Successful combined operation for Takayasu's arteritis.

We report a case of Takayasu's arteritis complicated with aortic regurgitation and severe stenosis of the left coronary ostium and the innominate artery. Simultaneous surgical treatment of aortic valve replacement, coronary ostium endarterectomy, and aortosubclavian artery bypass using saphenous vein were performed. We discuss the choice of simultaneous operation and the option of surgical treatment for coronary ostium lesion due to Takayasu's arteritis.     

Successful coronary revascularization using the PAS-Port system in a patient with Takayasu's arteritis and Behcet's disease

Coronary artery disease is rare in patients with Takayasu's arteritis or Behcet's disease. We report the case of a patient with concomitant Takayasu's arteritis and Behcet's disease who had angina pectoris develop due to severe narrowing of the left main coronary artery. The patient underwent revascularization with saphenous vein grafts with the assistance of the PAS-Port Proximal Anastomosis System (Cardica, Inc, Redwood City, CA). In conclusion, the PAS-Port Proximal Anastomosis System seems to be a safe and effective method of facilitating revascularization, particularly when severe calcification of the ascending aorta precludes cross-clamping during off-pump coronary artery bypass grafting.     

Surgical management of Takayasu's arteritis with extensive vascular involvement and bilateral renal artery stenosis

Proximal renal artery stenosis, in association with stenoses and/or aneurysms of aorta and its branches, suggests a Takayasu's disease and is an important, treatable cause of hypertension in young people. A 29-year-old black woman presents with systodiastolic hypertension and suprasternal and abdominal bruits. An aortography disclosed multiple aneurysms and stenosis of the aorta and its main branches, and bilateral stenosis of renal arteries. The patient underwent an unusual and well-succeeded surgical procedure consisting in the creation of a "ventral aorta", a bypass from the ascending aorta down to the aortic bifurcation, and revascularization of both kidneys. Takayasu's disease frequently courses with extensive involvement of vasculature and bilateral renal stenosis. Renovascular hypertension is a major complication, contributing to the high mortality of this disease. Renal revascularization plays an important role in the modification of the natural history of Takayasu's disease and is essential for long-term survival and prevention of ischaemic renal failure.     

Successful surgical treatment of Takayasu's arteritis associated with pyoderma gangrenosum

Pyoderma gangrenosum is rarely associated with Takayasu's arteritis. We report the successful surgical treatment of a 34-year-old woman who was diagnosed with pyoderma gangrenosum associated with thoracic aortic aneurysm and dissection due to Takayasu's arteritis. She underwent graft replacement of the thoracic aortic aneurysm under cardiopulmonary bypass, with perioperative management using prednisolone and cyclosporine A. She has had no evidence of the development of skin lesions or the progression of Takayasu's arteritis.     

Successful treatment in a patient with Takayasu's arteritis and Marfan syndrome

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.     

Pathogenesis of Takayasu's arteritis.

Takayasu's arteritis is an inflammatory disease of the arteries that involves large vessels. The inflammatory lesions in Takayasu's arteritis originate in the vasa vasorum and are followed by cellular infiltration, mainly composed of T cells (gammadelta lymphocytes, cytotoxic T lymphocytes, T helper cells), but also of natural killer cells, dendritic cells, monocytes and granulocytes, invading the outer layer of the media and/or its neighbouring adventitia. At this stage, positive production of inflammatory cytokines and/or adhesion molecules around these areas is remarkable. According to data available--in Takayasu's arteritis--interleukin-6, interleukin-1 and RANTES are released in large amounts by infiltrating inflammatory cells within damaged tissue, as well as by circulating inflammatory cells, and very likely help maintain the aberrant immune activation, by promoting endothelial cells activation and by inducing lymphocyte infiltration. Although the nature of the antigen that triggers the auto-immune process is still unknown, the infiltrating T lymphocytes may recognize one or a few self-antigens processed and presented in association with HLA. A considerable percentage of patients with Takayasu's arteritis have immune complexes in sera; moreover, on peripheral blood lymphocyte, Fc receptors and antibodies reactive against human endothelial cells (AECA) have been detected. This observation raised the possibility that AECA might have a role in endothelial cell activation and expression of adhesion molecules, which will facilitate leukocyte traffic.     

