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[A case of aortitis syndrome associated with hypertensive intracranial hemorrhage (author's transl)].

A case of aortitis syndrome associated with hypertensive intracranial hemorrhage is reported. A 38-year-old female was admitted in 1980 suffering from hypertension and blood pressure difference between the left and right arms which had existed for 10 years. Two weeks prior to admission, she noticed weakness and numbness of the right arm and leg. She was orientated and alert at the time of admission. Her right upper and lower extremities could not function with full strength against resistance. Also, right hemihypesthesia and hemiparesthesia were present. Left arm systolic blood pressure was 195 mmHg. Her pulse was palpable in both radial arteries. CT scans revealed a small high density area in the posterior crus of the internal capsule with a slight mass effect. There was no ventricular dilatation. She was given conservative treatment, such as with antihemorrhagic and antihypertensive drugs, and was discharged with good recovery after three months. Various studies were performed during her hospital stay. Based on angiographic findings, the most probable diagnosis was an extensive type of aortitis syndrome. Hypertension resulted from narrowing of the abdominal aorta and the right renal artery. Pulmonary scintiscanning using 99mTc-macroaggregated albumin revealed that her pulmonary arteries were also involved. Aortitis syndrome is not infrequent, but intracranial hemorrhage following after aortitis syndrome is rare. This may be because the carotid artery is involved in a high incidence of such cases, and therefore the intracranial blood flow is disturbed in most cases. Although the left common carotid artery was involved in the subject case, hypertensive intracranial hemorrhage occurred in the left posterior crus of the internal capsule. In a case of aortitis syndrome, therapy resulting in recirculation to the carotid artery involved is generally carried out in order to improve poor intracranial blood flow. However, it is suggested that control of blood pressure is necessary to prevent intracranial hemorrhage in aortitis syndrome.

Adult↗

Aspergillus aortitis after cardiac surgery.

OBJECTIVES: The aim of this study was to describe the clinical characteristics of Aspergillus aortitis in a small series of consecutive patients. BACKGROUND: Aspergillus infection of the ascending aorta after cardiopulmonary bypass surgery has rarely been reported and has always resulted in death. METHODS: Aspergillus aortitis was confirmed by pathologic and microbiologic analysis in eight men (61 +/- 8 years) of 9,375 consecutive patients who underwent cardiac surgery between 1975 and 2000. RESULTS: Patients presented with Aspergillus aortitis after aortic valve replacement (n = 5), coronary revascularization (n = 2), or both (n = 1). Initial symptoms appeared between the immediate postoperative period and up to two years after surgery. All patients had prolonged fever. Ante-mortem diagnosis was established in only three patients for whom transthoracic echocardiography was suggestive of aortic pseudoaneurysm and was confirmed by thoracic computed tomography or aortography. All patients had negative peripheral blood cultures. Seven patients died at short-term follow-up, and the one surviving patient was promptly treated by surgery and antifungal drugs. Pathologic examination confirmed Aspergillus aortitis with multi-organ dissemination without heart involvement in all patients except for two, in whom aortic valve endocarditis was found. Fungal cultures confirmed the presence of Aspergillus fumigatus in all patients. CONCLUSIONS: Aspergillus aortitis is typically found after aortic valve or coronary surgery. It commonly leads to lethal multi-organ dissemination without involvement of the intracardiac structure. This entity should be considered in patients with persistent fever and negative blood cultures after open-heart surgery involving significant aortic wall damage, irrespective of the postoperative period.

Adult↗

Lupus aortitis: a case report and review of the literature.

Systemic lupus erythematosus is a complex immunological and rheumatological disease that has numerous complications. Central to the pathogenesis of systemic lupus erythematosus is immune complex formation and deposition in blood vessels and end organs. This is a case report of an autopsy of a patient with systemic lupus erythematosus, end stage renal disease, peripheral vascular occlusive disease, pancreatitis, and aortitis. The aortitis was found to be immune complex mediated with deposition of IgG, C3, as well as fibrinogen in the wall of the aorta as shown by immunofluorescence. The hypercoagulable state of the patient is discussed with particular emphasis on the role of anticardiolipin antibodies, antiphospholipid antibodies, and anti-beta-2-glycoprotein I in the pathogenesis. This case is unique in that the immune complex mediated aortitis has not been described in the literature over the past 25 years. We recommend that the diagnosis of immune complex mediated aortitis be considered in the differential diagnosis of aortitis, particularly in the background of a patient with systemic lupus erythematosus.

Adult↗

Aortitis.

