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钙化性右心室纤维瘤导致流出道梗阻。1例成功切除病例报告。

Calcified right ventricular fibroma causing outflow obstruction. Report of a case with successful excision.

作者信息

Aryanpur I, Nazarian I, Razmara M, Sheikh M A, Khonsari S

出版信息

Am J Dis Child. 1976 Nov;130(11):1265-7. doi: 10.1001/archpedi.1976.02120120099020.

Abstract

A 5-year-old boy with clinical findings of pulmonic stenosis was found to have a large calcified mass in the right ventricular outflow region and a gradient of 120 mm Hg between the right ventricle and the pulmonary artery. At surgery, an ovalshaped tumor attached to the interventricular septum and obstructing the right ventricular outflow tract was removed. The child survived and is doing well. Histologically, the tumor had the characteristics of fibroma. A hemodynamic study three months after surgery showed almost complete abolishment of the gradient. To our knowledge this is the fifth reported case of calcified right ventricular fibroma with successful operation. In childhood intracardiac calcifications, together with obstruction, are highly suggestive of this lesion.

摘要

一名有肺动脉狭窄临床表现的5岁男孩,在右心室流出区域发现一个大的钙化肿块,右心室与肺动脉之间的压差为120毫米汞柱。手术中,切除了一个附着于室间隔并阻塞右心室流出道的椭圆形肿瘤。患儿存活且情况良好。组织学检查显示,该肿瘤具有纤维瘤的特征。术后三个月的血流动力学研究显示压差几乎完全消失。据我们所知,这是第五例报告的钙化性右心室纤维瘤成功手术的病例。在儿童心脏内钙化伴梗阻的情况下,高度提示这种病变。

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