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Clinical aspect of aortitis syndrome with special reference to the relation between prognosis and hypertension.

The present study was done to investigate the relation between the prognosis and hypertension, especially hypertension due to renal arterial stenosis, atypical coarctation of the aorta and aortic regurgitation in patients with aortitis syndrome. Sixty-five patients including 25 with normotension and 40 with hypertension who were hospitalized during the last 20 years were included in this study. Out of the 40 patients with hypertension, a cause of hypertension was found in 37 patients: renovascular hypertension alone in 13, renovascular hypertension with atypical coarctation in 11, renovascular hypertension and aortic regurgitation in 2, atypical coarctation alone in 5, aortic regurgitation alone in 5 and all of the three in the remaining one. Thirty-two hypertensive patients out of 40 were treated with antihypertensive drugs and the remaining 8 underwent surgical operation (renal vascular repair in 6 and nephrectomy in 2 for relief of hypertension. In the present data, 13 out of 40 patients with hypertension died despite of the treatment, while only one out of 25 patients with normotension died. According to the mortality in each type of cardiovascular lesion, 6 patients died out of 13 with renovascular hypertension alone, 5 patients died out of 11 with renovascular hypertension associated with atypical coarctation and 2 patients with renovascular hypertension associated with aortic regurgitation died. In contrast, there was no death in hypertensive patients with aortic regurgitation alone or atypical coarctation of the aorta alone. These results indicate that hypertension, especially renovascular hypertension is one of the major determinant of the prognosis in the patient with aortitis syndrome.

Adult↗

Unfolding of the aorta (aortitis) associated with pulmonary tuberculosis.

Dilatation of the thoracic aorta was observed in a significant number of normotensive tuberculous patients below the age of 40 years. Histological examination in a selected number of patients showed a non-specific aortitis. It is suggested that aortitis is probably due to an auto-immune response related to pulmonary tuberculosis and that tuberculosis should be recognized as a common cause of unfolding of the aorta in the African.

Adolescent↗

Regression of the left main trunk lesion by steroid administration in Takayasu's aortitis.

A 62-year-old man with unstable angina due to severe narrowing of the left main trunk (LMT) was examined. Emergency bypass surgery was performed with an internal mammary artery graft, instead of a saphenous vein graft, because of the thickened, edematous ascending aorta. Postoperative coronary angiography showed the lesion of the LMT markedly regressing. Presumably, this stenotic lesion of the LMT was caused by active aortitis and was partially reversible by steroid administration both during and after surgery. Steroid therapy can be added to the list of treatments for cases of LMT disease associated with Takayasu's aortitis, if signs of active inflammation are present.

Angina, Unstable↗

Multiple cerebral aneurysms associated with aortitis syndrome--case report.

A 48-year-old female presented with four aneurysms in the anterior half of the circle of Willis associated with aortitis syndrome. All the aneurysms were successfully clipped. In general, intracranial hemodynamic change, due to stenosis or occlusion of carotid arteries, is considered to initiate aneurysm growth in the vertebrobasilar system in this syndrome, but renal hypertension was probably involved in our patient. Careful screening for multiple aneurysms, even in the anterior circulation, should be undertaken in patients with aortitis syndrome who present with an aneurysm.

Aortitis↗

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.

Adult↗

Aortitis with multiple aneurysms mimicking infective endocarditis.

Aortitis usually produces aortic insufficiency by aortic root dilation. In rare cases the inflammation may involve the aortic valve cusps, causing valvular insufficiency. A patient in whom aortitis produced valvular masses, with aortic and peripheral arterial aneurysms, embolic episodes and aortic insufficiency is described. Valve replacement for suspected infective endocarditis was complicated by homograft dehiscence and multiple false aneurysms. Although immunosuppression was successful in decreasing the patient's vasculitis, he became infected and died of complications of aspergillus infection.

Adult↗

[An autopsy case of aortitis resulting in a tear of the aortic valve due to a rupture of the aneurysm of Valsalva's sinus].

