Medical Management and Revascularization In An Informative Case

1. Medical Management and Revascularization In An Informative Case Ami A. Shah, MD Disclosures: No Relevant Financial Relationships with Commercial Interests

2. Objectives Discuss current options for medical management Identify patients who could benefit from revascularization Evaluate merits and risks of surgical bypass versus angioplasty Recognize current limitations: low sample sizes, lack of RCT, and few studies evaluating drug eluting stents

3. Presentation of Illness 29-year-old Caucasian female transferred to JHH for occasional amaurosis fugax without permanent visual loss and fatigue. PMH significant for HTN and biopsy proven membranous nephropathy postpartum with stable renal function 2 years ago during her C-section, BP could be measured only in her lower extremities.�� Slow onset of fatigue Development of claudication in her upper extremities when she has to raise her arms above her head or comb her hair. Intermittent lightheadedness 

4. Initial Evaluation Early 6/06, shortness of breath Labs: hemoglobin 8 � 9, MCV 68, ESR of 111, and ferritin 75 � Admitted to an outside hospital for CHF Echo: EF 55%, mild MR, grade I diastolic dysfunction and moderate aortic regurgitation Transferred to another institution Chest CT: bilateral carotid artery stenosis and subclavian stenosis Angiogram: extensive occlusions of main branch vessels off aortic arch

5. Initial Management and Transfer Started on prednisone 20 mg TID, a statin, and ASA for likely TA. Diuresed; HTN managed with amlodipine, carvedilol, enalapril Transferred 6/14 to our institution due to unease taking care of a patient with Takayasu�s Felt a little bit better after starting prednisone but claudication symptoms persisted ANA negative, ANCA negative, creatinine 0.8 Team agreed with the diagnosis of Takayasu�s arteritis

6. Additional History Social History:��Works in a dentist�s office, lives in WV with 2 children and husband, no tobacco, alcohol, or illicits Family History:��Hypertrophic cardiomyopathy, CAD, diabetes and hypothyroidism.��No autoimmune diseases.

7. Physical Examination Temperature 36.6 Celsius, pulse 74, BP 168/89 measured in lower extremities, RR 18, O2 sat 98% RA�� Obese RRR, III/VI systolic ejection murmur at RUSB, I/IV diastolic murmur at RUSB, I/VI systolic murmur at the apex Carotid and subclavian bruits bilaterally Trace BLE edema No brachial or radial pulses bilaterally, 2 plus DP and PT pulses Normal CN and strength exam

9. Laboratory data K 5.6, BUN 51, creatinine 1.1 to 1.3 WBC 14.7 with 89% neutrophils, hematocrit 36.5, MCV 74.8, RDW 21, platelets 414,000 ESR 36, CRP < 0.3 pro-BNP 300 Urinalysis: no proteinuria and bland sediment Echocardiogram: EF 65%, trace MR and TR, moderate AR, and aortic root size upper limit of normal Chest x-ray: obesity, normal cardiac silhouette.

10. JHH Admission #1 (6/14-17/06) Management Pulsed with 1 gram of methylprednisolone IV daily for 3 days Fatigability and claudication symptoms improved Cardiology recommended Q6 month echocardiograms to monitor aortic root size Discharged home on prednisone 60 mg daily, methotrexate at 0.6 mL subcutaneously weekly [25mg/mL], folate, baby aspirin, her anti-hypertensive agents, a statin, levothyroxine, metformin, dapsone, Ca, and VitD with Vasculitis Center follow-up.

11. Readmission (7/15-22/06) At home: several episodes of syncope after laughing and continued blurry vision. Readmitted to an outside institution and transferred to JHH Working hypothesis: Likely sustained vessel damage from prior disease with extensive residual stenoses compromising cerebral perfusion pressure

12. MRI/MRA of chest and abdomen Ascending aorta at the pulmonary artery measured 3.8 cm Markedly narrowed: right and left common carotid arteries in the neck, right subclavian and axillary artery Left subclavian and axillary artery beyond the left vertebral markedly narrowed Right vertebral artery small but patent with surrounding increased T2 signal consistent with inflammation Mild narrowing of the celiac artery at its origin with mild post stenotic dilation. Patent SMA and no significant renal artery stenosis descending aorta at this level 2.6 cm descending aorta at the diaphragm 2.5 cm descending aorta at the level of renal arteries 1.7 cm Left subclavian patent with normal caliber at its origin and with a dominant and patent left vertebral artery. pulmonary arteries patent and normal.descending aorta at this level 2.6 cm descending aorta at the diaphragm 2.5 cm descending aorta at the level of renal arteries 1.7 cm Left subclavian patent with normal caliber at its origin and with a dominant and patent left vertebral artery. pulmonary arteries patent and normal.

