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[A case of aortitis syndrome which presented coarctation of aorta due to intraluminal flap formation in the middle of the thoracic aorta].

We present a rare case of aortitis syndrome associated with dilatation of aorta and coarctation-like effect due to the intraluminal flap formation originated from dissected wall of the aorta. A 31-year-old woman was admitted to our hospital complaining of shortness of breath, palpitation and cough. On admission, her physical status showed congestive heart failure and hypertension of upper extremities and hypotension of lower extremities. Bruits were audible over the neck, the anterior chest and the back. Serological studies showed active inflammation. Chest X-ray film showed upper mediastinal widening, cardiomegaly and pulmonary edema. Aortitis syndrome was strongly suggested by these clinical findings, so that prednisolone therapy was started on 3rd hospital day. Special examinations were performed several days later when inflammatory changes showed a tendency to improve. Chest CT scan, RI angiography and MRI studies showed an aneurysmal dilatation from the ascending aorta to the mid-thoracic aorta. Aortography demonstrated a flap at the terminal portion of this aneurysmal dilatation and grade II (Sellars) aortic regurgitation. There was a pressure difference of 80 mmHg between the parts abutting cranial and caudal sides of the flap. A surgical operation was, then, performed to correct the pressure difference. The dissected wall was extruded toward the aortic lumen creating a flap (2 cm in length). This flap was resected and an artificial graft was inserted. Histologically, the flap consisted of adventitia, media and intima.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Eosinophilic lymphofolliculosis (Kimura's disease) complicated with aortitis syndrome].

A 47 year-old-man was admitted because of mild congestive heart failure. A differential white blood cell count revealed eosinophilia and a biopsied inguinal lymph node showed well-developed lymphoid follicles with germinal centers, eosinophil infiltration and marked proliferating vessels with swollen endothelial cells. He was diagnosed as eosinophilic lymphfolliculosis (Kimura's disease). He also was hypertensive and vascular murmur was pointed out in his abdomen. Angiography revealed the complication of aortitis syndrome. Renal complication in eosinophilic lymphfolliculosis was reported in some cases and it was though that the disease had a clinical aspect of systemic diseases. The complication of aortitis syndrome in our case is interesting from a point of view of eosinophilic lymphfolliculosis as a systemic disease.

Angiolymphoid Hyperplasia with Eosinophilia↗

[A case report of aortitis syndrome with massive aortic regurgitation (AR), and ostial stenosis of the right coronary artery].

A 55-year-old female with massive aortic regurgitation and ostial stenosis of the right coronary artery due to aortitis syndrome was reported. The patient was admitted to the hospital with anterior chest pain and dyspnea on exertion. Retrograde aortogram showed massive aortic regurgitation and selective coronary angiogram revealed ostial stenosis of the right coronary artery. She was treated with aorto-coronary bypass (A-C bypass) and aortic valve replacement (AVR) with St. Jude Medical prosthetic valve. At operation, ostial stenosis of the right coronary artery due to aortitis syndrome was confirmed. Aortic valve replacement with a prosthetic valve and saphenous vein grafting to the distal right coronary artery were performed. Steroid therapy was started immediately after the operation. She recovered well and no complications was recognized after the operation.

Aortic Arch Syndromes↗

Cutaneous extravascular necrotizing granuloma in a patient with Takayasu's aortitis.

Cutaneous extravascular necrotizing granulomas (Churg-Strauss granulomas) have been noted in several systemic diseases, most often those producing or associated with systemic vasculitis. A young man with the clinical and histopathologic features of Takayasu's aortitis developed typical extravascular necrotizing granulomas of the skin. Takayasu's aortitis, like allergic granulomatosis and Wegener's granulomatosis, is a granulomatous vasculitis syndrome associated with the cutaneous Churg-Strauss granuloma.

Adult↗

[Cardiovascular involvement in the aortitis syndrome: a study using digital subtraction angiography].

