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年轻患者出现心源性休克和难治性室性心动过速:一例未被怀疑的致心律失常性心肌病导致紧急心脏移植的病例。

Young patient presenting with cardiogenic shock and refractory ventricular tachycardia: a case of unsuspected arrhythmogenic cardiomyopathy leading to urgent heart transplantation.

作者信息

Yu Benoit, Poulakos Nickolas, Beaulieu-Shearer Alexander, Turgeon Pierre Yves, Trahan Sylvain, Belzile David, Sénéchal Mario

机构信息

Faculty of Medicine, Université Laval Québec, QC, Canada.

Faculty of Medicine and Health Sciences, McGill University Montreal, QC, Canada.

出版信息

Am J Cardiovasc Dis. 2024 Apr 15;14(2):121-127. doi: 10.62347/TAEY9817. eCollection 2024.

DOI:10.62347/TAEY9817
PMID:38764547
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11101963/
Abstract

Arrhythmogenic right ventricular cardiomyopathy is an important differential diagnosis in young patients presenting with palpitations and/or dyspnea and must be appropriately investigated. A 23-year-old man presented with cardiogenic shock and monomorphic ventricular tachycardia. He reported palpitations and progressive dyspnea for more than two years, but those symptoms were attributed to anxiety without any further investigation by his family physician. Investigations after the catastrophic presentation in our center suggested terminal right-sided heart failure with severe hepatic insufficiency and acute kidney injury. The patient benefited from extracorporeal membrane oxygenation, followed by an urgent heart transplant 16 days later after the exclusion of liver cirrhosis. Histopathologic analysis of the explanted heart confirmed arrhythmogenic cardiomyopathy.

摘要

致心律失常性右室心肌病是年轻患者出现心悸和/或呼吸困难时的重要鉴别诊断疾病,必须进行适当的检查。一名23岁男性出现心源性休克和单形性室性心动过速。他自述心悸和进行性呼吸困难已两年多,但这些症状被其家庭医生归因于焦虑,未作进一步检查。在我们中心出现灾难性病情后进行的检查提示终末期右侧心力衰竭伴严重肝功能不全和急性肾损伤。患者受益于体外膜肺氧合,在排除肝硬化后16天接受了紧急心脏移植。移植心脏的组织病理学分析证实为致心律失常性心肌病。

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本文引用的文献

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Endomyocardial Biopsy: The Forgotten Piece in the Arrhythmogenic Cardiomyopathy Puzzle.心肌内膜活检:致心律失常性心肌病之谜中被遗忘的一块。
J Am Heart Assoc. 2021 Oct 5;10(19):e021370. doi: 10.1161/JAHA.121.021370. Epub 2021 Sep 25.
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Evolving Diagnostic Criteria for Arrhythmogenic Cardiomyopathy.心律失常性心肌病的诊断标准不断演变。
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Non-invasive diagnostic imaging tests largely underdiagnose cardiac cirrhosis in patients undergoing advanced therapy evaluation: How can we identify the high-risk patient?非侵入性诊断成像测试在很大程度上未能诊断出接受先进治疗评估的患者中的心源性肝硬化:我们如何识别高危患者?
Clin Transplant. 2021 Jun;35(6):e14277. doi: 10.1111/ctr.14277. Epub 2021 Apr 25.
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Diagnosis of arrhythmogenic cardiomyopathy: The Padua criteria.心律失常性心肌病的诊断:帕多瓦标准。
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Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: An Updated Review of Diagnosis and Management.致心律失常性右室心肌病/发育异常:诊断与管理的最新综述
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Clinical Diagnosis, Imaging, and Genetics of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: JACC State-of-the-Art Review.心律失常性右室心肌病/发育不良的临床诊断、影像学和遗传学:美国心脏病学会的现状评估。
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Diagnosing ARVC in Pediatric Patients Applying the Revised Task Force Criteria: Importance of Imaging, 12-Lead ECG, and Genetics.应用修订的工作组标准诊断小儿患者的致心律失常性右室心肌病:影像学、12导联心电图及遗传学的重要性
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9
Heart transplantation in arrhythmogenic right ventricular cardiomyopathy - Experience from the Nordic ARVC Registry.心律失常性右室心肌病心脏移植 - 北欧 ARVC 注册中心的经验。
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