Aortitis is defined as an inflammatory process that involves one or more layers of the aortic wall (internal elastic lamina, tunica media, and adventitia) and can be caused by multiple mechanisms. Clinical features are usually nonspecific and a high index of suspicion is required for making the diagnosis. Although noninvasive imaging studies are being increasingly used in evaluating patients for diagnosis, angiography remains the gold standard for confirming aortic involvement. When tissue is available, pathologic examination can aid in clarifying the diagnosis. Aortitis, irrespective of the underlying cause, frequently displays similar clinical, pathologic, and arteriographic features and therefore understandably presents a therapeutic and diagnostic dilemma. Whatever the cause, early identification and aggressive treatment is required in order to avert the potentially life-threatening sequelae. The treatment of aortitis is determined by the underlying cause. If diagnosed early, infectious aortitis responds to appropriate antimicrobial therapy, whereas noninfectious, immune-mediated aortitis requires immunosuppressive therapy. However, in many instances, primarily because of the delay in diagnosis, surgical intervention is necessary to treat the associated anatomic and physiologic sequelae. Less definitive and more controversial is the role of inflammation in both primary and secondary accelerated atherosclerosis of the aorta and its modality of treatment.

Journal Article↗

Aortitis diagnosed by F-18-fluorodeoxyglucose positron emission tomography in a patient with syphilis and HIV coinfection.

The most common manifestation of cardiovascular syphilis, a rare diagnosis since the introduction of penicillin, is aortitis of the ascending aorta. Since the majority of patients with uncomplicated aortitis are asymptomatic, early diagnosis is difficult. We report the case of an HIV-positive patient with asymptomatic syphilitic aortitis that was incidentally diagnosed with F-18-fluorodeoxyglucose (FDG) positron emission tomography (PET). We conclude that FDG-PET could become a promising new imaging technique for both diagnosis and follow-up of patients with syphilitic aortitis.

Adult↗

Behçet's aortitis and aortic regurgitation: a report of two cases.

Behçet's disease is a rare systemic vasculitis that may lead to neurologic complications and rare manifestations of aortitis and aortic regurgitation. We report 2 cases of Behçet's aortitis and aortic regurgitation. The first patient presented with acute stroke. Recognition of acute aortitis on echocardiography led to the diagnosis of vasculitis as the cause of the cerebral event. This case highlights the echocardiographic features of aortic root pathology from acute aortitis to subsequent aortic valve perforation. In both cases, severe aortic regurgitation necessitated aortic valve replacement. Both were complicated by valve dehiscence requiring reoperation, illustrating the postoperative morbidity in this inflammatory condition.

Adult↗

[Aortitis after G-CSF injections].

INTRODUCTION: Aortitis consists in aortic wall inflammation from infectious or non infectious cause. It may lead to aortic aneurysm with a risk of rupture, which is life-threatening and may justify surgical procedures. The cause of the aortitis is sometimes obscure. CASE REPORT: We report the case of a 55 years old woman who developed acute aortitis of the descending aorta after G-CSF (granulocyte-colony stimulating factor) injections for blood stem cells graft. No cause was found to the aortitis, the evolution was favorable after corticosteroid treatment, without aneurysm at six months. CONCLUSION: The present case rises the question of G-CSF (Neupogen responsibility in aortic lesions. Neutrophilic mediated diseases (Sweet's syndrome, pyoderma gangrenosum) and leukocytoclastic vasculitis were reported after G-CSF therapy. Neutrophils induced by G-CSF injections present functional abnormalities which may play a role in the pathogenesis of these diseases.

Aortitis↗

Surgical management of Behçet's aortitis: a report of eight patients.

BACKGROUND: Behçet's aortitis is one of the most severe manifestations of Behçet's disease. However, there have been few reports of this rare entity. This article we describe our experience with surgical treatment of Behçet's aortitis in 8 patients. METHODS: From May 1978 to February 1995, we operated on 8 patients with Behçet's aortitis. All patients were followed up for a total of 54.4 patient-years (mean, 6.8 years). RESULTS: There were no operative deaths, but 1 patient died in the hospital of ventricular fibrillation. Another death occurred as a result of pseudoaneurysm rupture 10 months after operation. Fourteen operations were performed on eight patients: a second operation was performed on four patients, and a third operation was performed on two patients. The translocated Bentall procedure, a modified Bentall procedure, was performed six times. One patient required a second operation for graft detachment, but the others have done well without further surgical intervention. Aortic valve replacement was performed six times, and three reoperations were needed after aortic valve replacement. CONCLUSIONS: The translocated Bentall procedure may be effective for Behçet's aortitis, but further investigation is necessary.

Adult↗

Aortitis syndrome associated with positive perinuclear antineutrophil cytoplasmic antibody: report of three cases.