13. MRI demonstration of carotid wall thickening and lumen narrowing and beading, especially on the left.

14. Mild narrowing of the celiac and SMA reported on MRI

15. Mild dilatation of the aorta but no aortic wall thickening in this image.

16. Right carotid artery thickening

17. Carotid duplex Left CCA occluded with no demonstrable flow. Left ICA and ECA not visible and presumably occluded. Small collateral vessels seen lateral to the expected location of the left CCA. Left subclavian and axillary arteries narrowed but not completely occluded. Right CCA markedly narrowed, >70% stenosis. Wall of the right CCA markedly thickened Right vertebral artery exhibits retrograde flow, suggestive of a steal phenomenon. right external carotid artery patent. right internal carotid artery difficult to visualize, but exhibits to-and-fro flow. visualized portions of the right subclavian artery patent. right external carotid artery patent. right internal carotid artery difficult to visualize, but exhibits to-and-fro flow. visualized portions of the right subclavian artery patent.

18. Cardiac evaluation and management Leg SBP as high as 240 Captopril and metoprolol doses titrated upward with a goal SBP of 160-180 Repeat echo unchanged Tried to obtain angiography and cardiac catheterization records from VA to obtain pressures measured proximal and distal to her stenoses

19. Ophthalmic Evaluation Ophthalmodynamometry: extremely poor retinal perfusion despite SBP 200s Fluoroscein angiography normal bilaterally

20. Vascular surgery Consulted given inactive disease detected on MRI/MRA. Good revascularization candidate but desired CTA of head, neck and chest which demonstrated similar findings to MRI/A. Left CCA displayed marked wall thickening and narrowing with the string sign which extends superiorly to approximately C3 level. Debated surgical bypass versus angioplasty +/- stent

21. Medical Management

22. Corticosteroids 60 patients followed prospectively 1970-1990 at NIH 48 patients treated with GC alone or with a cytotoxic agent Remission achieved in 60% treated with prednisone alone at doses of 1mg/kg/day for 1-3 mos; median time to remission 22 months >50% relapse during taper Development of new lesions at previously unaffected sites is common 25 patients required addition of cytotoxic agents 23% of all treated patients never achieved remission

23. Methotrexate Open-label pilot study of weekly low-dose MTX+GC Outcomes Measured: clinical characteristics, labs, angiographic findings, and ability to withdraw GC and MTX therapy Remission: no clinical or new angiographic signs of active disease Subjects: 18 patients entered; 2 dropped out, 16 followed for a mean period of 2.8 years (range 1.3-4.8 years) Methods: starting dose of methotrexate 15mg Qweek; doses increased up to 25mg/week to achieve remission Hoffman, G.S. et. al. Arthritis Rheum. 1994 Apr;37(4):578-82 Hoffman, G.S. et. al. Arthritis Rheum. 1994 Apr;37(4):578-82

24. Methotrexate Results Weekly administration of MTX (mean stable dose of 17.1 mg) and GC ? remissions in 81% 7 patients (44%) relapsed as GC tapered Retreatment led to remission. 3/7 successfully stopped GC Of those who achieved remission, 50% sustained remissions of 4-34 months (mean 18 months) 4 did not require GC or MTX for 7-18 months (mean 11.3 months) 3 patients had disease progression in spite of treatment . .