A 43-year-old man was admitted to a hospital because of acute dyspnea and nocturnal orthopnea. Echocardiogram and chest CT showed the dilation of thoracic aorta from the root to ascending portion. On the third hospital day, he died suddenly. At autopsy, the cause of death was indicated to be a tear of an aortic valve due to a rupture of the aneurysm of Valsalva's sinus, followed by acute aortic regurgitation and acute cardiac insufficiency. Histopathological findings of thoracic aorta revealed mesoaortitis, characterized by patchy destruction of the media with a moth-eaten appearance of the medial elastic laminae and a microgranuloma formation, a perivascular mononuclear cell infiltration of the vasa vasorum, and a fibrous thickening of the intima and adventitia. However, there were no abnormalities in main branches of aorta and abdominal aorta, and no systemic vasculitis. This case is a rare one in the clinical course, and may be important to be differentiated from other cases with aortitis, especially Takayasu arteritis and syphilitic aortitis.

Acute Disease↗

[Diagnosis of aortitis].

Aortitis can be a component of a variety of diseases, such as Takayasu arteritis, giant cell arteritis, Behçet's syndrome, Cogan's syndrome, spondylarthropathies, rheumatoid arthritis, systemic lupus erythematosus, sarcoidosis, Erdheim-Chester's disease and a variety of infectious processes like syphilis, Salmonella and others. The presentation is variable: aortic valve regurgitation, aneurysm, dissection, stenosis of the aorta or its initial branches. Sometimes systemic manifestations are at first presentation like fever or inflammatory syndrome. The differential diagnosis may be difficult in some situation like inflammatory aortic atherosclerotic aneurism, or retroperitoneal fibrosis. Some aortitis remain idiopathic. Corticosteroid and sometimes surgery are mostly required to avoid life-threatening complications.

Aortitis↗

Acupuncture treatment for multiple aortitis--a clinical report of 40 cases.

In order to evaluate the therapeutic effects of acupuncture-moxibustion on multiple aortitis, 80 cases (with involvement of the main arteries in the head and arms) were randomly divided into a treatment group treated with acupuncture-moxibustion, and a control group treated with TCM and western drugs. The therapeutic effects were compared. The total effective rate was 95% in the treatment group (including 15% of the cure rate and 62.6% of the marked relief rate), and the total effective rate was 75% in the control group (including 12.5% of the marked relief rate). There was a significant difference in the therapeutic effects (P<0.01) between the two groups. It can be concluded that acupuncture-moxibustion is obviously superior to the routine drug therapy for treatment of multiple aortitis.

Acupuncture Therapy↗

Aortitis caused by Mycobacterium fortuitum.

Aortic valve replacement was complicated by sternal wound infection with Mycobacterium fortuitum. The wound was treated with débridement and antibiotic therapy. Five months later the patient developed fever, and blood cultures yielded M fortuitum. At surgery, aortitis with pseudo-aneurysm formation was encountered. Mycobacterium fortuitum grew from the aortic lesion. This is the first report of M fortuitum causing aortitis, although this organism is known to infect sternal wounds and mediastinum. Mediastinal infection can progress despite a normal wound appearance. Its manifestations may be delayed and may include infections of the aorta as well as of the sternum and mediastinum.

Aortic Valve↗

[Coronary artery bypass grafting for ischemic heart disease combined with aortitis].

Coronary artery bypass grafting (CABG) was performed in a 67-year-old woman with aortitis. She had a past history of right radical mastectomy. Preoperative coronary angiogram showed diffuse stenotic lesions in both right and left coronary arteries. The pressure gradient between ascending aorta and peripheral radial artery was 90 mmHg and the cause of coronary stenosis seemed to be hypertension due to stenotic distal aorta. The aortogram showed occluded bilateral internal thoracic arteries (ITA) and stenotic abdominal aorta. The cephalic artery was supplied by a large meandering artery via dilated gastroepiploic artery (GEA). And thus the GEA was not useful for CABG. Quadruple CABG was performed with mild varicose saphenous vein (saphenous vein grafts to left anterior descending artery and third branch, and sequential saphenous vein graft to right posterior descending and atrioventricular branches). It seemed to be important to demonstrate the arterial lesion of ITA and GEA before CABG in patients with aortitis.

