Cases reported "Heart Valve Diseases"

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1/13. minocycline pigmentation of heart valves.

    minocycline, a derivative of tetracycline, is a broad spectrum antibiotic used in the treatment of gram-positive and gram-negative infections. Benitz et al. (1) were the first to report black discoloration of the thyroid gland in rats, dogs, and monkeys given minocycline. Since that time, there have been numerous reports in the literature describing minocycline related black pigmentation of the skin, thyroid gland, and other sites. We report an unusual case of minocycline induced pigmentation of the cardiac valves and coronary vessels. The pigment stained with Fontana-Masson and was reduced with bleaching. The exact nature of the pigment is unclear; however, various theories have been advocated. ochronosis is another cause of black pigmentation of the heart valves; the clinical history should allow distinction between the two.
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2/13. vasculitis masquerading as aortic valve endocarditis.

    Small vessel vasculitis and endocarditis can both present with multisystem involvement and may present a diagnostic dilemma. Renal and cardiac involvement is common in small vessel vasculitis and rarely small vessel vasculitis may cause heart block. When a patient presents with diffuse symptoms, deteriorating renal function, and heart block, endocarditis and vasculitis should be included in the differential diagnosis. The case is discussed of a man with a history of aortic valve endocarditis who presented again with similar symptoms, deteriorating renal function, and heart block. There was no evidence of aortic valve endocarditis with abscess formation. A renal biopsy confirmed small vessel vasculitis and the patient responded promptly to immunosuppressive treatment. Correct diagnosis is essential in such cases, as immunosuppression in true endocarditis can be catastrophic. In this case, with the correct diagnosis, immunosuppression proved life saving and prevented erroneous aortic valve surgery.
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3/13. Infective tricuspid valve endocarditis with pulmonary emboli caused by campylobacter fetus after tooth extraction.

    We describe a case of infective endocarditis caused by campylobacter fetus accompanied by pulmonary emboli. A 52-year-old man was referred to our hospital due to febrile temperatures with a history of dental treatment followed by eating raw meat. Computed tomography revealed multiple infiltrations and a nodule with low attenuation area and feeding vessels. A mobile mass, possible vegetation, attached to the tricuspid valve was detected by transthoracic echocardiography. Two blood cultures disclosed campylobacter fetus. Long-term antibiotic therapy was given, curing the infection with valvuloplasty. We presented the possibility that infective campylobacter fetus endocarditis after dental treatment was caused by eating raw meat.
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4/13. Bilateral semilunar valve disease in a child with partial deletion of the Williams-Beuren syndrome region is associated with elastin haploinsufficiency.

    elastin is an extracellular matrix protein that is the primary component of elastic fibers, and is expressed in the great vessels as well as the semilunar and atrioventricular valves. elastin haploinsufficiency, resulting from mutation or deletion of the elastin gene, is an important clinical problem that is typically characterized by arteriopathy. Herein is described a patient with elastin haploinsufficiency due to partial deletion of the Williams-Beuren syndrome region, resulting in bilateral semilunar valve disease and arteriopathy. Histochemical analysis of the aortic valve revealed decreased and disorganized elastin with loss of the normal trilaminar cusp organization. These findings suggest that elastin has a role in the pathogenesis of semilunar valve disease.
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5/13. Findings at necropsy in the heart of a patient with anticardiolipin syndrome.

    A 40 year old man with a history of myocardial infarction and hypertension presented with transient cerebral ischaemic attacks, aortic regurgitation, a raised erythrocyte sedimentation rate, and thrombocytopenia. The anticardiolipin syndrome was diagnosed and he was treated with prednisolone and warfarin. He died two years later after the development of acute heart failure. At necropsy his heart showed widespread arteriolar thrombosis without vasculitis, recanalised large vessel occlusion, and a "post-inflammatory" valvulitis of the aortic valve.
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6/13. Cardiovascular ochronosis.