25. Azathioprine 1996-2001: 15 of 65 consecutive newly diagnosed patients with TA not previously treated by any immunosuppressive therapy had active disease Active disease (2 or more): constitutional features, painful arteries, elevated ESR, elevated CRP Treatment: Azathioprine 2mg/kg/day and prednisolone 1mg/kg/day for 6 weeks to be tapered to 5-10 mg/day by 12 weeks Angiograms before therapy and at one year follow-up

26. Azathioprine Results All had complete resolution of systemic symptoms ESR and CRP decreased significantly at 3 months No change in peripheral pulses or limb BPs No progression or regression of lesions in any patient No new lesions identified

27. Mycophenolate: Case Reports Italian group with 3 patients with refractory TA Mycophenolate mofetil 1gm po BID. Clinical evaluation and WBC done weekly. Vascular lesions assessed by Doppler ultrasonography All 3 showed clinical benefit, and 2 resumed work Able to taper off steroid

28. Cyclophosphamide 20 TA patients prospectively followed for average 4.6 yrs 16 with active TA treated with GC; 8 responded 7 had poor response to prednisone (1mg/kg daily) after 3 mos 6/7 patients had clinical or angiographic progression on GC All 7 given cyclophosphamide 2mg/kg/day plus prednisone tapered to alternate day dosing 4/6: no progression of vascular lesions while on cyclophosphamide 2/6: progression of vascular lesions after 30 and 48 mos of therapy

29. TNF Inhibitors 7: etanercept 25mg BIW 8: infliximab 3-5mg/kg IV @ 0,2, 8 weeks; then Q4-8 weeks 3 infliximab (2 drug availability, 1 relapse) Active or relapsing disease: considered to be present in the setting of new vascular lesions by MRI or catheter-directed angiography, at previously unaffected sites. OR At least 2 of the following 5 features were present: 1) new onset of carotodynia or pain over other large vessels, 2) TIAs that could not be attributed to other factors, 3) new bruit or new asymmetry in pulses or blood pressure, 4) fevers without infection, or 5) a reproducible increase in ESR Complete and sustained remission defined as the absence of features of active disease, the absence of new lesions on imaging studies, and no glucocorticoid therapy for at least 6 months. If the glucocorticoid dosage could be reduced by 50% before the disease recurred, then partial remission. 7: etanercept 25mg BIW 8: infliximab 3-5mg/kg IV @ 0,2, 8 weeks; then Q4-8 weeks 3 infliximab (2 drug availability, 1 relapse) Active or relapsing disease: considered to be present in the setting of new vascular lesions by MRI or catheter-directed angiography, at previously unaffected sites. OR At least 2 of the following 5 features were present: 1) new onset of carotodynia or pain over other large vessels, 2) TIAs that could not be attributed to other factors, 3) new bruit or new asymmetry in pulses or blood pressure, 4) fevers without infection, or 5) a reproducible increase in ESR Complete and sustained remission defined as the absence of features of active disease, the absence of new lesions on imaging studies, and no glucocorticoid therapy for at least 6 months. If the glucocorticoid dosage could be reduced by 50% before the disease recurred, then partial remission.

30. TNF Inhibitors 10 had complete remission for 1-3.3 yrs without GC. 4 had partial remission with >50% reduction in GC dose. At ~12 months followup, median dose of prednisone zero. Therapy failed in 1 patient. Repeated imaging revealed that 5 patients (2 CR, 2 PR, and 1 treatment failure) had new lesions over ~12 month followup In 9/14 responders, increased anti-TNF dosage required to sustain remission. 2 relapses when etanercept interrupted. Remission reestablished upon restarting etanercept.

31. Revascularization: Surgical Bypass Or Percutaneous Intervention?

32. Revascularization Diagnosis occurs when stenotic and occlusive lesions already exist Such lesions are not reversible by medical management Often hemodynamically significant

33. Indications for revascularization Cerebrovascular disease due to cervicocranial vessel stenosis Coronary artery disease Moderate-severe aortic regurgitation Severe coarctation of the aorta Renovascular hypertension Limb claudication Progressive aneurysm enlargement with risk of rupture or dissection

34. Surgical Bypass Largest body of experience from bypass graft procedures. Good long term outcomes. On average, 20-30% of restenosis or occlusion. 3 deaths total. One death due to graft infection.Largest body of experience from bypass graft procedures. Good long term outcomes. On average, 20-30% of restenosis or occlusion. 3 deaths total. One death due to graft infection.