Successful treatment of primary bilateral renal mucormycosis with bilateral nephrectomy

Bilateral invasive renal mucormycosis has previously been associated with a 100% mortality rate. We report a case of bilateral invasive renal mucormycosis in a patient treated with amphotericin B and bilateral nephrectomy who survived and is currently disease-free.     

Chronic renal failure due to Takayasu's arteritis: recovery of renal function after nine months of dialysis

A twelve year old boy presented with sudden onset of severe hypertension and oligo-anuria. A diagnosis of Takayasu's Arteritis was made by aortography which demonstrated irregular narrowing of the lumbar aorta and renal arteries. Severe renal insufficiency necessitated maintenance hemodialysis. Hyperreninemic hypertension was intractable despite aggressive dialysis and multiple drug therapy. Renal biopsy after eight months of dialysis showed preservation of glomerular architecture. After nine months GFR improved spontaneously to 32 ml/min/1.73 m2 despite no improvement in his hypertension. This case report emphasizes the remarkable ability of renal parenchyma to recover function after sustained ischemia.     

Successful revascularization of an occluded renal artery after prolonged anuria

Renal atherosclerosis and fibromuscular dysplasia are the most common causes of curable human renovascular hypertension and renal failure. Vascular reconstruction often preserves renal function, but renal failure is rarely reversed, especially after days of anuria. We report a case of a 23-year-old woman who as a child underwent a nephrectomy for congenital hydroureter and renal hypoplasia. She later experienced fibromuscular dysplasia of the remaining renal artery, which ultimately progressed to a complete occlusion and 31 days of total anuria. The patient was revascularized, and within 2 months renal function returned with a blood urea nitrogen and creatinine of 9.0 and 1.0 mg/dl, respectively. After a follow-up of 6 months the patient's blood pressure remained 120/80 to 130/80 mm Hg without administration of hypertension medication. In this report we emphasize that under selected circumstances a kidney can survive prolonged ischemia and that delayed revascularization may reestablish renal function.     

Atheroembolism during percutaneous renal artery revascularization

INTRODUCTION: Atheroembolization during renal artery angioplasty and stenting (RA-PTAS) has been postulated as a cause for the inferior renal function results observed when compared with those with surgical revascularization. To further characterize procedure-associated atheroembolism, we analyzed recovered atheroembolic debris and clinical data from patients undergoing RA-PTAS with distal embolic protection (DEP). METHODS: RA-PTAS procedures were performed with DEP using a commercially available temporary balloon occlusion and aspiration catheter system between July 2005 and December 2006. Following RA-PTAS but prior to deflation of the distal occlusion balloon, the static column of blood proximal to the balloon was aspirated and submitted for embolic particle analysis. Angiograms, demographics, and laboratory data were reviewed. Glomerular filtration rate (eGFR) was estimated before RA-PTAS and at 4 to 8 weeks postintervention using the abbreviated Modification of Diet in Renal Disease formula. Associations between clinical factors, captured particle counts, and changes in renal function were examined using univariate techniques and multiple linear regression. RESULTS: Twenty-eight RA-PTAS procedures were performed with DEP. Mean total number of embolic particles counted per procedure was 2033 +/- 1553 for particles 20-60 microm and 265 +/- 132 for particles >60 microm. Significant positive associations with quantity of captured particles 20 to 60 microm were observed for African American race (P = .002), predilation (P = .005), and stent diameter (P < .001); a significant negative association was observed for preoperative aspirin use (P =.016). Quantity of captured particles >60 microm was positively associated with ratio of stent to renal artery diameter (P =.009). Change in eGFR was positively associated with preoperative aspirin use (P = .006) and preoperative eGFR (P < .001), while a negative association was observed for captured particle counts >60 microm (P = .015). CONCLUSION: These results demonstrate the liberation of thousands of atheroembolic particles during RA-PTAS. Clinical, anatomic, and device-related factors may be predictive of procedural embolization, and increasing captured particle counts >60 microm were associated with inferior renal function results. Further investigation is warranted to establish relationships between atheroembolism, end organ functional impairment, and clinical responses.     

