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Aortitis as a feature of rheumatoid arthritis is considered rare. We have, however, identified 10 patients with aortitis from among 188 consecutive autopsy cases of rheumatoid arthritis. There were 5 men and 5 women with a mean duration of rheumatoid arthritis of 9.6 years. Nine were rheumatoid factor positive and had associated nodules. In addition to standard treatment regimens, 9 patients received corticosteroids. Although involvement of the thoracic aorta was most common, involvement of both the thoracic and abdominal aorta was present in 4 cases. Two patients had aneurysmal dilatation of the thoracic aorta and 1 of the abdominal aorta. Microscopic features of aortitis included necrosis of medial smooth muscle and elastica, with an inflammatory infiltrate comprising primarily lymphocytes and plasma cells. A panmural aortitis was seen in 3 cases. Rheumatoid granulomas were noted in the aortic wall in 5. The diagnosis of aortitis was not made until autopsy in any case. Aortitis was hemodynamically significant in 3 patients. Two had congestive heart failure secondary to thoracic aortitis and aortic valvulitis, and 1 had rupture of an abdominal aortic aneurysm at a site involved by aortitis. Seven patients had rheumatoid vasculitis with a mean of 10 organs involved. Six of these died of complications directly related to vasculitis, including 4 patients with coronary arteritis and associated myocardial infarction. Aortitis can be a feature of severe rheumatoid arthritis and is often associated with rheumatoid vasculitis. Hemodynamic compromise does occur and may be fatal.
Aortitis is known to complicate a number of autoimmune diseases and syphilis. In most patients with autoimmune disease, arthritis is the initial presentation followed by aortic insufficiency. We report two cases of ostensibly healthy, middle-aged men in whom the initial manifestation of aortitis was sudden death. In each patient, there was extension of inflammation from the aorta into the atrioventricular node. These cases emphasize the importance of examining the conducting system in cases of sudden death associated with aortitis and no grossly evident cause of death. To our knowledge, this is the first report of aortitis presenting as sudden death.
Takayasu's aortitis is an arterial inflammatory disease of arteries of unknown etiology. Fainting is a common symptom and has been attributed to ypersensitivity of the baroreflex. We studied baroreflex sensitivity in 11 patients with Takayasu's aortitis and compared it with that of eight control subjects of comparable age. Baroreflex sensitivity was assessed by determining the slope of a regression line relating the rise of systolic arterial pressure to the prolongation of the R-R interval of the electrocardiogram during a transient rise of arterial pressure induced by an intravenous injection of phenylephrine. The average baroreflex slope of patients with Takayasu's arteritis (4.0 +/- 0.8 msec/mm Hg) was significantly less than that of control subjects (10.7 +/- 0.8 msec/mm Hg, P less than 0.001). Reduced baroreflex sensitivity in patients with Takayasu's aortitis may be due to the hardening of the arteries where baroreceptors lie, or to hypertension and/or cardiac disease which was present in most of the patients included in this study. Patients with Takayasu's aortitis who complained of fainting also showed the reduced baroreflex sensitivity. This indicates that fainting in this disease is not likely to be caused by the hyperreactivity of the baroreceptors as is commonly postulated.
Echocardiography was performed in 18 patients with the aortitis syndrome and in 20 age-matched normal volunteers. The aortic root dimension, the aortic dimension at the level of the sinotubular ridge, the aortic arch dimension, the left ventricular internal dimension, the left atrial dimension, the interventricular septal thickness, and the left ventricular posterior wall thickness were measured. All measurements, except for the left atrial dimension, were significantly greater in patients with aortitis syndrome than in the control subjects. We concluded (1) that the patients with the aortitis syndrome may have an enlarged or narrowed aorta, a dilated left ventricle and left atrium, and a thickened interventricular septum and left ventricular posterior wall; (2) that the incidence and the degree of these abnormalities depend on the presence of complications such as aortic regurgitation and arterial hypertension; and (3) that M-mode as well a cross-sectional echocardiography plays an important role in the assessment of the aorta and heart in the aortitis syndrome.
