Coronary ostial stenosis in Takayasu's arteritis

A patient with Takayasu's arteritis with left coronary ostial narrowing is presented. The dramatic clinical and pathologic findings are discussed in detail. Emphasis is placed on making the diagnosis as soon as possible, in order to expedite bypass surgery to prolong life.     

Coronary arterial stenosis and subclavian steal in Takayasu's arteritis

This report describes a young boy with previous myocardial infarction and hypertension. Demonstration of coronary arterial ostial stenosis and a variant of subclavian steal was documented by selective arteriography. Surgical resolution of the most important stenosis is descending aorta, permitted effective control of hypertension. It is considered that one could reach this arteritis through different etiologies, tuberculosis having a preponderant place, and that it is important in all cases to carry out coronary arteriography.     

Takayasu's arteritis

The cause of Takayasu's arteritis is still obscure. However, certain aspects of the disease have been greatly clarified over the past decade. The disease is worldwide in distribution, with no ethnic group being totally free from risk of the condition. The inflammatory and stenotic phases of the disease frequently coexist, and it is important that the condition be diagnosed before it has progressed to the phase of extensive, severe vascular occlusion. Corticosteroid therapy is effective in ameliorating both the more inflammatory aspects of the condition (fever, arthritis, myocarditis) and the hemodynamic problems of vascular stenoses, in some cases allowing return of previously absent pulses. The role of adjunctive cytotoxic therapy is less certain, although there is probably a place for it in cases characterized by corticosteroid resistance or severe consequences of hypercortisolism. Vascular reconstruction is safe and effective once the inflammatory phase of the disease has remitted or been controlled with drug therapy. Prognosis is related to specific disease complications associated with longer duration of disease. Therefore, treatment intervention may modify the natural history of the disease over the long term.     

Takayasu's arteritis with ostial and left main coronary artery stenosis

Takayasu's arteritis with coronary artery involvement is rare, and there is little published information on the subject. Coronary angiographic and histopathologic studies have revealed coronary artery lesions in 9% to 11% of cases. Coronary artery involvement consists mostly of stenosis or occlusion of the coronary ostia. We report the case of a 19-year-old woman who presented with crescendo angina. Upon investigation, we found that our patient had ostial and left main coronary arterial stenosis with left-dominant circulation; therefore, we decided that an arterial Y graft, performed on a beating heart, would provide better perfusion to the compromised myocardium than would a single graft to the left anterior descending artery. In addition, use of the Y graft obviated the need to perform a proximal anastomosis on an inflamed, edematous ascending aorta, and it conferred long-term graft patency of the internal mammary arteries. Timely coronary artery bypass grafting relieved our patient's angina, and in early follow-up she has shown good effort tolerance.     

Takayasu's arteritis: management of left main stem stenosis

Takayasu arteritis is a chronic vasculitis involving the aorta and its main branches, the pulmonary arteries, and the coronary tree, and needs to be considered in a young patient with angina, in particular when pulses are absent. This case illustrates the limitations of exercise testing in diagnosing the extent of coronary artery disease and the risks associated with coronary angiography in patients with inflammatory disease in the left main stem coronary artery. It also highlights the novel use of non-invasive scanning with positron emission tomography using 18-fluorodeoxyglucose in assessing remission from this disease. Revascularisation was performed with percutaneous transluminal coronary angioplasty and stenting as an emergency procedure, but treatment of the restenosis with directional atherectomy was based on a review of the available literature. The lymphocytic alveolitis seen in this patient has not been previously described in Takayasu's disease.     

Endoluminal stenting for unprotected left main stenosis in Takayasu's arteritis

We describe 2 patients with Takayasu's arteritis and left main coronary stenosis who were considered poor candidates for surgical revascularization. Elective left main coronary artery balloon angioplasty followed by endoluminal stenting was performed with excellent results. At 3-mo follow-up, one patient had evidence of in-stent restenosis, but the other remained asymptomatic. In selected patients with Takayasu's arteritis and left main coronary stenosis, elective endoluminal stenting can be used as a definitive procedure or as a bridge to surgical revascularization. Cathet. Cardiovasc. Diagn. 40:272–275, 1997. © 1997 Wiley-Liss, Inc.     

Pregnancy and Takayasu's arteritis

Thirteen patients with Takayasu's arteritis had 30 pregnancies between 1970 and 1982. The 11 pregnancies occurring before the disease became clinically evident were uneventful. Eleven patients had 19 pregnancies after manifestation of the disease. Four ended in abortion, four in cesarean section, and 11 in uneventful vaginal delivery at term. There was no major obstetric problem apart from hypertension, and there was no maternal death directly related to the pregnancy per se. All 15 babies were born without asphyxia and congenital abnormality. The nine with some evidence of intrauterine growth retardation could be predicted by a prognostic score taking into account the timing of therapy, the severity of the hypertension, and the extent of arterial involvement.     

Takayasu's arteritis and its therapy

Twenty patients with Takayasu's arteritis were followed prospectively for an average of 4.6 years. Sixteen patients with active inflammatory Takayasu's arteritis were treated with glucocorticosteroids; eight responded to therapy. Six patients had clinical or angiographic progression of their vasculitis on daily corticosteroid therapy. These patients were then given cyclophosphamide together with prednisone on alternate days. Four of these 6 patients had no progression of vascular lesions while receiving cyclophosphamide; two had progression of vascular lesions after 30 and 48 months of therapy. Vascular reconstructive surgery was successful in 7 patients who tolerated a total of 13 vascular surgical procedures without major complications. One bypass graft occluded after 13 months and was revised. With corticosteroid therapy, cytotoxic therapy, and surgery, no deaths due to Takayasu's arteritis or its treatment have occurred.     

Isolated pulmonary arterial stenosis caused by Takayasu's arteritis in an elderly male

Takayasu's arteritis has often been difficult to diagnose because of a lack of typical symptoms and other specific makers. We report here a case of Takayasu's arteritis in a 73-year-old man who was considered to exhibit isolated pulmonary artery involvement. Pulmonary hypertension and right heart failure and severe stenosis in the main trunk and left pulmonary artery were observed. There was nothing remarkable in his routine blood-sample tests other than increased CRP and ESR. There were neither infectious nor collagen diseases. Anti-cardiolipin antibody, Antiphospholipid Syndrome, PR3-ANCA and MPO-ANCA were negative. We diagnosed the patient as having Takayasu's arteritis based on chronic inflammation and the morphologic features of pulmonary artery lesion. However, other large vessels and the aorta were not involved. Treatment was started with glucocorticoids. The symptoms gradually improved, and pulmonary artery pressure estimated by echocardiography decreased along with inflammatiory markers. There were no remarkable changes in the stenotic lesions in the pulmonary artery but the flow limit in the left pulmonary artery was improved.     

Crohn's disease and Takayasu's arteritis

Takayasu's arteritis and inflammatory bowel disease are rarely found together in the reported literature. In particular, only one African-American patient with both Crohn's disease and Takayasu's arteritis has been reported. We present a case of an African-American patient previously diagnosed with Crohn's disease who subsequently developed Takayasu's arteritis and then presented with acute rectal bleeding. The differential diagnosis, diagnostic evaluation, and possible common pathophysiologic mechanism between the two diseases are discussed.