We recently experienced three cases of aortitis syndrome that were associated with perinuclear antineutrophil cytoplasmic antibody (ANCA). In the three cases, roentgenographic examination revealed the typical appearance of stenosis or occlusive subclavian arteries. In addition, two cases showed a thickened thoracic aorta wall and the remaining case had irregular stenosis of both common iliac arteries. All three cases had persistently increased ESR and CRP over the years. These findings suggested the diagnosis of aortitis syndrome. ANCA tests were performed because of rapidly progressive glomerulonephritis symptoms in two patients and marked excretion of beta(2)-microglobulin in urine in one patient. The test showed P-ANCA in all three patients, with two patients identified as anti-MPO antibody and the third patient as non-MPO antibody. The implication of ANCA in the pathogenesis of aortitis syndrome is presumed to be: ANCA, which plays an important role in the pathogenesis of small vessel vasculitis, induces vasculitis of the vasa vasorum in the aorta or main branches (or both) and this pathologic process results in the pathogenesis of aortitis syndrome.

Aged↗

Giant cell arteritis and polymyalgia rheumatica: usefulness of vascular magnetic resonance imaging studies in the diagnosis of aortitis.

OBJECTIVES: One of the unresolved challenges posed in giant cell (temporal) arteritis (GCA) is the detection and monitoring of large-artery complications, particularly aortitis. Recent investigations support vascular magnetic resonance imaging (MRI) studies in this issue. We report our preliminary experience with this imaging technique in the study of the aorta and its proximal branches in patients with GCA and/or polymyalgia rheumatica (PMR). METHODS: Between 2000 and 2003, six patients with GCA and/or PMR seen in our department were diagnosed with aortitis using vascular MRI studies. In all cases, the study was performed according to a specifically designed protocol that included MRI and MR angiography (MRA). RESULTS: MRI was a hepful non-invasive method for diagnosis of aortitis in all cases, providing accurate information about its extent. In particular, MRI had a higher ability to detect earlier stages of vasculitis disclosing subclinical aortitis in five of the six patients. The main signs of early vascular inflammation observed were vessel wall thickness and oedema (six cases) and increased mural enhancement on postcontrast T1-weighted images (four cases). MRA disclosed lumen changes (stenosis) in two patients. On follow-up studies, whereas vascular stenosis and vessel wall thickness remained invariable, vascular wall oedema and contrast enhancement improved significantly when disease activity decreased. CONCLUSION: MRI may be a useful technique for diagnosing patients with occult major artery involvement in GCA, whether presenting with classic symptoms of temporal arteritis or PMR. Its utility for monitoring the course of the disease and response to treatment requires further confirmation.

Aged↗

Abdominal aortitis due to Streptococcus pneumoniae and Enterobacter aerogenes: a case report and review.

Endovascular infections are 1 cause of fever of unknown origin. We describe a diagnostically challenging case of cryptogenic abdominal aortitis from Streptococcus pneumoniae and Enterobacter aerogenes. A 72-year-old male presented with epigastric pain, fevers, and chills. A computed tomography scan demonstrated enlargement and ulceration of the distal abdominal aorta, prompting urgent vascular surgery. Intraoperative tissue cultures grew S. pneumoniae and E. aerogenes and gatifloxacin was administered for 6 weeks. Spontaneous abdominal aortitis is uncommon and usually due to a single pathogen. This is the second reported case of polymicrobial infectious aortitis and to date, Enterobacter has only been reported in infected aortic grafts. Clinicians should maintain a high index of suspicion for infectious aortitis as the mortality, if only treated medically, approaches 100%.

Aged↗

Aspergillus niger aortitis after aortic valve replacement diagnosed by transesophageal echocardiography.

Aspergillus aortitis following cardiac surgery has an important role among the cardiac infections as almost all affected cases result in death. Survival of the patient with Aspergillus aortitis is dependent on early initiation of aggressive medical and surgical treatment. Transesophageal echocardiography proved very useful in the diagnosis of this uncommon case of aortitis. In this paper, we present a patient with aortitis caused by Aspergillus niger that hasn't been reported previously diagnosed by transesophageal echocardiography following cardiac surgery.

Aneurysm, False↗

Diagnosis and follow up of aortitis in the elderly.