35. Percutaneous Revascularization

36. 60 patients prospectively followed 68% had extensive vascular disease Stenotic lesions 3.6-fold more common than aneurysms (98% vs 27%). ESR not a consistently reliable marker of disease activity. Bypass biopsy specimens from clinically inactive patients showed histologically active disease in 44%. Clinically significant palliation after bypass Medical therapy required for 80%; 20% had monophasic self-limiting disease. Surgical Bypass: NIH Series 1970-1990 Most common clinical finding: bruit. Hypertension most often associated with renal artery stenosis. Only 33% had systemic symptoms on presentation. GC alone or with a cytotoxic agent failed to induce remission in 25% ~50% in remission later relapsed. Most common clinical finding: bruit. Hypertension most often associated with renal artery stenosis. Only 33% had systemic symptoms on presentation. GC alone or with a cytotoxic agent failed to induce remission in 25% ~50% in remission later relapsed.

37. Surgical 50 bypass procedures were done in 23 patients. Dacron was used for 25 bypass procedures. Autologous vessel grafts were most often selected for bypass of stenotic lesions of the renal and coronary arteries and, in only one patient, the subclavian artery. The saphenous vein was used in 10 of 11 autologous grafts. 30% of the bypass procedures were followed by complications: restenosis (24%), thrombosis (4%), hemorrhage (2%), and infection (2%). Complications occurred in 36% of synthetic grafts. Only 1 of 11 (9%) autologous grafts was followed by anastomotic stenosis. Nine patients had 12 bypass grafts placed for critical stenosis of carotid vessels and for prophylaxis against cerebrovascular accidents. The ascending aorta was the most common proximal anastomotic site. These 9 had a median follow-up of 44 months. Only 1 patient had a CVA since the procedure. Percutaneous 20 percutaneous transluminal angioplasty procedures were done in 11 patients. Most often done on subclavian and renal vessels. Only 56% of angioplasty procedures were successful on the first attempt, whereas only 33% succeeded on a second attempt. 3 patients eventually required a bypass procedure. 3 of 7 percutaneous transluminal angioplasty procedures that were done for recanalization of the renal arteries were successful, although in two cases a subsequent bypass procedure was required. 7 patients had percutaneous transluminal angioplasty or bypass graft placement (or both) for blood pressure control. Five of these 7 patients have improved blood pressure control. Three have discontinued all antihypertensive medications, and 2 require less medication. In patients with short, proximal stenotic lesions who require urgent relief, this procedure may be palliative. Surgical 50 bypass procedures were done in 23 patients. Dacron was used for 25 bypass procedures. Autologous vessel grafts were most often selected for bypass of stenotic lesions of the renal and coronary arteries and, in only one patient, the subclavian artery. The saphenous vein was used in 10 of 11 autologous grafts. 30% of the bypass procedures were followed by complications: restenosis (24%), thrombosis (4%), hemorrhage (2%), and infection (2%). Complications occurred in 36% of synthetic grafts. Only 1 of 11 (9%) autologous grafts was followed by anastomotic stenosis. Nine patients had 12 bypass grafts placed for critical stenosis of carotid vessels and for prophylaxis against cerebrovascular accidents. The ascending aorta was the most common proximal anastomotic site. These 9 had a median follow-up of 44 months. Only 1 patient had a CVA since the procedure. Percutaneous 20 percutaneous transluminal angioplasty procedures were done in 11 patients. Most often done on subclavian and renal vessels. Only 56% of angioplasty procedures were successful on the first attempt, whereas only 33% succeeded on a second attempt. 3 patients eventually required a bypass procedure. 3 of 7 percutaneous transluminal angioplasty procedures that were done for recanalization of the renal arteries were successful, although in two cases a subsequent bypass procedure was required. 7 patients had percutaneous transluminal angioplasty or bypass graft placement (or both) for blood pressure control. Five of these 7 patients have improved blood pressure control. Three have discontinued all antihypertensive medications, and 2 require less medication. In patients with short, proximal stenotic lesions who require urgent relief, this procedure may be palliative.