Quality of life of patients with Takayasu's arteritis

OBJECTIVE: Takayasu's arteritis (TA) is a chronic immune vasculitis that causes inflammation of the aorta and its branches and is clinically characterized by exacerbations and remissions. This study examined the quality of life (QoL) of patients with TA using the Medical Outcomes Study Short Form 36 (SF-36) Health Survey, a validated health related QoL questionnaire. METHODS: Questionnaires that included the SF-36 and demographic related variables were mailed to 392 patients enrolled in the Takayasu's Arteritis Research Association. Raw SF-36 scores, as well as Physical Health Summary (PHS) and Mental Health Summary (MHS) scores, were calculated according to standard protocols. Data were analyzed for predictors of superior QoL using univariate and stepwise logistic regression analysis. SF-36 scores were also compared with those of other chronic diseases associated with peripheral vascular disease (PVD) published in the literature. Results are reported as mean +/- standard error of the mean. RESULTS: A total of 158 patients (144 women, 14 men) with average age of 42.2 +/- 1.1 years responded to the questionnaire. Mean onset of symptoms occurred at 30.5 +/- 1.2 years, with a mean age at diagnosis of 34.7 +/- 1.2, and a median of four doctors were seen before diagnosis. The group underwent 299 TA-related surgical procedures (1.9 +/- 0.3), including coronary (38%), carotid (35%), upper extremity (30%), and lower extremity (26%) revascularization. PHS and MHS summary scores (39.2 +/- 1.0 and 44.5 +/- 1.0, respectively) were worse than mean scores for an age-matched healthy population as well as nationally reported scores for diabetes mellitus, hypertension, and coronary artery disease (all P < .0001). Multivariate predictors of better physical QoL were younger age (P = .003) and remission of the disease (P = .0002). The use of immunomodulating medications was associated with inferior physical QoL (P = .02). The sole predictor of better mental QoL was remission of disease (P = .002). CONCLUSION: TA is a rare disease with profound consequences on QoL. Scores for physical and mental health are worse compared with many other chronic diseases associated with PVD. Superior physical QoL is seen in younger patients, whereas inferior physical QoL is encountered in those who take immunomodulating medications. Because the only factor to influence positively both physical and mental QoL is disease remission, every effort should be directed to attenuate disease activity.     

The management of Takayasu's arteritis: personal experience

Takayasu's arteritis is a chronic vasculitis mainly involving the aorta and its main branches. Almost all patients have ischemic disorders of the involved vessels. Treatment is nowadays controversial. Relapses are frequent. In the present study, we elucidate the long-term outcomes of our patients with Takayasu's arteritis. Between November 1993 and October 2003, 10 patients with Takayasu's disease were treated. All patients underwent medical treatment (corticosteroid for 7 months, cyclophosphamide for 3 months, and methotrexate for 12 months). Two patients stopped treatment with methotrexate when renal failure occurred. Four patients underwent a surgical procedure. During treatment, erythrocyte sedimentation rate and C-reactive protein concentrations were determined as indexes of inflammatory activity and treatment responsiveness. Four out of eight patients (50%) who underwent the full treatment with methotrexate had no relapse of the disease. The other four patients (50%) had relapse of the disease but had a better response to new corticosteroid treatment. The two patients who stopped methotrexate treatment died from complications of Takayasu's arteritis. In our personal experience, long-term treatment with methotrexate demonstrated a certain efficacy in avoiding relapse, maintaining stability of results, and amplifying the effects of steroid in patients with relapse.     

Management of severe hypertension in childhood Takayasu's arteritis

Six children presented with severe hypertension caused by Takayasu's arteritis (TA), of whom four had bilateral renal artery narrowing and two coarctation syndrome. Two presented with hypertensive encephalopathy and four with congestive cardiac failure. All had a strongly positive skin reactions to purified protein derivative of mycobacterium tuberculosis. Bilateral renal arterial bypass grafts performed in two children resulted in prolonged normalization of their blood pressures, but the grafts clotted 12–18 months later. Primary renal autotransplantation was unsuccessful in two children, one with bilateral renal arterial narrowing and iliac vessel involvement and one with a long coarctation. Secondary renal autotransplantation was successful in a third child with localized aortitis. A successful aortic patch graft was performed in one child with coarctation of the aorta. Angiotensin-converting-enzyme inhibitors should be used with caution in treating the hypertension caused by TA, since bilateral renal arterial narrowing is common and their administration may result in renal insufficiency. The long-term prognosis is guarded in severely hypertensive children with extensive vascular disease due to TA.