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Lymphoplasmacytic aortitis and acute aortic dissection. An uncommon association.

A 43-year-old white man with a history of cigarette smoking, hypertension, nephrolithiasis, and cervical degenerative arthritis was hospitalized for sudden-onset severe, substernal, and pleuritic chest pain with epigastric radiation. Despite evaluation, the cause remained unclear and the patient expired on hospital day 5. Autopsy revealed acute Stanford type A aortic dissection, hemopericardium, and hemothorax. Grossly, the aorta and its branches, including uninvolved medium-sized arteries, displayed extreme mural fragility. Microscopic examination showed a primary lymphoplasmacytic aortitis-periaortitis without giant cells. Rents within the tunica media, medial-adventitial inflammation, and elastic fiber disruption were limited to sites of gross aortic dissection. Muscular arteries showed patchy, chronic arteritis-periarteritis without giant cell infiltrate or aneurysm formation. This case documents an unusual association of primary lymphoplasmacytic aortitis and aortic dissection.

Adult↗

[Rupture of the aortic arch due to bacterial aortitis--a case report of a patient undergoing successful surgical therapy].

A 59-year-old man came to our hospital because of hoarseness and chest pain. On initial examination, a chest roentgenogram showed no abnormality. Three weeks later, while the patient was waiting to undergo a computed tomographic (CT) scan, shock suddenly developed. A chest roentgenogram revealed an extremely wide mediastinal shadow. A CT scan revealed rupture of the aortic arch near the base of the left subclavian artery, although there was no aneurysm. An emergency operation was performed. The ruptured aortic arch was replaced with an artificial graft during cardiopulmonary bypass with selective cerebral perfusion. A diagnosis of infectious aortitis was not made until a pathologist discovered colonies of gram-positive cocci in the resected specimen of the aortic arch. Postoperatively, antibiotics were administered for only four days. The patient is well about two years after the operation. Infectious aortitis had a very low prevalence and rarely involves the thoracic aorta. However, we should be aware of that it may cause aortic rupture even in the absence of aneurysmal dilatation.

Aorta, Thoracic↗

Treatment of Takayasu's aortitis with self-expanding metallic stents (Gianturco stents) in two patients.

Gianturco self-expanding metallic stents were successfully placed to treat incomplete balloon dilatation of the abdominal aorta in 2 patients with Takayasu's aortitis. In 1 case eleven 1-cm long, 1-cm diameter stents were inserted in tandem to bridge a long stenosis. In the other patient, four stents 1.5 cm long, 1.5 cm in diameter were inserted in tandem to bridge what had been a complete occlusion prior to balloon angioplasty. Clinical symptoms, as well as angiographic findings, improved significantly after placement of the stents. The patients underwent repeat aortography at 10 and 8 months, respectively, and were followed clinically for a total of 18 months and 15 months, respectively, without clinical evidence of deterioration. Our results show the usefulness of expandable metallic stents in the treatment of aortic stenosis due to Takayasu's aortitis that remains unresponsive to balloon dilatation.

Adult↗

Disappearance of thyroid-stimulation blocking antibody by glucocorticoid therapy in a patient with primary myxedema who developed aortitis syndrome during L-thyroxine supplementation.

A 39-year-old woman with primary myxedema, who had the potent activities of thyrotropin-binding inhibitory immunoglobulins (TBII) and thyroid-stimulation blocking antibodies (TSBAb), developed aortitis syndrome about 6 months after the initiation of L-thyroxine (L-T4) supplementation. A 35 mg daily dose of prednisolone for aortitis syndrome was initiated, and the dose was gradually reduced. TBII and TSBAb activities were gradually decreased, and both reached normal levels (7.7% and 10.1%, respectively) 3 months after the initiation of prednisolone. Therefore, dose of L-T4 was gradually reduced, and L-T4 supplementation was stopped. Subsequently, however, recurrence of hypothyroidism was not observed. These observations indicate the possibility that hypothyroidism remits with disappearance of TBII and TSBAb activities in not only neonatal cases but also adult cases.