38. Surgical Bypass: NIH Series 1970-1990 50 bypass procedures in 23 patients Median follow-up 5.3 yrs 24% restenosis rate, unclear how many hemodynamically significant 36% of 39 procedures using synthetic grafts complicated by restenosis 9% of 11 procedures using autologous vessels associated with restenosis

39. Percutaneous Transluminal Angioplasty: NIH Series 1970-1990 20 PTA procedures done in 11 patients PTA procedures most often done on subclavian and renal vessels Only 56% of angioplasties successful on the 1st attempt Only 33% succeeded on a 2nd attempt Restenosis occurred within 3.5 to 13.6 months. 3 patients eventually required bypass Three of 7 percutaneous transluminal angioplasty procedures that were done for recanalization of the renal arteries were successful, although in two cases a subsequent bypass procedure was required. Seven patients had percutaneous transluminal angioplasty or bypass graft placement (or both) for blood pressure control. Five of these 7 patients have improved blood pressure control. Three have discontinued all antihypertensive medications, and 2 require less medication. Three of 7 percutaneous transluminal angioplasty procedures that were done for recanalization of the renal arteries were successful, although in two cases a subsequent bypass procedure was required. Seven patients had percutaneous transluminal angioplasty or bypass graft placement (or both) for blood pressure control. Five of these 7 patients have improved blood pressure control. Three have discontinued all antihypertensive medications, and 2 require less medication.

40. Cleveland Clinic 1979-2001 Retrospective chart review of 20 TA patients Primary outcome measure: patency of vessels by repeat invasive angiography or MRA Secondary outcome measures: periprocedural complications, morbidity, and mortality Interventions: bypass grafts, patch angioplasty, endarterectomy, percutaneous transluminal angioplasty (PTA), or stent placement.

41. Cleveland Clinic 1979-2001 35% of bypass grafts restenosed, occluded, or required another revascularization procedure. Represents a higher rate of restenosis than that found in atherosclerosis. For example, Fitzgibbon, et al describe the results of angiographic followup of 5065 coronary bypass grafts. Vein graft patency was 81% and 75% at one and 5 years, respectively. An 85�90% graft patency rate at 5 years can be expected following aortofemoral bypass grafting foratherosclerotic lesions; similarly, excellent results can be achieved following surgical revascularization for atherosclerotic renal artery disease. Why this apparent trend towards a higher graft failure rate in TA occurs is uncertain. Reports by Hall, et al10 and Pajari, et al3 suggest that overt or covert inflammation at the time of surgery is associated with a higher incidence of graft failure. In this article, all patients were considered to be in remission at the time vascular procedures were performed. No tissue samples from revascularized vessels available, but persistent inflammation at the site of graft anastomosis may have been responsible for at least some of the grafts later undergoing restenosis. In TA, chronically diseased vessels are typically fibrotic and often noncompliant, which may result in incomplete dilatation. It is known from trials in atherosclerosis that failure to achieve optimal dilatation is associated with a higher risk of restenosis. In addition, in TA, the noncompliant nature of the vessel wall often requires higher balloon inflation pressures and repeated inflation of the balloon may be required to obtain satisfactory results. This may expose the vessel wall to increased risk of injury. Persistent inflammation in the TA vessel at the time of dilatation/stenting may lead to enhanced myointimal proliferation. And finally, stenotic lesions in TA are characteristically long compared to the short and segmental lesions of atherosclerosis. Higher rates of restenosis correlate with the length of lesions in both atherosclerosis and TA. Conversely, best results of PTA in TA have been obtained in short, focal lesions. Liang�s results with percutaneous endovascular revascularization procedures, especially with stent implantation, differ from the positive outcomes reported by others. Different vessel characteristics (e.g., anatomic site, length of lesions, severity of lesions) may account for different outcomes. 35% of bypass grafts restenosed, occluded, or required another revascularization procedure. Represents a higher rate of restenosis than that found in atherosclerosis. For example, Fitzgibbon, et al describe the results of angiographic followup of 5065 coronary bypass grafts. Vein graft patency was 81% and 75% at one and 5 years, respectively. An 85�90% graft patency rate at 5 years can be expected following aortofemoral bypass grafting foratherosclerotic lesions; similarly, excellent results can be achieved following surgical revascularization for atherosclerotic renal artery disease. Why this apparent trend towards a higher graft failure rate in TA occurs is uncertain. Reports by Hall, et al10 and Pajari, et al3 suggest that overt or covert inflammation at the time of surgery is associated with a higher incidence of graft failure. In this article, all patients were considered to be in remission at the time vascular procedures were performed. No tissue samples from revascularized vessels available, but persistent inflammation at the site of graft anastomosis may have been responsible for at least some of the grafts later undergoing restenosis. In TA, chronically diseased vessels are typically fibrotic and often noncompliant, which may result in incomplete dilatation. It is known from trials in atherosclerosis that failure to achieve optimal dilatation is associated with a higher risk of restenosis. In addition, in TA, the noncompliant nature of the vessel wall often requires higher balloon inflation pressures and repeated inflation of the balloon may be required to obtain satisfactory results. This may expose the vessel wall to increased risk of injury. Persistent inflammation in the TA vessel at the time of dilatation/stenting may lead to enhanced myointimal proliferation. And finally, stenotic lesions in TA are characteristically long compared to the short and segmental lesions of atherosclerosis. Higher rates of restenosis correlate with the length of lesions in both atherosclerosis and TA. Conversely, best results of PTA in TA have been obtained in short, focal lesions. Liang�s results with percutaneous endovascular revascularization procedures, especially with stent implantation, differ from the positive outcomes reported by others. Different vessel characteristics (e.g., anatomic site, length of lesions, severity of lesions) may account for different outcomes.