Two autopsy cases of aortitis syndrome (Takayasu's aortitis) in the elderly are presented. Case 1 was an 81-year-old woman in whom hypertension was observed at age 37, and difference of right and left arm blood pressure was pointed out at age 65. She was referred to the authors' hospital at age 72. Chest X-ray and computed tomography of the thorax indicated atypical coarctation and diffuse calcification of the aorta. Case 2 was a 69-year-old woman in whom hypertension was pointed out at age 49, and blood pressure in the arms was found to differ from that in the legs at age 63. Chest X-ray and computed tomography showed diffuse calcification and marked narrowing of the descending aorta. Pathological examination revealed marked calcification in the thickened adventitia of the aorta with mild atherosclerotic change. Irregular fibrotic changes of the adventitia and degeneration of elastic fibers of the media of the aorta were noted in both cases, and were consistent with Takayasu's aortitis. This disorder is common in young women and only a small number of elderly cases are reported although its incidence is increasing. Diffuse calcification of the aorta with an absence of inflammatory signs, which is frequent in older patients, were observed in both case. Systemic hypertension is the most important risk factor with coarctation of the aorta in Takayasu's aortitis. Bypass surgery is recommended in young patients, however in elderly patients, it is generally avoided, in favor of medical control of hypertension.
Fourteen patients with aortic regurgitation due to aortitis syndrome that underwent surgical therapy were studied with particular emphasis on the long term merits of Bentall's operations over the conventional valve replacement. They were divided into two groups; Group A: 6 aortic valve replacements (AVRs) and Group B: 8 Bentall's operations. Post operative complications related to aortitis syndrome developed in 3 cases; a case of paravalvular leakage (Group A) and two cases of aneurysm formation at the coronary artery anastomotic sites (Group B). We speculated that these complications were due to abnormal healing and recurrent inflammatory process of the disease. Therefore we think that conventional valve replacement is not suited to cope with these problems. Since 1984, we have adopted the policy to perform Bentall's operations for all patients with aortitis requiring valve replacement. Bentall's operation required longer aortic cross-clamp time (212.1 +/- 53.9 min) than AVRs (73.0 +/- 25.0) and was more aggressive. However no patients with our current technique to reimplant the coronary artery button and distal graft to aorta anastomosis, which has been used since 1984, has developed pseudoaneurysm or valvular leakage. We conclude that our modified Bentall's operation is effective even for patients who have active aortitis syndrome or need redo operations.
A case of Takayasu's aortitis with severe bilateral coronary ostial stenosis is reported. A transaortic coronary endarterectomy was performed and sufficient patency was confirmed angiographically 9 years after the operation. This is the first report of late coronary angiography after a transaortic coronary ostial endarterectomy in Takayasu's aortitis. The efficacy of this procedure for coronary ostial stenosis in Takayasu's aortitis is emphasized.
Three cases of salmonella aortitis with rupture of the abdominal aorta were admitted to hospitals in a limited area of Southern Sweden during 18 months. Two patients with secondarily infected aneurysms died. One patient with a non-aneurysmal aortitis and retroperitoneal abscess is alive but still hospitalized 13 months after the accomplishment of an axillo-femoral by-pass. All 3 patients were elderly males without a history of recent foreign travel. The majority of salmonella patients in the area during the same time period were younger and had acquired the infection abroad. The epidemiology, diagnosis and treatment of salmonella aortitis is reviewed.
Bacterial aortitis is a rare but serious condition. Even when treated surgically, the prognosis is poor. Prompt diagnosis is mandatory. In the absence of specific clinical signs, radiological assessment by means of ultrasound and CT is most valuable. The treatment of choice is early surgical drainage, debridement and arterial reconstruction, preferably through uncontaminated tissue combined with antibiotic therapy. We report the case of a man aged 80 with Salmonella aortitis in whom the diagnosis of bacterial aortitis was made by means of ultrasound and CT, following which successful intervention was carried out.
A 45-year-old man presented with bilateral lower extremity claudication due to aortitis and periaortic fibrosis. This variety of aortitis has been described in the pathologic literature as chronic periaortitis, a subset of idiopathic retroperitoneal fibrosis. The association of this aortitis with abdominal aortic aneurysm has been described in the surgical literature as inflammatory abdominal aortic aneurysms. These entities are thought to be secondary to the same disease process. We review the possible etiology, diagnostic considerations, and management of these aortidites.
A 54-year-old man underwent aortic valve replacement for syphilitic aortic regurgitation. Eight years later, he was admitted with sudden precordial discomfort and symptoms of superior vena caval obstruction. Ascending aortic dissection was diagnosed by echocardiography and computed tomography of the thorax. The patient succumbed rapidly after admission. Postmortem findings and histological features were compatible with syphilitic aortitis, without significant atherosclerosis. This case report adds to the sparse literature on aortic dissection complicating syphilitic aortitis, and illustrates that, at certain stages of the disease process, syphilitic aortitis can lead to such a degree of mechanical instability of the aortic wall as to predispose to dissection.
