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1/392. mitral valve repair for anterior leaflet papillary fibroelastoma: two case descriptions and a literature review.

    Cardiac papillary fibroelastomas are rare cardiac tumors and have been considered a 'benign' incidental finding that may have significant clinical manifestations. In this paper we report two cases of mitral valve fibroelastoma: one was discovered by chance with transthoracic echocardiography in a young healthy man, the other was an intraoperative incidental finding in a middle aged man with a recent history of acute myocardial infarction. The mitral valve was repaired in both cases after excising the tumor. The patients did well and remain asymptomatic. A literature review was compiled which comprises previous case reports of 34 patients with mitral valve papillary fibroelastomas. Most were asymptomatic, but when symptoms occurred, they could be disabling, such as stroke, cardiac heart failure, myocardial infarction, and sudden death. Papillary fibroelastoma is amenable to simple surgical excision or in addition to mitral valve repair or replacement. recurrence has not been reported.
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2/392. Symptomatic mitral myxomatous transformation in the elderly.

    The clinical and pathological features of four patients with intractable heart failure, due to myxomatous change in the mitral valve, are described. It is suggested that this change may represent a response of ageing connective tissue to mechanical stress.
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3/392. Chordal rupture: a common complication of myxomatous degeneration of the mitral valve.

    In two patients with a clinical picture of acute mitral insufficiency, the presence of chordal rupture secondary to myxomatous degeneration of the mitral valve was disclosed during surgery. There was no evidence of previous rheumatic valvulitis, subacute bacterial endocarditis or other etiologies. It appears from the literature and from the cases described that ruptured chorda tendinea is a not uncommon complication of myxomatous transformation of the mitral valve. While this fact has been mentioned in anatomophological reports, clinicians are less aware of the association. In the absence of supportive evidence for a rheumatic or arteriosclerotic etiology, a clinical picture of acute mitral insufficiency should suggest ruptured chorda tendinea secondary to myxomatous degeneration of the mitral apparatus. This is particularly true in older patients, especially among those followed for a midsystolic click-late systolic murmur or an apical pansystolic murmur of pure mitral regurgitation.
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keywords = murmur
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4/392. osteogenesis imperfecta with mitral insufficiency due to ballooning of the mitral valve. A case report.

    A further case of osteogenesis imperfecta with valvular heart disease is added to the 12 already reported in the literature. The presence of a dilated mitral annulus and a ballooned mitral leaflet in this case together with the findings reported in the literature leave little doubt as to the relationship between the valvular lesion and the underlying connective tissue disorder.
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5/392. Partial left ventriculectomy in a two-year-old girl with dilated cardiomyopathy.

    We report the case of a two-year-old girl with end-stage dilated cardiomyopathy who was a status I heart transplant candidate. Partial left ventriculectomy and novel mitral valve repair were performed. Early hemodynamic and functional improvements were maintained at the 18-month follow-up.
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6/392. Cardiac involvement in coffin-lowry syndrome.

    coffin-lowry syndrome is an X-linked recessive syndrome of mental retardation, characteristic facies and skeletal anomalies. In one patient with the syndrome, we observed early recurrent episodes of congestive heart failure with intercurrent normalization and the late development of mitral insufficiency due to annular dilation and congenital abnormalities of the valve apparatus. This unusual course of cardiac involvement, the non-adaptation of the left ventricular contractility to the aggravation of the mitral insufficiency and the postoperative persistence of the ventricular dysfunction, underline the possible role of an associated primary myocardial disease. This clinical observation demonstrates clearly that a mitral valve malformation can occur in patients with the syndrome, but also the role of a dilated cardiomyopathy, which can be secondary to the mitral regurgitation, but is more likely a myocardial disorder occurring as part of the syndrome.
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keywords = heart
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7/392. Post-parturition infectious endocarditis in a patient with a normal mitral valve.

    A 29-year-old woman with no history of heart disease was admitted for the treatment of congestive heart failure. Six months earlier, she had given birth, then 20 days later developed a fever and cardiac failure ensued. An echocardiogram demonstrated severe mitral valve regurgitation. Her blood cultures were positive, and we made a diagnosis of mitral valve regurgitation due to infectious endocarditis. Despite treatment for congestive heart failure and antibiotic therapy, resulting in negative blood cultures, her congestive heart failure did not improve, and vegetation on the mitral valve was observed by echocardiography. We successfully removed the infected tissue with mitral valve plasty.
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ranking = 4
keywords = heart
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8/392. Malfunction of a Bjork-Shiley prosthetic heart valve in the mitral position producing an abnormal echocardiographic pattern.

    This report presents an echocardiographic study of a patient with fibrous ingrowth about the sewing ring and hinges of a Bjork-Shiley valve in the mitral position. The valve was obstructed and produced a distinct motion pattern. In addition to having a rounded diastolic motion, the disk excursion was decreased.
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keywords = heart
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9/392. Valve replacement for appetite suppressant-induced valvular heart disease.

    Valvular heart disease associated with the use of appetite-suppressant medication is a recently described clinical entity. Although the mechanism of valvular injury remains elusive pathologically, the valvular abnormalities resemble those observed in carcinoid syndrome. The incidence of clinically evident valvular heart disease is low with short-term (less than 3 months) exposure to appetite-suppressant drugs. Prolonged exposure to higher doses in addition to combination drug therapy confers an excess risk for valvular pathologic changes. We report the case of a patient with severe mitral regurgitation who had short-term exposure (3 weeks) to the combination of fenfluramine (20 mg) and phenteramine (15 mg).
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ranking = 6
keywords = heart
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10/392. Acute mitral regurgitation due to chordal rupture in a patient with neonatal marfan syndrome caused by a deletion in exon 29 of the FBN1 gene.

    The neonatal marfan syndrome is an autosomal dominantly inherited disease with an extremely poor prognosis. This report gives a clinical and echocardiographic description of an infant with a mutation in exon 29 of the fibrillin-1 gene (FBN1), a region in which this severe form of marfan syndrome seems to cluster. The infant died at the age of 3 months due to severe acute mitral regurgitation leading to intractable heart failure.
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keywords = heart
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