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原发孔型房间隔缺损合并永存左上腔静脉开口于无顶冠状静脉窦——一种罕见的情况。

Ostium primum atrial septal defect with persistent left superior vena cava opening into unroofed coronary sinus-A rare entity.

作者信息

Garg Ashok, Agrawal Deepak, Mishra Deepika, Sharma Gyarsi Lal

机构信息

Department of Preventive and Non invasive Cardiology, Jaipur Heart Institute, Jaipur, India.

Jaipur Heart Institute, Jaipur, India.

出版信息

Echocardiography. 2019 Jul;36(7):1421-1422. doi: 10.1111/echo.14408. Epub 2019 Jun 19.

Abstract

Raghib syndrome is a rare developmental complex consisting of termination of the left superior vena cava in the left atrium, absence of the coronary sinus, and an atrial septal defect commonly located at the posterior-inferior angle of the atrial septum. This complex was considered unique to Raghib syndrome; however, cases with a normal atrial septum have been reported where the orifice of the unroofed coronary sinus functions as the inter-atrial communication. Our patient demonstrated an isolated persistent left superior vena cava draining into the left atrium through unroofed coronary sinus and presence of ostium primum atrial septal defect.

摘要

拉吉卜综合征是一种罕见的发育复合体,由左上腔静脉汇入左心房、冠状窦缺如以及通常位于房间隔后下角的房间隔缺损组成。这种复合体曾被认为是拉吉卜综合征所特有的;然而,已有报道称存在房间隔正常的病例,其中无顶冠状窦口起到了心房之间的交通作用。我们的患者表现为孤立的持续性左上腔静脉通过无顶冠状窦汇入左心房,并存在原发孔型房间隔缺损。

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