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Three cases of giant cell arteritis involving the female genital tract of postmenopausal women are reported. The patients were 80, 64, and 57 years of age and presented with fatigue and anemia, fatigue and an abdominal mass, and fever and weight loss, respectively. Two of the patients had palpable pelvic masses; one had an ovarian mass visible on ultrasound examination. All three patients were anemic, and the erythrocyte sedimentation rate was elevated in the two women in whom it was tested. Exploratory laparotomy revealed ovarian tumors in two patients; one had a mucinous cystadenoma, and one had bilateral ovarian fibromas. The third patient had a cyst of the rete ovarii. Extensive giant cell arteritis of the small to medium-sized arteries was found unexpectedly in the ovaries and fallopian tubes of two patients who had prior hysterectomies and in the ovaries, fallopian tubes, and uterus of one patient. One patient was treated postoperatively with prednisone with improvement of symptoms and a decrease in the erythrocyte sedimentation rate. Of the two patients who received no therapy, one was found to have a thoracic aortic aneurysm 5 years postoperatively, and the other was alive without symptoms 17 years after the operation. Giant cell arteritis of the female genital tract is a rare finding in elderly women and may occur as an isolated finding or as part of generalized giant cell arteritis.
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PMID:Giant cell arteritis of the female genital tract. A report of three cases. 376 47

A 47-year-old woman was admitted to our hospital for repair of a ruptured thoracic aortic aneurysm. Her post-operative course was uneventful, but she had persistent complaints of anorexia and general fatigue that failed to improve. Thirty-one days after surgery, she complained of severe abdominal pain. Abdominal computed tomography (CT) and X-ray revealed extensive necrosis of the visceral organs. She underwent emergency abdominal exploration and was found to have necrosis of the liver, gallbladder, stomach, entire small bowel and colon. The extensive necrosis made resection of the involved organs unfeasible. The patient died one day after exploratory laparotomy.
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PMID:Acute extensive necrosis of the visceral organs after repair of a ruptured thoracic aortic aneurysm. 822 10

We report two cases of chronic aortic dissection whose prominent features were characterized by systemic symptoms (prolonged low grade fever, fatigue, weight loss) and biological acute phase response. In the first patient, a surgical repair of the descending thoracic aorta aneurysm was performed, allowing the disappearance of general manifestations, whereas spontaneous resolution occurred in the second case. Although aortic dissection is usually an acute and highly symptomatic event, the disorder can present--albeit rarely--as systemic illness and fever of unknown origin.
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PMID:Chronic aortic dissection masquerading as systemic disease. 956 1

Diagnosis is central to medicine. In spite of tremendous diagnostic technological advances, no infallible test exists and in the complex diagnostic process the physician may well get lost. The ultimate feedback on the accuracy of diagnosis is the autopsy. Five patients illustrate that the autopsy may disclose unexpected results. The first patient was a 9-year-old girl who suffered from daily abdominal spasmodic pain but each time recovered. She died suddenly; autopsy revealed intestinal intussusception. A 46-year-old man who was treated for hypertension developed pain in the chest and the lower back, but there were no other signs of myocardial infarction. He died suddenly; autopsy revealed a dissecting aortic aneurysm with rupture in the left pleural cavity. A 21-year-old woman, an excellent swimmer, drowned during a swim in the sea. Autopsy revealed severe widespread coronary disease with multiple myocardial infarction. A 32-year-old Surinam woman developed acute coma and died from cardiorespiratory arrest. At autopsy she had massive pulmonary embolism and generalized lymphadenopathy due to sarcoidosis. The last patient, a 32-year-old woman suffered from fatigue after her fourth child was born. She was admitted with severe dyspnoea and her chest X-ray showed interstitial fibrosis. She died presently and autopsy revealed metastatic colon carcinoma with pulmonary lymphangitis carcinomatosa. Systematic reviews of the results of autopsies show no decline in the percentage of false diagnoses and/or unexpected findings in spite of the enormous growth of the diagnostic armamentarium. Although we may radiologically 'slice' the body in incredible detail or investigate human cells at the molecular level, the autopsy has by no means become obsolete and is an invaluable tool for quality control and teaching.
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PMID:[Truth after death]. 1059 Jul 70

