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[反复喘息和呼吸窘迫的罕见病因:弯刀综合征]

[A rare cause of recurrent wheezing and respiratory distress: Scimitar syndrome].

作者信息

Ergül Yakup, Nişli Kemal, Güler Nermin, Aydoğan Umrah

机构信息

Department of Pediatric Cardiology, Medicine Faculty of İstanbul University, İstanbul, Turkey.

出版信息

Turk Kardiyol Dern Ars. 2011 Oct;39(7):595-8. doi: 10.5543/tkda.2011.01581.

Abstract

Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung. We present a 15-month-old boy who was diagnosed with Scimitar syndrome after examinations for recurrent wheezing and respiratory distress. The chest radiograph showed a scimitar sign in the right hemithorax, obscuring the contours of the right atrium. Echocardiography showed dilatation of the right heart cavities and increased flow in the inferior vena cava, without a cardiac abnormality. The patient underwent cardiac catheterization for radiographic and hemodynamic evaluations, during which a scimitar vein was detected, draining the right pulmonary veins to the inferior vena cava. Coil occlusion was performed on the abnormal artery arising from the infradiaphragmatic aorta. The patient was referred to surgery for repair of the anomalous pulmonary venous return and resection of the sequestered pulmonary segment.

摘要

弯刀综合征的特征是右肺部分或全部肺静脉异常回流。我们报告一名15个月大的男孩,他因反复喘息和呼吸窘迫接受检查后被诊断为弯刀综合征。胸部X线片显示右半胸有弯刀征,模糊了右心房轮廓。超声心动图显示右心腔扩张,下腔静脉血流增加,无心脏异常。患者接受了心导管检查以进行影像学和血流动力学评估,在此期间检测到一条弯刀静脉,将右肺静脉引流至下腔静脉。对膈下主动脉发出的异常动脉进行了弹簧圈封堵。患者被转诊至外科进行异常肺静脉回流修复和隔离肺段切除术。

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