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镜像右位心及左位心合并房室不一致型双出口右心室：1例罕见病例报告

Atrioventricular Discordance with Double-Outlet Right Ventricle in Mirror Imagery and Levocardia: A Very Rare Case Report.

作者信息

Farruggio Silvia, Agati Salvatore, Calvaruso Davide, Mambro Corrado Di, Caruso Elio

机构信息

Mediterranean Pediatric Cardiology Center "Bambino Gesù", San Vincenzo Hospital, Taormina, ME, Italy.

出版信息

J Cardiovasc Echogr. 2020 Oct-Dec;30(4):227-230. doi: 10.4103/jcecho.jcecho_65_20. Epub 2021 Jan 20.

DOI:10.4103/jcecho.jcecho_65_20
PMID:33828947
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8021085/
Abstract

A newborn without prenatal diagnosis, with bronchial and abdominal situs inversus in levocardia, was referred to our hospital for accurate evaluation; echocardiography showed venoatrial connections in mirror-image arrangement, atrioventricular (AV) discordance, and double-outlet right ventricle (DORV). Additional cardiac malformations were double upper caval district, atrial communication, subpulmonary interventricular communication, and moderate subvalvular and valvular pulmonary stenosis. Few days after birth, the patient presented low oxygen saturation and the heart team decided for a palliative surgery. We describe a very rare case in a newborn with bronchial-abdominal mirror imagery, AV discordance, and DORV in levocardia.

摘要

一名未进行产前诊断的新生儿，心脏位置正常但支气管和腹部脏器反位，被转诊至我院进行准确评估；超声心动图显示腔静脉与心房连接呈镜像排列，房室不一致，以及右心室双出口。其他心脏畸形包括双侧上腔静脉区、心房交通、肺动脉下室间隔交通，以及中度瓣膜下和瓣膜性肺动脉狭窄。出生几天后，该患者出现低氧饱和度，心脏团队决定进行姑息性手术。我们描述了一例非常罕见的新生儿病例，其具有支气管-腹部镜像、房室不一致和心脏位置正常的右心室双出口。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7b/8021085/48634e171c9a/JCE-30-227-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7b/8021085/977d63e78c75/JCE-30-227-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7b/8021085/7de2938c1933/JCE-30-227-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7b/8021085/afb71bb74a67/JCE-30-227-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7b/8021085/48634e171c9a/JCE-30-227-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7b/8021085/977d63e78c75/JCE-30-227-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7b/8021085/7de2938c1933/JCE-30-227-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7b/8021085/afb71bb74a67/JCE-30-227-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed7b/8021085/48634e171c9a/JCE-30-227-g004.jpg

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Atrioventricular Discordance with Double-Outlet Right Ventricle in Mirror Imagery and Levocardia: A Very Rare Case Report.镜像右位心及左位心合并房室不一致型双出口右心室：1例罕见病例报告
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本文引用的文献

1
Is an Appreciation of Isomerism the Key to Unlocking the Mysteries of the Cardiac Findings in Heterotaxy?对异构现象的认识是解开心脏异构现象相关发现之谜的关键吗？
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The Triply Twisted Heart: Cyanosis in an Adult With Situs Inversus, Levocardia, Double Outlet Right Ventricle, and Malposition of the Great Arteries.三扭结心脏：一名具有镜面右位心、左位心、右心室双出口及大动脉转位的成年患者的发绀情况
Cardiol Res. 2015 Dec;6(6):362-366. doi: 10.14740/cr440w. Epub 2015 Dec 16.
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为了区分所谓的内脏反位而确定支气管形态。
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Cardiovascular manifestations of heterotaxy and related situs abnormalities assessed with CT angiography.CT 血管造影评估的心脏转位和相关内脏位置异常的心血管表现。
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Isolated levocardia: prenatal diagnosis, clinical importance, and literature review.孤立性左位心：产前诊断、临床意义及文献综述
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Hearts with isomerism of the right atrial appendages - one of the worst forms of disease in 2005.右心耳异构的心脏——2005年最严重的疾病形式之一。
Cardiol Young. 2005 Dec;15(6):554-67. doi: 10.1017/S1047951105001708.
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Ventricular function after anatomic repair in patients with atrioventricular discordance.房室不一致患者解剖修复后的心室功能
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