Aged↗

Antemortem diagnosis of giant cell aortitis.

A 67-year-old man well while receiving steroid therapy for giant cell arteritis, developed symptoms of ischemic heart disease and was found to have critical left main coronary stenosis. An aortic biopsy at the time of bypass grafting revealed giant cell aortitis. We discuss the finding of clinically discordant aortitis; the etiology of the concurrent coronary stenosis and therapy with prednisone-dapsone.

Aged↗

Inflammatory aneurysm of the aorta, aortitis, and coronary arteritis.

Inflammatory aneurysm of the aorta is not merely a variant of atherosclerosis, but more probably it is a manifestation of a peculiar systemic disease related to idiopathic retroperitoneal and mediastinal fibrosis. Rarely, aortitis and coronary arteritis may coexist with the inflammatory aneurysm; one of each of such unusual cases are described. Because inflammatory aneurysms of the aorta tend to occur in elderly persons, aortitis and coronary arteritis, when present, cannot be distinguished from the concomitant age-related atherosclerosis without a careful histologic examination.

Aortic Aneurysm↗

Orthostatic hypertension with nephroptosis and aortitis disease.

A 53-year-old woman with nephroptosis and aortitis disease was found also to have orthostatic hypertension. When standing, she had high renin levels and normal catecholamine values, with a reduced baroreflex sensitivity. This orthostatic hypertension largely may be due to an activation of the renin system caused by nephroptosis and partly due to a reduced baroreflex sensitivity caused by aortitis. Captopril and propranolol hydrochloride were effective for the treatment of hypertension.

Aortitis↗

Chronic aortitis following furosemide therapy.

A significant increase in histopathologic nonspecific chronic aortitis was found when 100 autopsy specimens from 1965 were compared with series from 1974 and 1980. The chronic aortitis was correlated with furosemide diuretic administration during the last hospitalization. Furosemide is albumin bound, produces vasodilation, and is suspected of having induced a subclinical haptenic immunologic reaction in about one third of those treated.

Adult↗

Primary aortitis in childhood.

The case of a 5-year-old boy affected by autoimmune aortitis is reported. The onset was acute, the progression rapid. Hypertension, absence of right radial pulse, elevated ESRand immune globulin levels, cellular and humoral immunity against blood vessel wall were observed. The tests suggested an autoimmune origin of the aortitis. On immunosuppressive treatment the signs of activity disappeared and the condition improved and after captopril treatment the blood pressure decreased.

Antibody Formation↗

[A case of aortitis operated twice Bentall's operation due to valve (graft) detachment].

A 40-year-old man had received emergency Bentall's operation due to acute aortic regurgitation (AR) of aortitis in October, 24, 1990. On out patient clinic, the CRP had been kept from 0.5 to 1.5 mg/dl in dose of 10 mg of prednisolone. In the midnight of November, 17, 1992, his condition deteriorated to circulatory shock and echocardiography revealed massive AR due to para-annular graft detachment with large pseudoaneurysm of 50 mm. He was transferred for emergency operation. The composite graft shifted 2-3 cm above the annulus and detached in three quarter of the annulus. Distal anastomosis was also detached in half of anastomosis and coronary anastomosis almost detached. He was operated on modified Bentall's operation employing Piehler's technique and he is now followed in dose of 10 mg of prednisolone. There are some cases of aortitis showing recurrent valve or graft detachment like this case, even if modified Bentall's operation have been done to decrease the mechanical stress to the annulus and the inflammation have been controlled under the dosage of steroid.

Adult↗

Coronary artery stenosis in Reiter's syndrome: a complication of aortitis.

Seronegative spondyloarthritis may be complicated by a variety of cardiac lesions including aortitis. We describe a patient with Reiter's syndrome who presented with angina and new onset heart murmurs. Cardiac catheterization revealed myocardial ischemia and aortic insufficiency. Our patient underwent bypass surgery with aortic valve replacement. Severe narrowing of the coronary ostia was noted and aortitis was documented by pathologic examination.

Adult↗