominal pressure and immediately deflated. There was no on-table neurodeficit.
Fig. 6: Balloon inflated across the stenosis.
VID 3.mp4VID 4.mp4VID 3 : 6 mm X 40 mm balloon advanced across the stenosis in the brachiocephalic artery.
VID 4 : 8 mm X 40 mm balloon balloon inflated across the stenosis in the brachiocephalic artery.
The post angioplasty arteriogram showed good luminal gain in the brachiocephalic artery. The final angiogram showed significant resolution of stenosis with adequate luminal gain with good antegrade flow in distal circulation. (Fig. 7, Video 5)
The post angioplasty arteriogram showed good luminal gain in the brachiocephalic artery. The final angiogram showed significant resolution of stenosis with adequate luminal gain with good antegrade flow in distal circulation. (Fig. 7, Video 5)
VID 5.mp4Fig 7 Post angioplasty adequate luminal gain in brachiocephalic artery.
Video 5 Post angioplasty angiogram shows adequate luminal gain in the brachiocephalic artery and good flow across the right vertebral and subclavian artery. The narrowed calibre of the right common carotid artery is also seen.
Discussion:
Discussion:
Takayasu’s arteritis (TA) was first described by Mikito Takayasu in 1908 and is called nonspecific aortoarteritis (NSAA), aortic arch syndrome, and pulseless disease, among others.
Takayasu’s arteritis (TA) was first described by Mikito Takayasu in 1908 and is called nonspecific aortoarteritis (NSAA), aortic arch syndrome, and pulseless disease, among others.
It is an uncommon idiopathic, chronic disease with cell-mediated inflammation involving the aorta, its major branches, and the pulmonary arteries. (4)
It is an uncommon idiopathic, chronic disease with cell-mediated inflammation involving the aorta, its major branches, and the pulmonary arteries. (4)
Demographically, oriental population is most commonly affected by the disease. Dilative and aneurysmal lesions predominate in Philippines, Thailand, Israel, Japan, and Western India, whereas stenotic lesions are more commonly seen in North India and Japan. (5) Sites of involvement by steno-occlusive lesions also show geo graphic predilections. It has been reported that the aortic arch and its branches were mainly involved in Japanese patients whereas the abdominal aorta and its branches were mainly involved in Indian patients.
Demographically, oriental population is most commonly affected by the disease. Dilative and aneurysmal lesions predominate in Philippines, Thailand, Israel, Japan, and Western India, whereas stenotic lesions are more commonly seen in North India and Japan. (5) Sites of involvement by steno-occlusive lesions also show geo graphic predilections. It has been reported that the aortic arch and its branches were mainly involved in Japanese patients whereas the abdominal aorta and its branches were mainly involved in Indian patients.
None of the diagnostic criteria is entirely satisfactory, but the clinical diagnosis is seldom difficult. Essentially, the diagnosis depends on the typical angiographic morphology, history or presence of constitutional symptoms suggestive of a systemic illness, and the differential diagnosis from other, similar conditions such as other causes of inflammatory aortitis (e.g., syphilis, tuberculosis, giant cell arteritis, Buerger’s, Behc¸et’s, Cogan and Kawasaki diseases, spondyloarthropathies) and developmental abnormalities (e.g., Ehlers–Danlos syndrome, Marfan’s syndrome).
None of the diagnostic criteria is entirely satisfactory, but the clinical diagnosis is seldom difficult. Essentially, the diagnosis depends on the typical angiographic morphology, history or presence of constitutional symptoms suggestive of a systemic illness, and the differential diagnosis from other, similar conditions such as other causes of inflammatory aortitis (e.g., syphilis, tuberculosis, giant cell arteritis, Buerger’s, Behc¸et’s, Cogan and Kawasaki diseases, spondyloarthropathies) and developmental abnormalities (e.g., Ehlers–Danlos syndrome, Marfan’s syndrome).
The criteria for the diagnosis of Takayasu’s arteritis as suggested by Ishikawa are shown in Table 1.