Cardiovascular involvement in 22 cases with clinically confirmed aortitis syndrome was assessed using digital subtraction angiography (DSA) with an ADAC DPS-4100 system added to Siemens Angioskop A. The aorta and its branches, and the pulmonary vessels were examined for the extent, severity, and type of involvement. Left ventriculography was also performed for all patients. Digital subtraction angiograms of diagnostic quality were obtained in all the patients. Significant arterial narrowing was demonstrated by DSA in at least one vascular segment of each patient. DSA showed 32 total and 43 subtotal occlusions in 484 segments. DSA also imaged aneurysmal dilatation of the aortic root in one patient; an aneurysm of the innominate artery in two; a large anastomotic vessel connecting the superior and inferior mesenteric arteries in three; diffuse hypokinesis of the left ventricular wall in one; and stenosis of the pulmonary arterial branches in four patients. Clinical symptoms and signs did not correlate closely with the findings of DSA. It is concluded that the radiographic imaging of DSA is adequate and can be used to replace that of conventional angiography in evaluating the aortitis syndrome.

Adolescent↗

[A case of coronary artery bypass grafting using patch mounted graft technique for aortitis syndrome with left coronary ostial stenosis].

Coronary artery bypass grafting (CABG) was performed on a 36-year-old female for treatment of aortitis syndrome with left coronary ostial stenosis. The left anterior descending artery was anastomosed with the left internal thoracic artery. The circumflex artery was anastomosed with the saphenous vein. In the proximal anastomosis, the ascending aorta was sutured with a gelatin sealed knitted Dacron graft patch and anastomosed with the saphenous vein to prevent late occlusion due to intimal proliferation. Graft angiography 40 days and one year after the operation showed good patency of both grafts. Patch mounted graft technique seemed to be suitable for CABG in aortitis patients with the high incidence of late occlusion.

Adult↗

[Successful surgical repair of composite graft detachment occurred 5 months after combined Bentall's operation and graft replacement with active aortitis syndrome].

Ninteen-year-old male with annuloaortic ectasia and resultant massive aortic valvular regurgitation, along with aortic aneurysmal dilatation extending from the root to the distal arch, underwent surgical repair at our hospital. Operative procedure comprised composite graft replacement of aortic valve-ascending aorta (Bentall's operation with Piehler's modification), combined with the aortic arch replacement with reconstruction of the neck vessels. Physical, laboratory, and histopathological findings revealed active aortitis syndrome as the etiology of this aortic disease. Five months after operation, this patient was readmitted because of his anemia and complaint of dyspnea. Two days after admission, his condition rapidly deteriorated into pulmonary edema with fall of blood pressure to 70 mmHg. Fluoroscopic examination taken after emergency intubation and mechanical ventilatory support showed malfunction of the aortic valve prosthesis. Circulatory assist was accomplished with intraaortic balloon counterpulsation and percutaneous cardiopulmonary bypass support, and emergent operation was carried out. On reentry into the anterior mediastinum, expansion of the aortic wall wrapping the graft was found. Incision into the wrapped aortic wall revealed dehiscence of the composite graft from the aortic annulus at the sites of right coronary cusp and noncoronary cusp position. Pooled blood between the graft and the wrapped aortic wall compressed the composite graft, resulting in tilting of the bi-leaflet mechanical valve prosthesis and restraint of the motion of one occluder. Retachment of the composite graft to the annulus from outside of the wrapped aortic root wall by seven felt-buttressed mattress sutures of 3-0 polypropylene was performed. Postoperative course was smooth and successive strict antiinflammatory therapy with corticosteroid has successfully controlled the aortitis and the patient has been well after a follow-up of 18 months.

Adult↗

[CABG performed for LMT orifice stenosis 15 years after AVR in a case of aortitis syndrome].

