Granulomatous coronary arteritis (Takayasu's disease)

The pathologic findings in two patients who died of nonspecific, granulomatous, coronary arteritis (Takayasu's disease) were studied. In one patient, the disease involved the aortic base, with extension into the aortic valve, coronary arteries, and interventricular septum, causing sudden death. Segmental involvement of the abdominal aorta produced an aneurysm. The other patient showed proximal coronary-artery involvement, with severe stenosis and resulting acute myocardial infarction. Newly developed lesions were found in the splenic and renal arteries. The aortic arch was not involved in either patient.     

Coronary arteritis with marked fibrous periarteritis: case report.

A case is presented of a 43-year-old man who died suddenly and unexpectedly from cardiac malignant arrhythmia. Autopsy revealed isolated coronary arteritis involving primarily the left coronary stem and extending into the proximal half of its anterior descending branch. The most striking feature of the disease was marked fibrous periarteritis. Similar changes were present segmentally in the peripheral third of the right coronary artery, and also as small isolated foci of adventitial fibrosis in the aortic base. With some hesitation, we classify the case as coronary Takayasu's arteritis.     

Thoracic aortic aneurysm associated with aortitis--case reports and histological review.

Ten cases of post-inflammatory aneurysm of the thoracic aorta were studied morphologically using aortic wall and aortic valve tissue resected during the surgical repair of the lesions. Four cases of aortic aneurysm in Behcet's disease showed massive perivascular infiltration of the lymphocytes and plasma cells in adventitia and vasa vasorum. Fibrous obliteration of the vasa vasorum was seen, and neutrophilic infiltration was also associated in two cases with recurrent prosthetic valve failure. Three cases of aortic aneurysm associated with Takayasu's arteritis showed similar histologic features, but giant cells and histiocytic reactions were additional findings. Plasma cell infiltration was more pronounced compared to the Behcet's aortitis. One case of luetic aortitis showed a non-specific chronic inflammatory reaction with florid endothelial cell proliferation. One case of ankylosing spondylitis showed fibrous thickening at the periannular portion of the aortic valve cusps, but this finding was also seen in an aortic valve of a patient with Behcet's disease. One case without any disease association showed similar features of chronic inflammation with granuloma formation. The findings suggest that they have similar histologic features indistinguishable in terms of the clinical disease association, but a granulomatous reaction could be expected in Takayasu's arteritis.     

Coronary artery lesions in Takayasu's arteritis--clinical and angiographic study

Two hundred and twenty five patients of Takayasu's arteritis were studied over 13 years. Male:Female ratio was 1:7. Mean age of the study population was 19 +/- 4 years. Of these 225 patients, 75 patients had symptoms and/or signs of cardiac involvement and these patients were subjected to coronary angiography. Significant coronary artery occlusion (i.e. more than 50% narrowing of luminal diameter) was present in 9 patients. Incidence of coronary artery lesions in Takayasu's arteritis is 12% in this study. The proximal segments of coronary arteries were involved while the distal segments were spared. Out of 34 patients with angina pectoris, only 3 patients had significant coronary arterial narrowing.     

Coronary involvement in Takayasu's arteritis

Coronary involvement may appear in up to a third of patients with Takayasu's arteritis. This affliction may have a dominant impact on the clinical manifestations of the patient. Occlusion of the ostia of the left main coronary artery and of proximal segments of the coronary arteries is the most frequent finding of the coronary vasculature in patients with Takayasu's arteritis. Beyond the vasculitic process enhanced atherosclerosis has a significant and detrimental impact on the disease development. Revascularizations are often unsuccessful particularly when the inflammatory disease is not under satisfactory control. New techniques have been developed in order to use vessels that have not been damaged by the disease as grafts.     

Aortic insufficiency caused by Takayasu's arteritis without usual clinical features

A 30-year-old woman with rapidly progressive, fatal aortic insufficiency was studied. Pathologic studies revealed that the aortic insufficiency was caused by dilation of the aortic root due to Takayasu's arteritis that, unusually, was confined to the ascending aorta and aortic arch. When other diseases that bring about aortic insufficiency can be excluded, Takayasu's arteritis should be considered, even if most of its manifestations are absent.     

