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先天性矫正型大动脉转位：一种疾病，多种表现——病例系列

Congenitally corrected transposition of the great arteries: one disease, diverse manifestations-a case series.

作者信息

Benesch Vidal Maria Luisa, Park Han Ki, Toda Kouichi, Kandavello Geetha, Youngmin Eun Lucy, Zentner Dominica, Komori Motoki, Sinning Christoph

机构信息

Department of Cardiology, University Heart and Vascular Centre Hamburg, Martinistr. 52, 20251 Hamburg, Germany.

Division of Cardiovascular Surgery, Department of Thoracic and Cardiovascular Surgery, Severance Cardiovascular Hospital, Yonsei University College of Medicine, 50-1, Yonsei-Ro, Seodaemun-gu, Seoul 03722, Korea.

出版信息

Eur Heart J Case Rep. 2025 Apr 8;9(5):ytaf174. doi: 10.1093/ehjcr/ytaf174. eCollection 2025 May.

DOI:10.1093/ehjcr/ytaf174
PMID:40336938
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12056722/
Abstract

BACKGROUND

Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect with heterogenous clinical manifestations that can pose both diagnostic and management challenges throughout life.

CASE SUMMARY

We describe four patients with ccTGA and different presentations including sudden cardiac arrest, progressive heart failure, post-partum heart failure, and NSTEMI.

AIMS AND DISCUSSION

This case series aims to illustrate the importance of multimodality imaging to assist the diagnosis and support treatment strategies in patients with ccTGA. Direct long-term sequalae, such as arrythmias and heart failure, and the associated management challenges are highlighted. Additionally, the challenges of managing pregnancy with a sRV and the development of acquired heart disease demonstrate the clinical care challenges in caring for this population across the life span.

摘要

背景

先天性矫正型大动脉转位（ccTGA）是一种罕见的先天性心脏缺陷，临床表现多样，在患者一生中都会给诊断和治疗带来挑战。

病例总结

我们描述了4例ccTGA患者，其表现各不相同，包括心脏骤停、进行性心力衰竭、产后心力衰竭和非ST段抬高型心肌梗死。

目的与讨论

本病例系列旨在说明多模态成像在辅助ccTGA患者诊断及支持治疗策略方面的重要性。强调了诸如心律失常和心力衰竭等直接的长期后遗症以及相关的治疗挑战。此外，单心室妊娠管理的挑战和获得性心脏病的发展表明了在该人群全生命周期护理中面临的临床挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/f3be7e326555/ytaf174f8.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/146dda0af3ae/ytaf174il2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/9f0acb5fc9c1/ytaf174f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/a5e95add3d47/ytaf174f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/e8ca348ae1e6/ytaf174f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/23c15622b231/ytaf174f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/a2a3f5872ffa/ytaf174f5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/d188406ec07f/ytaf174f6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/6265092dbd9e/ytaf174f7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/f3be7e326555/ytaf174f8.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/146dda0af3ae/ytaf174il2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/9f0acb5fc9c1/ytaf174f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/a5e95add3d47/ytaf174f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/e8ca348ae1e6/ytaf174f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/23c15622b231/ytaf174f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/a2a3f5872ffa/ytaf174f5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/d188406ec07f/ytaf174f6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/6265092dbd9e/ytaf174f7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f0f/12056722/f3be7e326555/ytaf174f8.jpg

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JACC Adv. 2022 May 14;1(2):100027. doi: 10.1016/j.jacadv.2022.100027. eCollection 2022 Jun.
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End-stage heart failure in congenitally corrected transposition of the great arteries: a multicentre study.先天性矫正型大动脉转位的终末期心力衰竭：一项多中心研究。
Eur Heart J. 2023 Sep 7;44(34):3278-3291. doi: 10.1093/eurheartj/ehad511.
3
Overview of Long-Term Outcome in Adults with Systemic Right Ventricle and Transposition of the Great Arteries: A Review.
系统性右心室和大动脉转位成人的长期预后概述：一项综述
Diagnostics (Basel). 2023 Jun 28;13(13):2205. doi: 10.3390/diagnostics13132205.
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Treatment of advanced heart failure in adults with congenital heart disease: a narrative review and clinical cases.先天性心脏病成人晚期心力衰竭的治疗：一篇叙述性综述及临床病例
Cardiovasc Diagn Ther. 2022 Oct;12(5):727-743. doi: 10.21037/cdt-22-230.
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2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death.2022年欧洲心脏病学会室性心律失常患者管理和心脏性猝死预防指南
Eur Heart J. 2022 Oct 21;43(40):3997-4126. doi: 10.1093/eurheartj/ehac262.
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