OBJECTIVES: To evaluate the correlation of MRI and [(18)F]FDG-PET scans with the clinical course and inflammatory markers in patients with aortitis. METHODS: Eight patients with aortitis presenting with unspecific GCA-like symptoms were examined. Aortitis was diagnosed and followed up by [(18)F]FDG-PET and MRI. The aorta was divided into three vascular regions (ascending aorta, aortic arch, and descending aorta) to localise the aortic inflammation and compare both imaging techniques. RESULTS: were correlated with clinical and laboratory examinations. RESULTS: At diagnosis, 20/24 vascular regions from eight patients were positive by [(18)F]FDG-PET scan and 15/21 aortic regions by MRI. Patients were treated with corticosteroids and followed up for a mean (SD) of 13.3 (4.7) months. In [(18)F]FDG-PET, 11/20 (55%) initially pathological aortic regions returned to normal in the follow up examination, which correlated closely with the clinical and laboratory follow up examination. Conversely, in MRI, 14/15 initially affected vascular regions were unchanged. CONCLUSIONS: [(18)F]FDG-PET and MRI are both effective techniques for detecting early aortitis and have a high correlation with laboratory inflammatory measures. However, during the follow up examination, [(18)F]FDG-PET uptake decreased in line with the clinical symptoms and inflammatory serum markers, whereas MRI scans gave more static results.

Aged↗

Giant aneurysm of the left main coronary artery in Takayasu aortitis.

Takayasu aortitis is a chronic inflammatory vasculitis characterised by stenosis or obliteration of large and medium sized arteries. Although coronary arteries are affected in approximately 10% of cases, most of the lesions are luminal narrowing, and coronary aneurysm formation is extremely rare. A case is described of giant aneurysm of the left main coronary artery complicated with Takayasu aortitis in a 46 year old Japanese woman who was followed until her death at age 71. Pronounced intimal proliferation and adventitial fibrous thickening of the involved arterial wall usually induce constriction or occlusion at the orifice of the main branch of the aorta in Takayasu aortitis. However, systemic hypertension, which resulted from renovascular stenoses in this case, is likely to have enlarged the vessel lumen before replacement of medial and adventitial fibrosis after extensive destruction of medial elastic fibres in the left main coronary artery. Moreover, associations such as autoimmune hepatitis, chronic thyroiditis, and Sjögren syndrome strongly suggests that Takayasu aortitis may be an autoimmune disease.

Aorta↗

Aortic valve replacement for aortic regurgitation caused by aortitis.

Between January 1984 and December 1998, 19 patients (16 with Takayasu's arteritis, 3 with non-Takayasu's aortitis) underwent surgical treatment for aortic regurgitation resulting from the aortitis. Of the 19 patients, 14 had aortic valve replacement (AVR) and 5 had aortic root replacement. One patient (5.3%) died of graft infection during the hospital stay. During the follow-up period, 1 (5.6%) of the 18 postoperative patients died of paravalvular leakage due to valve detachment, which also required redo-operations in 2 patients with non-Takayasu's aortitis. Both patients were operated on during the active phase of the inflammation without perioperative steroid therapy. Although transmural pledgeted sutures were used for replacement of the detached prosthetic valve in 1 of these 2 patients, disruption of the aortic wall resulted in recurrence of valve detachment. In the other patient, aortic root replacement was successfully performed with the Cabrol technique in the second operation. Perioperaitve steroid therapy plays an important role in preventing complications after AVR when the valve replacement is carried out during the active phase of the inflammation, and for patients with non-Takayasu's aortitis, aortic root replacement should be considered to reduce the tension on the suture line and the native aortic valve annulus.

Adult↗

Primary aortoenteric fistula related to septic aortitis.

CONTEXT: Primary aortoenteric fistulas usually result from erosion of the bowel wall due to an associated abdominal aortic aneurysm. A few patients have been described with other etiologies such as pseudoaneurysm originating from septic aortitis caused by Salmonella. OBJECTIVE: To present a rare clinical case of pseudoaneurysm caused by septic aortitis that evolved into an aortoenteric fistula. CASE REPORT: A 65-year-old woman was admitted with Salmonella bacteremia that evolved to septic aortitis. An aortic pseudoaneurysm secondary to the aortitis had eroded the transition between duodenum and jejunum, and an aortoenteric fistula was formed. In the operating room, the affected aorta and intestinal area were excised and an intestine-to-intestine anastomosis was performed. The aorta was sutured and an axillofemoral bypass was carried out. In the intensive care unit, the patient had a cardiac arrest that evolved to death.

Aged↗

Redo Bentall operation for the aortitis syndrome.

The aortitis syndrome is a chronic inflammatory arterial disease with an unknown etiology that may present as a variety of vascular lesions. The surgical treatment of aortitis syndrome is associated with many potential difficulties due to the inflammatory nature of the disease. A patient with the aortitis syndrome underwent the Bentall operation for annulo-aortic ectasia and aortic regurgitation 11 years prior to presentation. The operation was not performed during the active inflammatory phase. An anastomotic dehiscence required reoperation, which was performed with Piehler's method. In the aortitis syndrome, the exclusion technique, Carrel patch repair of the coronary arteries and pledgeted anastomoses should be performed for aortic root reconstruction.

Adult↗

Sudden death associated with aortitis and fibrosclerosing disease of the conduction system.

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.

Aortitis↗