42. 62 revascularization procedures in 20 patients. Followup available for 52 procedures. 11/31 bypass grafts restenosed between 1 day to 168 months after surgery 3/7 PTA restenosed after 1-72 mos 5/7 stents restenosed after 2-45 months No deaths CONCLUSION: Despite providing short term benefit, endovascular revascularization procedures associated with a high failure rate in TA. Cleveland Clinic 1979-2001

43. Renal Revascularization in TA-induced RAS 27 patients with TA-induced RAS underwent intervention Primary patency rates determined Late effects on BP, renal and cardiac function, survival analyzed All had HTN (mean BP, 167/99 mm Hg; 2.5 antihypertensive medications per patient). Mean estimated GFR in patients not receiving HD was 76 mL/min. 3 patients HD-dependent 2 had intractable congestive heart failure 40 interventions: 32 aortorenal bypass, 2 repeat implantations, 4 nephrectomies, 2 transluminal angioplasties Autologous grafts in 20, prosthetic materials in 12

44. Postoperative morbidity 19%. No deaths. 3 graft stenoses, all due to intimal hyperplasia; 2 revised successfully 3 graft occlusions At 1, 3, and 5 years of follow-up, primary patency was 87%, 79%, and 79%, respectively Decreased BP to a mean of 132/79 mm Hg (P<.0001) Need for antihypertensive medications reduced to 1/patient (P<.01). Mean GFR increased to 88 mL/min (P<.005) 2 patients no longer required HD. CHF resolved in both patients Renal Revascularization in TA-induced RAS

45. Surgical Outcomes 1955-1995 Retrospective review of 106 consecutive patients with TA who underwent surgical treatment Ages 5 to 69 years (mean+/-SEM, 31.7+/-1.3 years) 12 early hospital deaths, all in patients operated before 1981 Remaining 94 followed for a mean of 19.8 years 31/94 died; CHF cause of death in 45% Serious long-term complication: anastomotic aneurysm, cumulative incidence at 20 years of 13.8%. Overall cumulative survival rate at 20 years was 73.5%.

46. Surgical Outcomes 1955-1995 Patients classified according to Ishikawa prognostic criteria preop1, 15 year survival rate in Stage 3 patients was 82% Complications: retinopathy, severe HTN, grade 3 or 4 AR, aneurysms2 Ishikawa: Long-Term Outcome for 120 Japanese Patients With Takayasu's Disease: Clinical and Statistical Analyses of Related Prognostic Factors Ishikawa: Long-Term Outcome for 120 Japanese Patients With Takayasu's Disease: Clinical and Statistical Analyses of Related Prognostic Factors

47. Surgical Outcomes 1955-1995 Surgery seemed to increase the long-term survival of patients with stage 3 TA Conservative treatment recommended for stage 1 or 2 disease Anastomotic aneurysms occur at any time after surgery ? need lifetime serial imaging to detect early aneurysms.