The case is a 54-year-old female at age 39 years, she underwent aortic valve replacement (AVR) for aortic valve regurgitation (AR) due to aortitis syndrome at a certain hospital. At the 15th year after AVR she was diagnosed with unstable angina due to LMT orifice stenosis and underwent CABG at this hospital. A distal side of anastomosis was performed on the LAD branch seg 6, an artificial vascular patch was placed for the proximal side of anastomosis to prevent inflammation of the aortic wall from spreading to the proximal side of anastomosis. On the postoperative cardiac catheter examination, the graft was found to be patent well. In the case of CABG complicated with aortitis syndrome, stenosis and obstruction of the proximal side of anastomosis long time after surgery pose a problem. However, the method so as to perform a proximal anastomosis by placing an artificial vascular patch on the aortic wall is considered to be one of the useful means which can avoid such a problem.

Angina, Unstable↗

Salmonella aortitis. A report of a successfully treated case with a comprehensive review of the literature.

A successfully treated case of Salmonella infection of the thoracic aorta is described. Salmonella enteritidis was isolated from blood cultures and from operative cultures of the aorta. Computed tomography suggested the site of infection, and the resection of the thoracic aortic aneurysm with specific antibiotic therapy resulted in a satisfactory outcome. Although once invariably fatal, improved diagnostic roentgenographic techniques, more potent antibiotics, and surgical advances have resulted in at least 45 reported survivors over the last 15 years. The pathogenesis, clinical and laboratory characteristics, and the treatment of aortitis due to Salmonella species are reviewed in 98 cases reported in the English-language literature since 1948. Early diagnosis and a combination of prompt surgical intervention and antibiotic therapy are essential for survival.

Aneurysm, Infected↗

Progression of Takayasu's aortitis in a young Japanese woman: serial angiographic study.

Progression of Takayasu's aortitis was observed by serial angiography over 2 years. Acute aortic regurgitation due to marked dilatation of the proximal ascending aorta was followed by aneurysmal dilatation of the distal ascending aorta. Aneurysmal dilatation of the carotid arteries then developed, with subsequent obstruction of both the bilateral vessels by thrombosis.

Adult↗

Value of F-18 FDG hybrid camera PET and MRI in early takayasu aortitis.

Takayasu aortitis (TA) is a chronic inflammatory and fibrotic vasculitis of large- and medium-sized arteries. Early stages of the disease show a panarteritis and inflammatory wall thickening of the aorta and its branches, whereas advanced (fibrotic) stages comprise stenosis, aneurismatic transformation and occlusion. Magnetic resonance imaging visualises early-stage disease with high accuracy and is considered to be the method of choice in the diagnosis of TA. The aim of this article is the detailed comparison of FDG-PET performed with a hybrid camera and MR imaging in five patients with early TA. Five patients (median age 60 years) were enrolled during an ongoing prospective study on [18F]2'-deoxy-2-fluoro-D-glucose (FDG) hybrid camera PET in patients with fever of unknown origin (FUO). These patients underwent MR imaging after establishing the diagnosis of TA. Abnormal FDG uptake in the wall of the aorta was noted in all patients. The bracheocephalic artery and the common carotid arteries were visualized in 3 cases. Increased uptake of the subclavian artery was found in 3 patients and in 4 patients pathological uptake was noted in the ilio-femoral vessels. Of 34 vascular regions studied, 26 (76%) showed elevated FDG uptake. On transversal MR images vessel wall thickening and contrast enhancement of the thoracic aorta was found in 4 patients (ascending aorta/aortic arch: n=2; descending aorta: n=3; abdominal aorta: n=1). Additionally, vessel wall pathologies of the subclavian and the common carotid arteries could be shown in 1 patient and in another patient in the ilio-femoral arteries. No abnormalities were found using contrast-enhanced MR angiography. Of 28 vascular regions studied, 9 (32%) showed vasculitis on MRI. The FDG-PET is a suitable whole-body screening method in the primary diagnosis of early TA, especially in those cases with early disease that present with uncharacteristic symptoms such as FUO. Both MRI and MRA remain indispensable in the exact determination of the pathomorphological changes and in the documentation of complications such as stenosis, aneurismatic transformation and occlusion.

Aged↗

Evolution of salmonella aortitis towards the formation of abdominal aneurysm.

