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一例违背胚胎学的弯刀综合征罕见病例。

A Curious Case of Scimitar Syndrome That Defies Embryology.

作者信息

Hoenig Samuel M, Robinson Justin, Szugye Nicholas, Sharew Betemariam, Shah Shawn M, Costello John, Fuchs Margaret, Karamlou Tara

机构信息

Case Western Reserve University School of Medicine, Cleveland, Ohio.

Department of Pediatric Cardiac Surgery, Cleveland Clinic, Cleveland, Ohio.

出版信息

Ann Thorac Surg Short Rep. 2024 Jul 26;2(4):832-835. doi: 10.1016/j.atssr.2024.07.005. eCollection 2024 Dec.

Abstract

Scimitar syndrome is a rare condition described by unique anatomic features that consist mainly of an abnormal connection of the right pulmonary veins to the inferior vena cava and right atrial junction, as well as an anomalous systemic arterial supply to the right lung. We present the case of a 60-year-old man with an atypical variant of scimitar syndrome that was embryologically perplexing and anatomically challenging to correct. We highlight key surgical and procedural considerations for a patient with scimitar syndrome presenting with this complex surgical anatomy.

摘要

弯刀综合征是一种罕见疾病,其具有独特的解剖学特征,主要包括右肺静脉与下腔静脉及右心房交界处的异常连接,以及右肺的异常体循环动脉供血。我们报告了一例60岁男性患有弯刀综合征非典型变异型的病例,该病例在胚胎学上令人困惑,在解剖学上矫正具有挑战性。我们强调了对于具有这种复杂手术解剖结构的弯刀综合征患者的关键手术和操作注意事项。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/868d/11708552/289063dbbfd2/gr1.jpg

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