Uncommon associations and catastrophic manifestation in Takayasu arteritis: an autopsy case report

Takayasu arteritis, a chronic inflammatory vasculitis affecting aorta and its major branches, is complicated by stenosis, occlusion, and aneurysm formation. The aneurysm formation and subsequent complications such as heart failure, aortic regurgitation, and aneurysm rupture can be fatal. The aortic aneurysm rupture is a rare and fatal complication with only a few cases reported in the English literature. The involvement of coronary artery in Takayasu occurs in about 10% patients, and the coronary artery aneurysm is the least common manifestation. Here, we describe a case of Takayasu arteritis with abdominal aortic aneurysm rupture and coronary artery aneurysm. This patient also had associated systemic inflammatory diseases like sarcoidosis and Hashimoto's thyroiditis.     

Coronary arteritis and aortoarteritis in the elderly males

Summary Two elderly males died suddenly of acute myocardial infarction. Autopsy disclosed extensive and severe coronary arteritis, associated with aortitis. In most of all of the epicardial coronary arteries, fibrous thickening of the adventitia was remarkable and muscle layers of the media were nearly destroyed by infiltration of lymphocytes and plasma cells. The aorta showed adventitial thickening with destruction of the elastic layers of the outer media. In the literature, similar extensive coronary arteritis has been reported in five cases of Takayasu's arteritis. All cases, including ours, were elderly or middle-aged males who died suddenly of myocardial infarction without the antemortem detection of the underlying arteritis. These aspects differ from the typical Takayasu's arteritis and we suggest that these cases are considered as a separate entity.     

Primary aorto-esophageal fistula due to Takayasu's aortitis.

BACKGROUND: Aneurysmal dilatation in Takayasu's arteritis is a recognized complication; however, fistula formation, especially to the esophagus, is very rare. METHODS: A 22-year-old male presented with severe hematemesis. Investigation by means of esophagogastroscopy and CT scan revealed a saccular aneurysm in the proximal descending aorta with communication to the esophagus. The patient was taken to theater, the aneurysm excised and replaced by a graft. RESULTS: Gross examination of the aneurysm showed multiple points of outpouching from the aneurysm. Histopathological examination of the showed marked intimal fibromyxoid thickening, loss of outer medial muscle and elastic fibers and marked fibrosis of the adventitial layer. The histological features were in keeping with Takayasu's arteritis. No evidence of tuberculosis was noted. CONCLUSIONS: This case illustrates an unusual complication of Takayasu's arteritis, in the form of a fistula between the aorta and the esophagus, which resulted in massive hematemesis and the ultimate demise of the patient.     

The effect of aortic wall and aortic leaflet stiffening on coronary hemodynamic: a fluid–structure interaction study

Pathologies of the aortic valve such as aortic sclerosis are thought to impact coronary blood flow. Recent clinical investigations have observed simultaneous structural and hemodynamic variations in the aortic valve and coronary arteries due to regional pathologies of the aortic valve. The goal of the present study is to elucidate this observed and yet unexplained phenomenon, in which a local pathology in the aortic valve region could potentially lead to the initiation or progression of coronary artery disease. Results revealed a considerable impact on the coronary flow, velocity profile, and consequently shear stress due to an increase in the aortic wall or aortic leaflet stiffness and thickness which concur with clinical observations. The cutoff value of 0.75 for fractional flow reserve was reached when the values of leaflet thickness and aortic wall stiffness were approximately twice and three times their normal value, respectively. Variations observed in coronary velocity profiles as well as wall shear stress suggest a possible link for the initiation of coronary artery disease.     

Unusual arteritis causing myocardial infarction in a child

A 5-year-old girl presented with a diffuse inflammatory disease that consisted of fever, lymphadenopathy, splenomegaly, and anterior uveitis. A chest x-ray film indicated an apparently nodular infiltrate. Her condition deteriorated abruptly, and she died of acute myocardial infarction. Autopsy revealed an extensive vasculitis that involved the aorta, pulmonary arteries, and coronary vessels. An aneurysm of the left coronary artery was noted. We present and discuss this case as an unusual arteritis in childhood, closely resembling Takayasu's disease. The presence of extensive erythrophagocytosis in sinus histiocytes hinted at a viral or immunologic origin for this child's disease.     