It is important for patient management to evaluate coronary arterial involvement in aortitis syndrome. Twenty one cases of aortitis syndrome who experienced chest pain were examined using exercise thallium scintigraphy. The patients were divided into four groups according to the angiographic findings. There were: five patients with left main coronary arterial involvement (group A), four with left or right coronary arterial involvement (group B), nine with aortic regurgitation (group C), and three with pulmonary arterial involvement (group D). In group A and B, all patients had positive ECGs and thallium perfusion defects. Group A patients showed extensive anterolateral perfusion defects, which were compatible with left main coronary arterial involvement. Group C and D patients, who had normal coronary arteries, showed no remarkable perfusion defects although five had positive ECG findings. Thus, the sensitivity and specificity of exercise scintigraphy for detection of myocardial ischemia were 9/9 and 12/12, while those of stress ECG were 9/9 and 7/12 (58%), respectively. It is recommended that exercise thallium scintigraphy be used for detecting clinically occult but significant coronary arterial involvement in aortitis syndrome with chest pain.
It is important in patient management to evaluate coronary arterial involvement in aortitis syndrome. Twenty-one cases of aortitis syndrome who experienced chest pain were examined using exercise thallium scintigraphy. The patients were divided into 4 groups according to the angiographic findings. There were five patients with left main coronary arterial involvement (group A), four with left or right coronary arterial involvement (group B), nine with aortic regurgitation (group C), and three with pulmonary arterial involvement (group D). In groups A and B, all patients had positive ECGs and thallium perfusion defects. Group A patients showed extensive anterolateral perfusion defects, which were compatible with left main coronary arterial involvement. Groups C and D patients, who had normal coronary arteries, showed no remarkable perfusion defects although five had positive ECG findings. Thus, the sensitivity and specificity of exercise scintigraphy for detection of myocardial ischemia were 9/9 and 12/12, while those of stress ECG were 9/9 and 7/12 (58%), respectively. it is recommended that exercise thallium scintigraphy be used for detecting clinically occult but significant coronary arterial involvement in aortitis syndrome with chest pain.
In patients who have aortitis syndrome associated with aortic regurgitation, aortic valve replacement (AVR) is frequently complicated by perivalvular leakage (PVL). A 47-year-old male with aortitis syndrome and severe aortic regurgitation was treated by AVR, modified in such a way as to prevent PVL. The aortic cusps were not resected and were placed between Teflon pledgets and the aortic annulus. We believe that this technique is useful for preventing PVL in patients with aortitis syndrome in which the aortic annulus is severely inflammed.
A patient with aortitis syndrome had a pleural effusion which subsided but reappeared with an exacerbation of aortitis symptoms while under antituberculosis treatment. The character of the fluid was that of an exudate, and the glucose concentration was normal. Clinical and laboratory features of the case suggest that the effusion was part of the aortitis syndrome per se.
Coronary artery disease associated with aortitis syndrome was studied in relation to the coronary artery lesions and age. Four (21%) of 19 patients with aortitis syndrome had coronary artery disease and the mean age of the 4 patients was 53 +/- 14 years. The coronary artery lesions included ostial stenosis in 2 patients and main branch stenosis in 2, but both types of lesion were isolated. The latter patients were older and had coronary lesions like arteriosclerosis on angiogram. Myocardial infarction and congestive heart failure were not present, but a severe coarctation of the aorta was observed in the latter. It was suggested that coronary artery disease might not be rare past middle age, and might include coronary ostial stenosis caused by aortitis and coronary main branch stenosis probably based on secondary arteriosclerosis whose onset was past middle age.
A 46-year-old Japanese woman, who had been diagnosed as having aortitis syndrome 4 years earlier, was admitted to our hospital in May 1989. The diagnosis of aortitis syndrome was confirmed by intravenous digital subtraction angiography which showed stenotic lesions in each subclavian artery, the left common carotid artery, and the descending aorta. Coronary arteriography revealed diffuse and prominent dilatation of entire coronary artery segments. Moreover, a left ventriculogram showed complete obstruction of the mid-ventricle during systole. Thus, we diagnosed this case as aortitis syndrome complicated by coronary artery ectasia and mid-ventricular obstruction. The causal relations of these findings are discussed.