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合并右心房血栓的埃布斯坦畸形

Ebstein Anomaly With Right Atrial Clot.

作者信息

Kumar Prakash, Singhal Gaurav, Sinha Santosh Kumar, Pandey Umeshwar, Thakur Ramesh, Varma Chandra Mohan

机构信息

LPS Institute of Cardiology, GSVM Medical College, Kanpur, UP, 208002, India.

出版信息

Cardiol Res. 2015 Oct;6(4-5):319-323. doi: 10.14740/cr409w. Epub 2015 Oct 25.

DOI:10.14740/cr409w
PMID:28197250
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5295571/
Abstract

Ebstein anomaly (EA) is a rare congenital malformation of the tricuspid valve (TV), often associated with other cardiac malformations, especially atrial septal defect/patent foramen ovale (PFO) which is present in 80-90% of patients and predisposes to paradoxical embolization. We describe the case of a 17-year-old female, who presented with worsening exertional dyspnea, fatigue and pedal edema and atrial fibrillation (AF). Transthoracic echocardiography showed EA with severely dilated right atrium (RA), small functional right ventricle (RV), low velocity flow across TV with spontaneous echo contrast and giant clot in RA. Fortunately for the patient, contrast and transesophageal echocardiography revealed an intact interatrial septum with no PFO preventing any paradoxical embolism from large clot in RA, more so in the background of AF. Important differential diagnosis of congenitally unguarded TV orifice was ruled out due to presence of septal and anterior leaflets of TV and associated chordae.

摘要

埃布斯坦畸形（EA）是一种罕见的三尖瓣先天性畸形，常与其他心脏畸形相关，尤其是房间隔缺损/卵圆孔未闭（PFO），80%至90%的患者存在该情况，且易发生反常栓塞。我们描述了一名17岁女性的病例，她出现劳力性呼吸困难、疲劳、足部水肿和房颤症状加重。经胸超声心动图显示埃布斯坦畸形，右心房（RA）严重扩张，功能性右心室（RV）小，三尖瓣处血流速度低且有自发显影，右心房内有巨大血栓。对患者来说幸运的是，造影和经食管超声心动图显示房间隔完整，无卵圆孔未闭，可防止右心房内大血栓发生任何反常栓塞，在房颤背景下更是如此。由于三尖瓣的隔叶和前叶以及相关腱索的存在，排除了先天性无保护三尖瓣口的重要鉴别诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/f10d2b625ba7/cr-06-319-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/5b24302e9ccb/cr-06-319-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/41e5d4c5929c/cr-06-319-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/1da30977cbba/cr-06-319-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/b0eb1922eac1/cr-06-319-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/310174ed1c5c/cr-06-319-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/d101077cb9c4/cr-06-319-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/f10d2b625ba7/cr-06-319-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/5b24302e9ccb/cr-06-319-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/41e5d4c5929c/cr-06-319-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/1da30977cbba/cr-06-319-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/b0eb1922eac1/cr-06-319-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/310174ed1c5c/cr-06-319-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/d101077cb9c4/cr-06-319-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8291/5295571/f10d2b625ba7/cr-06-319-g007.jpg

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本文引用的文献

1
Congenitally unguarded tricuspid valve orifice with a giant right atrium and a massive clot in an asymptomatic adult.
Indian Heart J. 2001 Jul-Aug;53(4):503-4.
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Echocardiographic spectrum of congenitally unguarded tricuspid valve orifice and patent right ventricular outflow tract.先天性无保护三尖瓣口及右心室流出道通畅的超声心动图表现谱
Int J Cardiol. 2000 Jul 31;74(2-3):153-7. doi: 10.1016/s0167-5273(00)00263-1.