Takayasu's arteritis treated by percutaneous transluminal angioplasty with stenting in the descending aorta

A 17-yr-old young woman was referred to our hospital with a 2-yr history of claudication of the lower extremities and severe arterial hypertension. Physical examination revealed significantly different blood pressures between both arms (160/92 and 180/95 mmHg) and legs (92/61 and 82/57 mmHg). The hematological and biochemical values were within their normal ranges, except for the increased erythrocyte sedimentation rate (83 mm/hr) and C-reactive protein (6.19 mg/L). On 3-dimensional computed tomographic angiography, the ascending aorta, the aortic arch and its branches, and the thoracic and, descending aorta, but not the renal artery, were shown to be stenotic. The diagnosis of type IIb Takayasu's arteritis was made according to the new angiographic classification of Takayasu's arteritis, Takayasu conference 1994. Percutaneous transluminal angioplasty with stenting was performed on the thoracic and abdominal aorta. After the interventional procedures, the upper extremity blood pressure improved from 162/101 mmHg to 132/85 mmHg, respectively. She has been free of claudication and there have been no cardiac events during 2-yr of clinical follow-up.     

Primary arteriopathy in Takayasu's disease

Three patients with classic Takayasu's arteritis affecting the aorta and its major branches were found to have a unique small pulmonary arteriopathy characterized by deficiency of the outer media, with capillary ingrowth both here and in the thickened fibrosed intima. In one patient there was also evidence of a mononuclear cellular arteritis of the affected arteries and widespread focal angiomatoid like lesions. Another patient had similar, but less well-defined dilatation lesions. This patient's major coronary arteries showed an active arteritis with ingrowth of reparative granulation tissue capillaries into both media and intima, yielding an appearance not dissimilar to that seen in the small pulmonary arteries. The outer pulmonary artery medial defects observed in our patients bear some similarity to experimental acute immune-complex arteritis and may thus provide tenuous evidence for an autoimmune basis for Takayasu's disease.     

Activated myeloid dendritic cells accumulate and co-localize with CD3+ T cells in coronary artery lesions in patients with Kawasaki disease

Emerging evidence implicating the participation of dendritic cells (DCs) and T cells in various vascular inflammatory diseases such as giant cell arteritis, Takayasu's arteritis, and atherosclerosis led us to hypothesize that they might also participate in the pathogenesis of coronary arteritis in Kawasaki disease (KD). Coronary artery specimens from 4 patients with KD and 6 control patients were obtained. Immunohistochemical and computer-assisted histomorphometric analyses were performed to detect all myeloid DCs (S-100(+), fascin(+)), all plasmacytoid DCs (CD123(+)) as well as specific DC subsets (mature myeloid DCs [CD83(+)], myeloid [BDCA-1(+)] and plasmacytoid DC precursors [BDCA-2(+)]), T cells (CD3(+)), and all antigen-presenting cells (HLA-DR(+)). Co-localization of DCs with T cells was assessed using double immunostaining. Significantly more myeloid DCs at a precursor, immature or mature stage were found in coronary lesions of KD patients than in controls. Myeloid DC precursors were distributed equally in the intima and adventitia. Mature myeloid DCs were particularly abundant in the adventitia. There was a significant correlation between mature DCs and HLA-DR expression. Double immunostaining demonstrated frequent contacts between myeloid DCs and T cells in the outer media and adventitia. Plasmacytoid DC precursors were rarely found in the adventitia. In conclusion, coronary artery lesions of KD patients contain increased numbers of mature myeloid DCs with high HLA-DR expression and frequent T cell contacts detected immunohistochemically. This suggests that mature arterial myeloid DCs might be activating T cells in situ and may be a significant factor in the pathogenesis of coronary arteritis in KD.     

Fibronectin biosynthesis and cell-surface expression by cardiac and non-cardiac endothelial cells.

We examined the biosynthesis and surface expression of fibronectin, an adhesive glycoprotein, in several types of cultured porcine endothelial cells: pulmonary artery, thoracic aorta, coronary artery, aortic valve, and mitral valve. We used immunocytochemical staining to compare the levels of fibronectin present in these same tissues in vivo. Using endogenous radiolabeling, we found that all cell types except aortic valve endothelial cells synthesized and released into the culture media substantial quantities of fibronectin. Using radioiodination of intact cells, we found that, whereas both thoracic aorta and pulmonary artery cells had measurable fibronectin on the surface, aortic valve, mitral valve, and coronary artery cells had little cell-surface fibronectin present. Immunocytochemical staining showed that all endothelial regions except aortic valve had substantial quantities of immunoreactive fibronectin in vivo. These data suggest that the aortic valve endothelium may be distinct from other endothelia. Such differences could be important for the pathogenesis of valvular disease.     