The criteria for the diagnosis of Takayasu’s arteritis as suggested by Ishikawa are shown in Table 1.
The management strategies of TA include medical therapy with steroids or immunosuppressive agents and revascularization procedure (6,7). During the active phase of the disease, steroids have been shown to improve the systemic inflammatory symptoms within a few days to weeks. Cytotoxic drugs- methotrexate or cyclophosphamide, have been used when steroids could not induce remission (7). In the chronic stage, the principle of treatment is revascularization of the affected organ either by surgery or balloon angioplasty (8). Surgical treatment has been used to bypass the stenosed segment. However, diffuse, proximal, and multifocal involvement of the arch vessels in this disease makes surgical revascularization difficult. Surgical repair of stenoocclusive lesions at the origin of arch vessels is much more complex because it requires an intrathoracic approach, as opposed to endarterectomy of cervical carotid bifurcation stenosis in atherosclerosis and is often complicated by graft reocclusion, anastomotic site aneurysm, and morbidity. Compared to surgical repair, PTA can offer a less invasive, cost effective, and safe therapeutic option for relief of stenotic lesions in patients with TA.
The management strategies of TA include medical therapy with steroids or immunosuppressive agents and revascularization procedure (6,7). During the active phase of the disease, steroids have been shown to improve the systemic inflammatory symptoms within a few days to weeks. Cytotoxic drugs- methotrexate or cyclophosphamide, have been used when steroids could not induce remission (7). In the chronic stage, the principle of treatment is revascularization of the affected organ either by surgery or balloon angioplasty (8). Surgical treatment has been used to bypass the stenosed segment. However, diffuse, proximal, and multifocal involvement of the arch vessels in this disease makes surgical revascularization difficult. Surgical repair of stenoocclusive lesions at the origin of arch vessels is much more complex because it requires an intrathoracic approach, as opposed to endarterectomy of cervical carotid bifurcation stenosis in atherosclerosis and is often complicated by graft reocclusion, anastomotic site aneurysm, and morbidity. Compared to surgical repair, PTA can offer a less invasive, cost effective, and safe therapeutic option for relief of stenotic lesions in patients with TA.
Various studies have been performed in which percutaneous interventions for neurological manifestations in Takayasu’s arteritis have benefited the patients significantly in the long term. One such study by Hyun et al (9), suggested that neurological manifestations improved post procedure with no restenosis over a follow up period of 9 months to 7 years.
Various studies have been performed in which percutaneous interventions for neurological manifestations in Takayasu’s arteritis have benefited the patients significantly in the long term. One such study by Hyun et al (9), suggested that neurological manifestations improved post procedure with no restenosis over a follow up period of 9 months to 7 years.
In a meta analyses of PTA in atherosclerotic lesions, restenosis rates seemed relatively lower than in TA: 17% after renal artery stenting and 3% after stenting in subclavian arteries(10). Thus, our approach included strict control of active disease with the administration of immunosuppressive agents before and after the endovascular treatment. The intensity and duration of the immunosuppressive regimen were determined according to each patient’s clinical condition and disease activity. Prednisolone was given to all patients with elevated ESR before endovascular treatment; methotrexate was added if a patient was unresponsive to steroids. This protocol was successful in lowering the ESR before intervention in our study. Sharma et al. recommended that patients in emergency basis should be administered steroids prior to endovascular procedure and continued or a prolonged period thereafter. (11)
In a meta analyses of PTA in atherosclerotic lesions, restenosis rates seemed relatively lower than in TA: 17% after renal artery stenting and 3% after stenting in subclavian arteries(10). Thus, our approach included strict control of active disease with the administration of immunosuppressive agents before and after the endovascular treatment. The intensity and duration of the immunosuppressive regimen were determined according to each patient’s clinical condition and disease activity. Prednisolone was given to all patients with elevated ESR before endovascular treatment; methotrexate was added if a patient was unresponsive to steroids. This protocol was successful in lowering the ESR before intervention in our study. Sharma et al. recommended that patients in emergency basis should be administered steroids prior to endovascular procedure and continued or a prolonged period thereafter. (11)
References:
References:
1. Liu YO. Radiology of aortoarteritis. Radiol Clin North Am 1985;23:671—88.
1. Liu YO. Radiology of aortoarteritis. Radiol Clin North Am 1985;23:671—88.
2. Sharma S, Rajani M, Talwar K. Angiographic morphology in nonspecific aortoarteritis (Takayasu’s arteritis): a study of 126 patients from North India. Cardiovasc Intervent Radiol 1992;15:160—5.
2. Sharma S, Rajani M, Talwar K. Angiographic morphology in nonspecific aortoarteritis (Takayasu’s arteritis): a study of 126 patients from North India. Cardiovasc Intervent Radiol 1992;15:160—5.
3. Sekiguchi M, Sazuki J. An overview of Takayasu arteritis. Heart Vessels 1992;7:6—10.
3. Sekiguchi M, Sazuki J. An overview of Takayasu arteritis. Heart Vessels 1992;7:6—10.
4. Numano F, Okawara M, Inomata H, Kobayashi Y. Takayasu’s arteritis. Lancet 2000;356(9234):1023–1025
4. Numano F, Okawara M, Inomata H, Kobayashi Y. Takayasu’s arteritis. Lancet 2000;356(9234):1023–1025
5. Domingo RT, Maramba TP, Torres LF, Wesolowski SA. Acquired aorto-arteritis. A worldwide vascular entity. Arch Surg 1967;95(5):780–790
5. Domingo RT, Maramba TP, Torres LF, Wesolowski SA. Acquired aorto-arteritis. A worldwide vascular entity. Arch Surg 1967;95(5):780–790
6. Hoffman GS. Treatment of resistant Takayasu’s arteritis. Rheum Dis Clin North Am 1995;21:73-80
6. Hoffman GS. Treatment of resistant Takayasu’s arteritis. Rheum Dis Clin North Am 1995;21:73-80
7. Ishikawa K. Effects of prednisolone therapy on arterial angiographic features in Takayasu’s disease. Am J Cardiol 1991;68:410-413
7. Ishikawa K. Effects of prednisolone therapy on arterial angiographic features in Takayasu’s disease. Am J Cardiol 1991;68:410-413
8. Tada Y, Sato O, Ohshima A, Miyata T, Shindo S. Surgical treatment of Takayasu arteritis. Heart Vessels Suppl 1992;7:159 167
8. Tada Y, Sato O, Ohshima A, Miyata T, Shindo S. Surgical treatment of Takayasu arteritis. Heart Vessels Suppl 1992;7:159 167
9. Percutaneous Transluminal Angioplasty for Brain Ischemia due to Takayasu's Arteritis. Neurointervention. 2009;4(2):94-100.
9. Percutaneous Transluminal Angioplasty for Brain Ischemia due to Takayasu's Arteritis. Neurointervention. 2009;4(2):94-100.
10. Hadjipetrou P, Cox S, Piemonte T, Eisenhauer A. Percutaneous revascularization of atherosclerotic obstruction of aortic arch vessels. J Am Coll Cardiol 1999;33:1238-1245
10. Hadjipetrou P, Cox S, Piemonte T, Eisenhauer A. Percutaneous revascularization of atherosclerotic obstruction of aortic arch vessels. J Am Coll Cardiol 1999;33:1238-1245
11. Sharma BK, Jain S, Bali HK, Jain A, Kumari S. A follow-up study of balloon angioplasty and de-novo stenting in Takayasu arteritis. Int J Cardiol 2000;75 Suppl 1:S147-152
11. Sharma BK, Jain S, Bali HK, Jain A, Kumari S. A follow-up study of balloon angioplasty and de-novo stenting in Takayasu arteritis. Int J Cardiol 2000;75 Suppl 1:S147-152
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