    A 64-year-old man with alkaptonuric ochronosis required aortic valve replacement for severe aortic stenosis and single-vessel aortocoronary artery bypass grafting for a subtotally occluded obtuse marginal branch of the circumflex coronary artery. Operative findings included ochronosis of a partly calcified aortic valve and the aortic intima. The aortic valve and a punch biopsy specimen of the ascending aorta were removed at surgery and were studied with transmission electron microscopy and light microscopy. The ultrastructural studies of the aortic valve revealed intracellular and extracellular deposits of ochronotic pigment. A portion of the extracellular ochronotic pigment represented degenerated cells. Large deposits of extracellular ochronotic pigment were associated with areas of valvular calcification. Electron microscopic study of the aorta disclosed ochronotic pigment in macrophages and smooth-muscle cells. Aggregates of extracellular ochronotic pigment in the intima and media appeared to be in locations of necrotic cells. light microscopy also showed intracellular and extracellular deposits of ochronotic pigment. Our study suggests that extensive extracellular deposits of ochronotic pigment in the aortic valve may serve as a stimulus for dystrophic calcification. This may play a role in the development of aortic valve calcification and aortic stenosis associated with alkaptonuric ochronosis. To our knowledge, this is the first ultrastructural study of the aortic valve and aorta in alkaptonuric ochronosis.
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7/13. Diagnostic value of pulsed Doppler echocardiography in combined heart defects.

    26 patients with combined heart defects were examined by means of ultrasound methods (one-dimensional, two-dimensional and Doppler echocardiography). The results were compared with the results of clinical and roentgenological examinations. Taking into account literary data on high sensitivity and specificity of Doppler echocardiography, its informative value was assessed in cases when affection of two or more valves results in a balancing of haemodynamic changes. It was documented that pulsed Doppler EchoCG makes possible to assess the character and the direction of blood flow in different phases of the cardiac cycle in the heart cavities and the great vessels, and thereby provides additional information on the involvement of the valvular apparatus and on the state of haemodynamics. The sensitivity of this method is particularly high in the detection of regurgitant flow.
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8/13. An autopsied case of a two-month-old infant with granulomatous pancarditis having severe vasculitis and valvulitis.

    A 2-month-old female infant with pancarditis was reported. The patient died after a 26-day clinical course. The autopsy showed pancarditis, including inflammation in all 4 valves and chordae, fibrinoid vasculitis, pharyngolaryngitis and atrophy of the lymph nodes. Microscopic examination revealed proliferative inflammation in the endocardium, valve, myocardium, epicardium and coronary vessels. The histological findings suggested the etiology to be a rheumatic-type reaction at an extremely young age.
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9/13. Multiplane transesophageal echocardiography: our initial experience.

    We present our experience in the diagnostic assessment of a wide spectrum of cardiovascular disorders using multiplane transesophageal echocardiography (MP-TEE). Two hundred and seventeen patients in the age range of 11-71 years were subjected to MP-TEE from January to November 1993. The male:female ratio was 1.1:1. One hundred and ten patients had predominantly mitral valve disease of rheumatic origin, eleven had mitral valve prolapse, twenty patients had aortic valve disease and thirty seven patients had more than one valve involvement. Six patients with suspected prosthetic heart valve dysfunction and ten patients of hypertrophic cardiomyopathy were also studied. Two patients had unexplained pulmonary hypertension, three had pericardial disease and three had proximal aortic dissections. Twenty six patients with congenital heart disease were studied of which nineteen had atrial septal defects, one had corrected transposition of great vessels with pulmonic stenosis and one adult had Ebstein's anomaly of the tricuspid valve. In our experience, MP-TEE enhances the versatility of TEE by providing incremental diagnostic information and enhancing delineation of pathology. The procedure was well-tolerated and no complications occurred.
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10/13. aortic valve disease in patients with Wegener's granulomatosis.

    Wegener's granulomatosis usually affects the upper and lower respiratory tract as well as the kidney. Cardiac involvement is rare, although electrocardiographic abnormalities, coronary artery vasculitis, cardiac arrhythmias, and myocardial infarction have been described. We report two cases of aortic valve disease associated with Wegener's granulomatosis that were progressive despite clinical remission of Wegener's disease in both patients. One patient has undergone successful valve replacement and the other is currently awaiting surgery. Aortic valve histopathology showed myxoid degeneration that was most likely due to previously active vasculitis affecting the vessels of the aortic wall and valvular necrosis with subsequent progressive degeneration of the cusps.
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