Adult↗

Bacterial aortitis resulting in rapid development of infective "mycotic" aneurysm.

Aortitis and aneurysms with an infective etiology, though uncommon, must be recognized at imaging so that early medical and surgical intervention can improve survival. We report a case in which serial CT scans illustrate the short-interval development of an infective "mycotic" aneurysm resulting from bacterial aortitis.

Journal Article↗

Paroxysmal hypertension in aortitis syndrome.

Three patients with aortitis syndrome ehibited paroxysmal hypertension which seemed to result from baroreceptor dysfunction. All of the patients had signs of active inflammation of aortitis syndrome and stenotic carotid and subclavian arteries. During the attacks, the blood pressure rose to at least 230 mm. Hg systolic and the heart rate exceeded 100. However, with prolonged administration of steroid hormones, the attacks ceased. In two patients with dilated thoracic aortas and aortic regurgitation, the attacks of paroxysmal hypertension occurred without apparent precipitating factors and were followed by anginal pain with marked ST depression. The sympathicotonic state resulting from the disturbance of the baroreceptors was considered to be responsible for the attacks. In another patient, the attacks occurred in the course of treatment with a steroid hormone and were provoked only by voluntary micturition. This post-micturition hypertension was presumed to be an expression of abnormal overshooting following a fall in blood pressure after voiding.

Adult↗

Clinical features and course of aortitis syndrome in Japanese women older than 40 years.

The clinical features and course of aortitis syndrome were studied in 11 women older than 40 years of age. The patients were Japanese women, mean age 57 +/- 6 years old, who were followed for 6.9 +/- 3.8 years. Data from 24 young patients were used for comparison. In the older patients, systemic hypertension (73%), calcification of the aorta (73%), left ventricular hypertrophy (92%) and cardiomegaly (82%) were frequent, whereas the erythrocyte sedimentation rate was normal in 5 patients and only slightly accelerated in 6. C-reactive protein was positive in 2. The incidence of cardiac involvement and inflammatory signs was significantly different from findings in the young patients. Aortic regurgitation (AR) (55%) was significantly more frequent and renal artery stenosis was not observed. Other arterial lesions revealed a pattern similar to those seen in the young patients. An irregular luminal surface, kinking and calcification were present in the lesions in the older patients. The survival rate at 5 years was 80%. Five of 6 patients with AR had congestive heart failure, 4 of whom died. One died after a stroke. Thus, aortitis syndrome in older patients has a long course. There is usually an associated AR, renal artery stenosis is rare and other arterial lesions do not change a great deal. The prognosis may be good, but depends on the association of AR.

Adult↗

Severe aortic regurgitation secondary to idiopathic aortitis.

Clinical and morphologic features are described in two relatively young adults with aortic regurgitation secondary to chronic aortitis. The regurgitation in each was severe enough to require aortic valve replacement. Both patients had normochromic, normocytic anemia, considerable weight loss despite congestive cardiac failure, and negative serologic tests for syphilis. These systemic manifestations in association with the aortitis suggest that both had Takayasu's arteritis. In addition, one patient had total occlusion at the origin of one subclavian artery (classic pulseless disease). Takayasu's arteritis must be added to the list of causes of severe aortic regurgitation.

Adult↗

Transaortic patch angioplasty for left coronary ostial stenosis in a patient with Takayasu's aortitis.

A 35-year-old woman who had left coronary ostial stenosis and aortic valve regurgitation due to Takayasu's aortitis underwent transaortic patch enlargement of the stenosed left coronary ostium in combination with aortic valve replacement. This technique may be suitable and recommendable as an alternative to aortocoronary bypass grafting or endarterectomy for coronary ostial stenosis in Takayasu's aortitis.

Adult↗

Surgical treatment of ascending aortic aneurysms in patients with giant cell aortitis.