We reported a rare case of tuberculous aneurysm of the aorta managed successfully with urgent surgical therapy. A 35-year-old woman was admitted to our hospital complaining of fatigue and hemoptysis. Laboratory tests showed severe anemia, slight liver dysfunction, elevated level of C-reactive protein, and negative syphilis serologies. The chest roentgenogram revealed widening of right upper mediastinum, two nodular shadows in right middle lobe, and left-sided infiltration shadow with pleural effusion. The pleural effusion was bloody and its level of adenosine deaminase was normal. Culture of pleural effusion specimen remained negative. A computed tomography scans of the chest revealed an aortic aneurysm on the aortic hiatus. Rapid increase in pleural effusion was followed by hemothorax a few hours later. After operation, she received antituberculosis therapy. Histopathologically, the resected lung showed inflammatory process including granulation of giant cells and epithelioid cells. The specimens of the aortic aneurysm revealed rupture of whole layer of aortic wall and inflammatory cell infiltrations. These findings suggested that the case to be a tuberculous aneurysm of the aorta. Therefore, we diagnosed the case as the rupture of tuberculous aneurysm of the aorta.
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PMID:[A case of tuberculous aneurysm of the aorta]. 1110 73

Although the technical success of stent-graft implantation is established and relatively safe, data on the long-term safety and efficacy of endovascular repair are just emerging. Because several late complications of aortic stent-graft placement have been observed, life-long follow-up remains essential. Imaging methods form an integral part of every stage of endovascular aortic aneurysm repair. The current imaging strategy should include initial plain films, CT angiography, and color-coded Duplex sonography. Plain films are an excellent means to detect migration, angulation, kinking, and structural changes of the stent mesh, including material fatigue, at follow-up. Helical CT angiography is considered a potentially revolutionary method for the noninvasive complete postprocedural assessment of aortic sten-grafting. Current data justify the use of biphasic C angiography as the postprocedural imaging technique of choice in most patients [118]. Ultrasound offers the advantages of low cost and lack of radiation exposure. High-quality ultrasound reliably excludes endoleaks in patients after stent-grafting of AAAs. There is a substantial variability, however, in measuring the diameter of aneurysm sacs; thus, confirmation using an alternative study is prudent in cases that demonstrate a significant change in size during follow-up. MR angiography serves as an attractive alternative to CT angiography in patients with impaired renal function or known allergic reaction to iodinated contrast media. With current techniques, the visualization of aortic stent-grafts (with the exception of stainless-steel-based devices) is sufficient with MR angiography. There is evidence that MR imaging is superior to CT angiography in detecting small type 2 endoleaks or for excluding retrograde perfusion in patients with suspected endotension. The role of diagnostic catheter angiography is limited to assessment of vascular pathways in equivocal cases or for suspected endotension. Currently, a consensus view about postprocedural management after aortic stent-graft implantation is lacking. The authors propose performing a baseline CT angiography at discharge and a biphasic CT angiography and Duplex ultrasound scan at three months. In patients with no evidence of an endoleak, CT angiography, plain film and Duplex sonography (abdomen) should be repeated every year after endovascular repair. If an endoleak is present at follow-up, immediate appropriate treatment should be initiated.
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PMID:Imaging of aortic stent-grafts and endoleaks. 1217 Nov 86

We report the case of a 61-year-old female, who presented with a history of chronic fatigue, dyspnea on exertion, a widened mediastinum with tracheal deviation on chest X-ray, and a neck mass. After a diagnostic workup, the patient was found to have a paratracheal mass extending into the chest in addition to a 6.5 cm ascending aortic aneurysm with aortic insufficiency, and a 70% stenosis of the right coronary artery. She underwent successful resection of a substernal goiter via a neck incision facilitated by a previously performed sternotomy for a concomitant ascending aortic root replacement and a bypass utilizing the RIMA to the distal RCA.
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PMID:Management of an ascending aortic aneurysm with coronary artery disease and tracheal compression from a substernal goiter. 1572 45

The association between dermatomyositis and restrictive cardiomyopathy has not been reported before. We present here the clinical, echocardiographic and muscle biopsy data for a patient with dermatomyositis and restrictive cardiomyopathy. In a 78-year-old male with a history of arterial hypertension, recurrent episodes of atrial fibrillation and syncopes, rupture of an infra-renal aortic aneurysm with complications (recurrent QT-prolongation, lumbo-sacral plexopathy, transient ischaemic attack, peripheral embolism), monoclonal gammopathy, subdural haematoma, focal seizures, megaloblastic anaemia, leucopenia, eosinophilia, elevated muscle enzymes and increasing tiredness, dermatomyositis was diagnosed upon clinical presentation, muscle enzyme and muscle biopsy findings. Cardiological examination revealed atrial fibrillation, left anterior hemiblock and restrictive cardiomyopathy. After the exclusion of various differentials for restrictive cardiomyopathy, a causative relationship between restrictive cardiomyopathy and dermatomyositis was assumed. This case suggests the need for suspecting restrictive cardiomyopathy in patients with dermatomyositis. Patients with dermatomyositis should undergo a comprehensive cardiological investigation as soon as the neurological diagnosis is established.
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PMID:Restrictive cardiomyopathy in dermatomyositis. 1676 71