48. Coronary involvement 1972-2001: 81/130 TA patients had selective coronary angiography; 31 had abnormal coronary angiographic findings 24 coronary artery stenoses > 75%, 3 coronary artery-bronchial artery anastomoses, 3 aneurysmal coronary ectasias Among stenoses, ostium most frequently involved (87.5%) 23/24 patients with stenoses treated surgically Mean follow-up 9.65 years, 100% follow-up rate 2 (8.7%) in-hospital deaths and 3 (13%) late deaths; patency > 85% Actuarial survival rate 86.5% +/- 7.3% at 5 years and 81.4% +/- 8.4% at 10 years 81 patients with Takayasu arteritis had selective coronary angiography because of chest pain, ST-T�segment changes on electrocardiography, cardiomegaly caused by atypical coarctation, aortic regurgitation, severe stenosis of systemic arteritis, and renovascular hypertension. Four fistulas and 4 aneurysms in 7 patients were not treated surgically. Coronary steal phenomenon was always associated with occluded pulmonary arteries and pulmonary hypertension. Aneurysmal coronary ectasia was related to severe aortic hypertension with or without aortic regurgitation and atypical coarctation.81 patients with Takayasu arteritis had selective coronary angiography because of chest pain, ST-T�segment changes on electrocardiography, cardiomegaly caused by atypical coarctation, aortic regurgitation, severe stenosis of systemic arteritis, and renovascular hypertension. Four fistulas and 4 aneurysms in 7 patients were not treated surgically. Coronary steal phenomenon was always associated with occluded pulmonary arteries and pulmonary hypertension. Aneurysmal coronary ectasia was related to severe aortic hypertension with or without aortic regurgitation and atypical coarctation.

49. Subclavian artery angioplasty 1986-1995 61 SC artery angioplasties done in 55 consecutive patients with aortoarteritis (n = 32) and atherosclerosis (n = 23) PTA for 56 stenotic lesions and 5 total occlusions PTA successful in 52 (92.8%) stenotic lesions and 3 (60%) total occlusions 3 patients (5.4%) had complications, managed nonsurgically

50. Subclavian artery angioplasty 1986-1995 Patients with aortoarteritis: younger female diffuse involvement Required higher balloon inflation pressures Had more residual stenosis Luminal diameter of stenoses were similar before PTA

51. Mean 43.3 mos follow-up of 40 patients Restenosis more often observed in aortoarteritis, particularly in those with diffuse arterial narrowing Lesions could be effectively redilated Clinical symptoms showed marked improvement after successful angioplasty. Subclavian artery angioplasty 1986-1995

52. Balloon angioplasty for renovascular HTN PTA of renal arteries performed in 54 consecutive patients with hypertension and TA-induced RAS Angioplasty successful in 67 (89.3%) of 75 lesions attempted. Degree of stenosis decreased from 88.3% to 23.5% (p < 0.001)

53. Improvement in HTN (p < 0.001) in 48 hrs After mean 26.4 mos follow-up, BP reduced to normal or improved in 93% Angiographic restudy an average of 14 mos after: restenosis at the same site in 7 of 52 (13.5%) lesions Balloon angioplasty for renovascular HTN

54. Who benefits from revascularization?

55. Summary of Bypass vs Angioplasty in TA Fibrotic, noncompliant vessels ? incomplete dilatation Need higher balloon inflation pressures and repeated inflation of the balloon Persistent inflammation at time of dilatation/stenting ? enhanced myointimal proliferation Stenotic lesions in TA long compared to the short, segmental lesions of atherosclerosis Bypass grafting has best long term patency rates Data with drug eluting stents needed

56. Back to our patient� Decision made to taper glucocorticoids over 2 months to 20mg daily and increase MTX to 17.5mg weekly Plan for surgical bypass to improve cerebral perfusion once at lower steroid dose Unable to taper beyond 30mg daily due to rising ESR

57. Operative Intervention 9/15/06: ascending aorta to left carotid bifurcation bypass with Dacron graft Left axillary artery explored for planned bypass to that vessel but thrombosed all the way out to the axilla Postoperative course: unable to extend left wrist and fingers possibly due to brachial plexus injury during exposure of distal left axillary artery Visual symptoms resolved on POD 2