Anomalous origin of the left coronary artery from the right side of the aortic valve in Syrian hamsters (Mesocricetus auratus)

This study describes the coronary artery distribution patterns associated with the anomalous origin of the left coronary artery from the right side of the aortic valve in Syrian hamsters. The hearts of 15 affected animals were examined by means of a corrosion-cast technique, histology and scanning electron microscopy. The hamsters belonged to a laboratory inbred colony with a high incidence of coronary artery anomalies and bicuspid aortic valves. The aortic valve was tricuspid in eight hamsters and bicuspid in the other seven. In all cases, the right coronary artery was normal, whereas the left main coronary artery trunk arose from the right aortic sinus or from the right side of the ventral aortic sinus when the aortic valve was bicuspid. In 12 specimens, the left main trunk crossed the infundibular septum and then divided into the left circumflex branch and the obtuse marginal branch. In another specimen, the course of the left main trunk was ventral to the right ventricular outflow tract; in the remaining two, it surrounded the aorta dorsally. In man, some of these distribution patterns may cause myocardial ischaemia and sudden death. The present findings prove that the origin of the left coronary artery from the right aortic sinus occurs in primitive mammals such as the Syrian hamster, suggesting that the defect may occur in other mammalian species. Its possible occurrence should be borne in mind in domestic animals, especially in those with signs of myocardial ischaemia after strenuous activity.     

Prevalence of coronary artery disease in patients with valvular heart disease

To evaluate the usefulness of preoperatie coronary angiography in patients undergoing preoperative investigation because of valvular heart disease, we performed coronary angiography in a consecutive series of 329 patients. The prevalence of significant coronary artery disease was 32%. Asymptomatic coronary artery disease was present in 13%. Angina pectoris proved to be a poor predictor of coronary artery disease in aortic valve disease. In mitral valve disease, however, the specificity was high. A cost-benefit calculation was carried out in order to assess what advantage routine coronary angiography might have. According to this, coronary angiography should be performed in all patients suffering from valvular heart disease with angina pectoris, whereas it can be omitted in younger patients without angina. A cut-off point of 60 years seems appropriate for aortic valve disease and 65 years for mitral valve disease.     

50例老年心脏瓣膜病的外科治疗分析

目的总结老年心脏瓣膜病患者的外科治疗经验。方法对50例老年患者（60-72岁）施行瓣膜置换术，其中行二尖瓣置换（MVR）27例，二尖瓣及主动脉瓣置换（BVR）8例，主动脉瓣置换（AVR）15例，同时行三尖瓣成形术（TVP）30例，冠状动脉旁路移植术（CABG）4例。结果本组围手术期死亡4例；出院后随访1-48个月，心功能恢复至Ⅰ级18例，Ⅱ级30例，Ⅲ级2例。结论随着体外循环心肌保护技术的发展，手术技术和外科监护技术的成熟完善，加强围手术期的处理，老年瓣膜置换术是安全有效的。     

Fatty infiltration of a stenotic aortic valve: an unusual histopathologic finding

Aortic valve tissue excised during stenotic valve replacement surgery commonly exhibits histopathologic changes including prominent calcification of variable severity. We present briefly a case of a 78‐year‐old man with aortic valve stenosis and coronary artery disease undergoing aortic valve replacement and coronary artery bypass grafting. After pathologic examination of excised tissue, the aortic valve was determined to have nodular calcification and myxoid degeneration, as well as evidence of prominent, contiguous fatty infiltration of the valve's spongiosa layer. Although osseous and chondroid metaplasia have been described within excised cardiac valves, a significant constituent of adipose tissue contiguous through the length of a valve and not representing a discrete mass‐forming, neoplastic lesion has been only described in isolated case reports.     

Temporal arteritis with giant cell aortitis, coronary arteritis, and myocardial infarction

Extracranial giant cell arteritis occurs in 10% to 15% of patients with temporal arteritis and polymyalgia rheumatica. Aorta and its major branches are most often involved and death may result from an unsuspected ruptured aortic aneurysm or aortic dissection. Involvement of coronary arteries by giant cell arteritis resulting in death from myocardial infarction is extremely rare. This article describes one such case: an 84-year-old man who died of acute myocardial infarction with the unexpected autopsy finding of giant cell aortitis and coronary arteritis, and who, three years earlier, had sudden onset of bilateral blindness and biopsy-proven temporal arteritis.