Pseudoaneurysm of the infrarenal aorta due to Behcet s disease is very rare. We report a case of pseudoaneurysm associated with Behcet s disease in a 17-year-old boy with a recurrent right ventricular thrombus successfully treated with revascularization using arterial homograft patch. Echocardiography examination revealed a right ventricular mass, thought to be a thrombus in an unusual location. Postoperatively, the findings of the pathologic examination confirmed the mass as a thrombus. When the patient was subsequently re-admitted to the emergency unit with complaints of severe abdominal pain, fever, fatigue, sinus tachycardia, and a pulsating and tender abdominal mass, a right ventricular thrombus and a large pseudoaneurysm of the abdominal aorta were found on echocardiography and angiography. The patient underwent resection of the aortic aneurysm and aortoplasty, using arterial homograft patch, and received immunosuppressive and anticoagulation therapy. The thrombus of the right ventricle disappeared 4 months later. This case indicates that a right ventricular thrombosis in Behcet s disease may be managed by medical therapy.
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PMID:Arterial homograft and medical therapy in pseudoaneurysm of infrarenal aorta concomitant with recurrent right ventricular thrombus in Behcet's disease. 1695 82

Patent arterial duct (PAD) is a congenital heart abnormality defined as persistent patency in term infants older than three months. Isolated PAD is found in around 1 in 2000 full term infants. A higher prevalence is found in preterm infants, especially those with low birth weight. The female to male ratio is 2:1. Most patients are asymptomatic when the duct is small. With a moderate-to-large duct, a characteristic continuous heart murmur (loudest in the left upper chest or infraclavicular area) is typical. The precordium may be hyperactive and peripheral pulses are bounding with a wide pulse pressure. Tachycardia, exertional dyspnoea, laboured breathing, fatigue or poor growth are common. Large shunts may lead to failure to thrive, recurrent infection of the upper respiratory tract and congestive heart failure. In the majority of cases of PAD there is no identifiable cause. Persistence of the duct is associated with chromosomal aberrations, asphyxia at birth, birth at high altitude and congenital rubella. Occasional cases are associated with specific genetic defects (trisomy 21 and 18, and the Rubinstein-Taybi and CHARGE syndromes). Familial occurrence of PAD is uncommon and the usual mechanism of inheritance is considered to be polygenic with a recurrence risk of 3%. Rare families with isolated PAD have been described in which the mode of inheritance appears to be dominant or recessive. Familial incidence of PAD has also been linked to Char syndrome, familial thoracic aortic aneurysm/dissection associated with patent arterial duct, and familial patent arterial duct and bicuspid aortic valve associated with hand abnormalities. Diagnosis is based on clinical examination and confirmed with transthoracic echocardiography. Assessment of ductal blood flow can be made using colour flow mapping and pulsed wave Doppler. Antenatal diagnosis is not possible, as PAD is a normal structure during antenatal life. Conditions with signs and symptoms of pulmonary overcirculation secondary to a left-to-right shunt must be excluded. Coronary, systemic and pulmonary arteriovenous fistula, peripheral pulmonary stenosis and ventricular septal defect with aortic regurgitation and collateral vessels must be differentiated from PAD on echocardiogram. In preterm infants with symptomatic heart failure secondary to PAD, treatment may be achieved by surgical ligation or with medical therapy blocking prostaglandin synthesis (indomethacin or ibuprofen). Transcatheter closure of the duct is usually indicated in older children. PAD in preterm and low birth weight infants is associated with significant co-morbidity and mortality due to haemodynamic instability. Asymptomatic patients with a small duct have a normal vital prognosis but have a lifetime risk of endocarditis. Patients with moderate-to-large ducts with significant haemodynamic alterations may develop irreversible changes to pulmonary vascularity and pulmonary hypertension.
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PMID:Patent arterial duct. 1959 90


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