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[ACC2011]二尖瓣乳头样纤维弹性组织瘤:青年中风的罕见原因

Fibroelastoma of the Mitral Valve: a Rare Cause of Stroke in the Young

作者:  ChauhanC,SethiN,PatelT,TalluriS   日期:2011/5/10 14:44:40

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Papillary fibroelastomas are benign cardiac tumors usually occurring in adults over the age of 50. We report a young woman with a fibroelastoma of the mitral valve who presented with right hemiparesis.

Abstract

Introduction:
    Papillary fibroelastomas are benign cardiac tumors usually occurring in adults over the age of 50. We report a young woman with a fibroelastoma of the mitral valve who presented with right hemiparesis.

Case Presentation:
    A 36-year-old African American woman presented with stroke-like symptoms. Past medical history was positive for hypertension and transient ischemic attack (TIA). Physical examination was significant only for right-sided hemi-paresis and hyper-reflexia as well as dysphasia.

    Lab work-up was normal. Head CT was negative for hemorrhage. MRI showed acute ischemia involving the left posterior frontal lobe and the left posterior insular cortex.

    Serum lupus anticoagulant and serum anti-phospolipid antibody titers were normal. Bilateral carotid Doppler studies ruled out stenosis. Transesophageal echocardiography (TEE) showed a mass of the anterior segment of the posterior mitral leaflet suggestive of fibroelastoma.

Discussion:
    Papillary fibroelastomas are rare cardiac tumors, roughly 7% of all primary cardiac tumors. It follows myxoma and lipoma in frequency. They can arise anyplace where endocardium is present but 90% are seen on valvular endothelium.

    Stroke or TIA is the most common presentation followed by myocardial infarction, pulmonary emboli, syncope and sudden death. Embolic events are more common as the tumor acts as a source for platelet thrombi formation. TEE is the diagnostic gold standard. The pathognomonic echocardiographic finding of fibroelastoma is its resemblance to a sea anemone because of numerous papillary fronds. Cardiac catheterization has no diagnostic role.

    Excision of the tumor with reconstitution or replacement of the involved valve is the primary mode of treatment. Decision to resect the tumor is independent of size, site and symptoms.

Conclusion:
    The diagnosis of papillary fibroelastomas should be considered in young individuals with an ischemic stroke as surgical resection can prevent future strokes.
 

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二尖瓣乳头样纤维弹性组织瘤中风

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