BACKGROUND: Giant cell aortitis is a rare cause of ascending aortic aneurysm disease despite giant cell arteritis being a common cause of vasculitis. We evaluated an 8-year experience with surgical repair with regard to preoperative variables, extent of disease, required surgical procedures, and the propensity to develop additional great vessel aneurysms. METHODS: Thirty-seven patients (29 female, 8 male; aged 69.6 +/- 9.5 years) were operated on from 1995 to 2002. Ten (27%) patients had a history of steroid treatment for temporal arteritis or polymyalgia rheumatica 8.9 +/- 3.9 years before. Nineteen (51%) patients had +3 or +4 aortic regurgitation. Maximal aneurysm size was 6.1 +/- 0.8 cm. Thirty (81%) patients underwent polyethylene terephthalate fiber (Dacron) tube graft replacement of the ascending aorta, 4 (11%) had a modified Bentall procedure, 2 (5%) had a valve-sparing aortic root reconstruction, and 1 (3%) had aortorrhaphy. Twenty-two (59%) patients required 22 +/- 9 minutes of hypothermic circulatory arrest for hemiarch or complete aortic arch replacement. Twenty-six (70%) patients had concomitant cardiac procedures. RESULTS: There was no early mortality. Morbidity was reexploration for bleeding in 3 (8%) patients, stroke in 3 (8%), left vocal cord paralysis in 2 (5%), renal failure in 2 (5%), and gastrointestinal bleeding in 1 (3%). Mean follow-up was 2.8 +/- 2.3 years. Four-year actuarial survival was 74% (95% confidence interval, 57% to 94%). Other descending or abdominal aortic or great vessel aneurysms occurred in 17 (46%) patients. Four patients had prior aneurysm surgery, 8 are monitored with aneurysms, and 5 underwent repair of an aneurysm in the follow-up period. Of 8 late deaths, 3 were caused by complications of a descending thoracic aneurysm. No patient required replacement of a native aortic valve that was preserved during the initial operative procedure. CONCLUSIONS: Ascending aortic aneurysms caused by giant cell aortitis can involve the aorta from the aortic root through the aortic arch, thus requiring a tailored operative approach. The aortic valve tissue is spared from the pathologic process. Other aneurysms of the aorta and great vessels occur in nearly half of patients. Frequent surveillance of the remaining aorta is mandatory.

Aged↗

Transesophageal echocardiographic diagnosis of Aspergillus fumigatus aortitis after percutaneous coronary intervention.

Aspergillus aortitis is an uncommon infection with high mortality and has been reported in patients after cardiopulmonary bypass. We report the first case of Aspergillus aortitis in an immunocompetent man immediately after percutaneous coronary intervention to an aortocoronary bypass graft. In this case, transesophageal echocardiography played a pivotal role in diagnosis.

Aged↗

[Syphilitic aortitis. Experience of an internal medicine unit].

INTRODUCTION: Infectious aortitis remains a rare disease. It is characterized by an endarteritis of infectious origin generally followed by the development of a so called mycotic aneurysm. Those infectious aneurysms account for 0.5 to 1.3% of all aortic aneurysms. Of the infectious agents, Treponema pallidum has a particular place. Cardiovascular syphilitic infection was very common at the beginning of the XX(th) century with a prevalence of 6.9% of all autopsies. In 1950-1960, the prevalence had decreased to less than 1%. Since 1990, syphilis was considered as disappeared. EXEGESIS: we report syphilitic aortitis in four patients. Diagnosis, treatment, and prognosis are detailed. CONCLUSION: A syphilitic infection of the aorta should be looked for in every patient suffering from an inflammatory or infectious disease of aorta.

Adult↗

Stenosis in the aorta caused by non-specific aortitis: results of treatment by percutaneous stent placement.

