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下腔静脉和肝静脉先天性异常引流至左心房并伴有长QT综合征:多模态成像、陷阱与注意事项

Congenital anomalous drainage of the inferior vena cava and hepatic veins into the left atrium associated with long QT syndrome: Multimodality imaging, pitfalls, and caveats.

作者信息

Malakan Rad Elaheh, Radmehr Hassan, Taghizadeh Ashkan, Pouraliakbar Hamidreza

机构信息

Tehran University of Medical Sciences, Tehran, Iran.

Iran University of Medical Sciences, Tehran, Iran.

出版信息

Echocardiography. 2023 Oct;40(10):1127-1136. doi: 10.1111/echo.15673. Epub 2023 Aug 17.

Abstract

Isolated congenital anomalous drainage of the inferior vena cava (IVC) and partial hepatic veins (HV) into the left atrium (LA) via an interatrial communication (IAC), associated with the normal connection of the IVC to the right atrium is exceedingly rare. Therefore, there is a dearth of knowledge regarding the management of these cases. To date, there has been no report of abnormal IVC drainage caused by abnormal IVC-IAC alignment. Much more frequently, patients have an abnormal connection or abnormal drainage mediated by a persistent Eustachian valve that allows blood to pass from the inferior vena cava to the left atrium. Herein, we report an 8-year-old boy with anomalous IVC and hepatic vein drainage into the LA due to IVC-IAC malalignment. We describe the findings of multimodality imaging, including transthoracic, transesophageal, contrast, and speckle-tracking echocardiography, cardiac angiography, and cardiac magnetic resonance imaging. We go over the diagnostic and therapeutic pitfalls and caveats of this case that can apply to similar patients.

摘要

孤立性先天性下腔静脉(IVC)及部分肝静脉(HV)经房间隔交通(IAC)异常引流至左心房(LA),而IVC与右心房连接正常的情况极为罕见。因此,对于这些病例的处理缺乏相关知识。迄今为止,尚无因IVC-IAC排列异常导致IVC引流异常的报道。更为常见的情况是,患者存在由持续存在的欧氏瓣介导的异常连接或异常引流,使得血液从下腔静脉流入左心房。在此,我们报告一名8岁男孩,因IVC-IAC排列异常导致IVC及肝静脉异常引流至LA。我们描述了多种影像学检查结果,包括经胸、经食管、造影及斑点追踪超声心动图、心脏血管造影和心脏磁共振成像。我们回顾了该病例的诊断和治疗陷阱及注意事项,这些可应用于类似患者。

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