We present a case of salmonella aortitis with necrosis of the arterial wall and its evolution to the formation of an abdominal aneurysm over a 6-day period. Computed tomography was performed before and after the development of a mycotic aneurysm. Presurgical aortography was also performed. The findings obtained give a dynamic concept to this pathology and show its rapid evolution in a short period of time.

Aneurysm, Infected↗

Etiologic aspects of coagulopathy in Takayasu's aortitis.

Sixteen patients with Takayasu's aortitis were studied to evaluate the significance of coagulopathy in the etiology. In the initial stage of the disease (three cases) characterized by severe inflammatory symptoms with aortic lesions localized in the thoracic portion, marked hyperfibrinogenemia, and hypofibrinolytic activity of the plasma were observed. In the intermediate stage (five cases) characterized by moderate inflammation and aortic lesions expanded into thoracic and abdominal portions, hyperfibrinogenemia and hypofibrinolytic activity were also seen; in three of these cases, hypercoagulability was evident because of the presence of fibrinogen derivatives having gamma-gamma-diads in the plasma. When the inflammation subsided after the establishment of aortic lesions, the end-stage (eight cases), hyperfibrinolytic activity with normal plasma fibrinogen was observed. It is suggested that hypercoagulability may be in response to the arterial disease and may possibly play a part in the development of this disorder.

Adrenal Cortex Hormones↗

The surgical treatment of aortic regurgitation secondary to aortitis.

We describe the operative and perioperative management of 11 patients with aortic regurgitation due to aortitis. All patients required aortic valve replacement because of severely uncoapted cusps secondary to dilatation of the ascending aorta. The right coronary ostium was narrowed in 5 patients and consequently necessitated a smaller coronary tip for the administration of cardioplegic solution. To implant the prosthetic valve, pledgeted 2-0 Tevdek sutures were placed through the aortic valve annulus either from the ventricular side or from outside the aortic wall. Steroids were administered to 4 patients preoperatively and 8 patients postoperatively. Postoperative dehiscence of the prosthesis was seen in 1 of the 3 patients not given any steriods. We conclude that it is important to arrest the inflammatory reaction before operation and if the aortic valve must be replaced, to reinforce the implanted prosthesis with pledgeted sutures. Also, we suggest the possible importance of steroid therapy.

Adult↗

Indium-111 labelled leucocyte uptake in aortitis.

Inflammatory conditions of the aorta may present with non-specific clinical features, including unexplained fever. Indium-111 labelled leucocyte imaging may be performed in such patients to look for the presence of occult sepsis or to assess the activity of a known vasculitis. Of approximately 1100 patients to undergo leucocyte scintigraphy for these indications over a 5 year period, three had focal leucocyte uptake in the aorta. The final diagnoses were: (1) periaortitis in Wegener's granulomatosis; (2) aortic dissection in giant cell arteritis; and (3) streptococcal aortitis with impending rupture. In all three cases the uptake was initially not thought to be in the aorta, but in bowel, a paravertebral abscess and in the lumbar spine respectively. Further imaging with CT and MRI led to the correct diagnoses. As the aorta is a rare site of focal leucocyte uptake, errors in image interpretation are likely. The rapid diagnosis of inflammatory conditions of the aorta is essential, however, as they may be life-threatening if unrecognized; therefore awareness of the aorta as a potential site of uptake is important. Urgent referral for further imaging is imperative in these cases as a false or delayed diagnosis may lead to avoidable morbidity and mortality.

Aged↗

Nocardia asteroides aortitis with perforation of the aorta.

A 53-year-old man died of nocardial aortitis eight months after undergoing aortic valve replacement. Autopsy revealed vegetation in the area of a previous aortotomy incision and a small perforation of the aorta enclosed by pericardium immediately above the prosthetic valve ring. Prosthetic valve endocarditis was not present. Multiple splenic infarcts, microabscesses of both kidneys, and myocarditis were identified. Antemortem blood cultures and postmortem cultures of the aortic vegetation grew Nocardia asteroides.

Aortic Rupture↗