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病例报告:与先天性矫正型大动脉转位相关的异常情况:意料之外之事需有所预料。

CASE REPORT Anomalies Associated With Congenitally Corrected Transposition of Great Arteries: Expect the Unexpected.

作者信息

Parikh Valay, Shariff Masood A, Saiful Faisal B, Rizvi Syed Bilal, Goyal Nikhil, Asgarian Kourosh T, McGinn Joseph T, Snyder Thomas, Singh Vijay A

机构信息

Departments of Internal Medicine.

出版信息

Eplasty. 2013;13:e6. Epub 2013 Jan 24.

Abstract

OBJECTIVE

Congenitally corrected transposition of great arteries (CCTGA) is characterized by atrioventricular and ventriculoarterial discordance. Characterizations of these anomalies are important because they may influence surgical approach and management.

METHODS

We present a case of newly diagnosed CCTGA at the age of 50. He presented with sudden onset of shortness of breath for the first time and was diagnosed with CCTGA. Echocardiogram, magnetic resonance imaging, and cardiac catheterization were utilized to elucidate the pathology.

RESULTS

Intraoperatively, patient's CCTGA and ventricularization of the right ventricle were confirmed. The severe systemic atrioventricular valve regurgitation was replaced with a bioprosthetic valve (Medtronic Mosaic No. 29) with placement of epicardial ventricular leads for possible future placement of automatic implantable cardioverter defibrillators. Pathology report confirmed a degeneration of the systemic atrioventricular valve.

CONCLUSIONS

Significant coronary artery anomalies have also been described in literature with CCTGA. The variances encountered in this case are excellent examples of the intricacies associated in diagnosis and surgical care in patients with CCTGA.

摘要

目的

先天性矫正型大动脉转位(CCTGA)的特征是房室和心室动脉不一致。这些异常的特征很重要,因为它们可能会影响手术方法和管理。

方法

我们报告一例50岁新诊断的CCTGA病例。他首次出现突发气短,被诊断为CCTGA。使用超声心动图、磁共振成像和心导管检查来阐明病理情况。

结果

术中证实患者为CCTGA及右心室心室化。严重的体循环房室瓣反流被一个生物瓣膜(美敦力Mosaic 29号)置换,并放置了心外膜心室导联,以便未来可能植入自动植入式心律转复除颤器。病理报告证实体循环房室瓣退变。

结论

文献中也描述了CCTGA患者存在显著的冠状动脉异常。该病例中遇到的差异是CCTGA患者诊断和手术治疗复杂性的极佳例证。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/587b/3556635/613424e2b4af/eplasty13e06_fig1.jpg

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