AIM: To evaluate the short-term results of percutaneous stent placement in treating stenosis in the aorta caused by non-specific aortitis (Takayasu's disease). METHOD: Five patients were treated by this method, all of whom had uncontrolled hypertension, haemodynamically significant stenosis in the aorta and a clinically inactive disease. Stents were placed to treat an obstructing dissection in four and recurrent stenosis in one patient. All procedures were carried out via the percutaneous transfemoral route, utilizing self-expanding stents. RESULTS: The lesion was located in the thoracic aorta in three patients and in the abdominal aorta in two patients. The stenosis decreased from 81+/-2 to 7+/-3%, the pressure gradient fell from 97+/-5 to 9+/-2 mmHg, the blood pressure improved from 200+/-3/124+/-2 to 131+/-2/81+/-2 mmHg, and the drug requirement fell from 4+/-.2 to 1.3+/-.3 (P value for all <0.001). No complication was encountered. At follow-up at 13+/-4 months, all of the patients had improved clinically. Intimal hyperplasia within the stent was seen in two patients who underwent follow-up angiograms. CONCLUSION: Percutaneous aortic stenting is useful in treating selected patients with non-specific aortitis. Further information about the long-term behaviour of intimal hyperplasia and the response of stented segments to the aging process needs to be understood before elective stenting in this young patient population can be advocated.

Adolescent↗

In situ reconstruction of septic aortic pseudoaneurysm due to Salmonella or Streptococcus microbial aortitis: long-term follow-up.

OBJECTIVE: This study was undertaken to illustrate the safety of in situ reconstruction of septic aortic pseudoaneurysm (SAP) secondary to microbial aortitis, with or without long-term antibiotic treatment. METHODS: Data for patients with SAP (11 abdominal, 4 thoracic) operated on between 1993 and 1999 were reviewed. Computed tomography and aortography showed septic pseudoaneurysm in all patients before surgery. After diagnosis of SAP, all patients underwent aneurysm resection and extensive debridement, with in situ prosthetic grafting or patch repair angioplasty. The graft in 10 of the 11 patients with abdominal SAP was also wrapped with an omental pedicle. In vitro active parenteral antibiotic therapy was prescribed for all patients for at least 2 to 8 weeks after surgery. RESULTS: All 15 patients had positive preoperative blood cultures or intraoperative tissue cultures for Salmonella spp (n = 12), viridans Streptococcus (n = 1), group G Streptococcus (n = 1), or Streptococcus pneumoniae (n = 1). There were two perioperative deaths (13.3%), one 6 days after surgery and the other 19 days after surgery, and two late deaths, at 8 and 10 months after surgery, neither of which was related to aortic repair. One patient was unavailable for follow-up. The other 10 patients have been regularly followed up with abdominal ultrasound or computed tomography (mean, 84 months; range, 47-118 months). To date, there has been no graft infection, thrombosis, false aneurysm, or subsequent aortic surgery in these 10 patients. CONCLUSION: SAP due to Salmonella and streptococcal microbial aortitis can be successfully treated with resection of the aneurysm and extensive debridement, followed by in situ prosthetic graft interposition or patch repair aortoplasty. This is a safe and effective treatment that may result in complete remission of SAP. Postoperative parenteral antibiotic therapy should be continued for 2 to 8 weeks. Although usually recommended, lifelong suppressive antibiotic therapy appears to be nonessential with this approach.

Aged↗

Mediastinal mass caused by syphilitic aortitis.

A 47 year old man presented with hoarseness and chest pain found to be due to proliferative syphilitic aortitis. The case is unusual as the syphilitic aortitis caused a mediastinal mass without affecting the lumen of the aorta.

Humans↗

Aortitis syndrome with fatal acute aortic regurgitation due to aortic dilatation and aortic valve perforation--a case report.

This is a case report of a fifteen-year-old female with aortitis syndrome complicated by acute fatal aortic regurgitation due to the aortic valve perforation, as well as to aortic dilatation, which was confirmed by the autopsy. Aortic valve perforation was first recognized as the important cause of acute aortic regurgitation in the aortitis syndrome in this report.

Acute Disease↗

Unexplained fever and aortic regurgitation: a rare presentation of Takayasu's aortitis--a case report.

Takayasu's aortitis is rare in male patients. The authors describe the case of a 48-year-old man with a fever of unknown origin, progressive general fatigue, and dyspnea due to aortic regurgitation. A severely thickened aorta was noted during aortic valve replacement. The diagnosis was based on a histologic examination of an operative specimen of the ascending aorta. Takayasu's aortitis should be considered even in male patients with fever of unknown origin and progressive aortic regurgitation.

Aortic Valve↗