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使用体外膜肺氧合(VA-ECMO)和手术切除治疗与原发性纵隔淋巴瘤(PGL)相关的复发性气管软化症(rTTS):一例报告

Management of rTTS associated with PGL using VA-ECMO and surgical resection: a case report.

作者信息

Zhou Kechun, Liu Guoliang, Chen Yi, Lin Li, Lan Pin

机构信息

Department of Emergency Medicine, The Fifth Affiliated Hospital of Wenzhou Medical University, Lishui Central Hospital, Lishui Hospital of Zhejiang University, Lishui, Zhejiang, China.

Department of Emergency Medicine, People's Hospital of Lishui Liandu District, Lishui, Zhejiang, China.

出版信息

Front Cardiovasc Med. 2024 Sep 30;11:1468415. doi: 10.3389/fcvm.2024.1468415. eCollection 2024.

Abstract

BACKGROUND

Paraganglioma (PGL) is a neuroendocrine tumor located outside the adrenal gland that can secrete catecholamines. Clinical manifestations include headaches, hypertension, and, rarely, cardiomyopathy. Among these, reverse Takotsubo cardiomyopathy (rTTS) is a rare Takotsubo cardiomyopathy (TTS) associated with a surge in catecholamines.

CASE INTRODUCTION

This article reports a case of a hypertensive patient admitted for recurrent dizziness and chest tightness. During treatment, the patient suddenly experienced chest tightness and shortness of breath, followed by refractory cardiogenic shock, and was eventually diagnosed with rTTS. The patient gradually recovered and was successfully discharged after receiving treatments, including tracheal intubation with mechanical ventilation, extracorporeal membrane oxygenation (ECMO), and surgery.

CONCLUSION

The diagnosis of rTTS is significantly aided by the presence of free plasma metanephrines and specific changes observed in cardiac ultrasound. In the treatment of severe rTTS, ECMO can serve as a crucial life support technology. Under VA-ECMO support, early resection of the PGL after accelerated preoperative preparation may be a feasible approach.

摘要

背景

副神经节瘤(PGL)是一种位于肾上腺外的神经内分泌肿瘤,可分泌儿茶酚胺。临床表现包括头痛、高血压,很少见的还有心肌病。其中,反向Takotsubo心肌病(rTTS)是一种与儿茶酚胺激增相关的罕见Takotsubo心肌病(TTS)。

病例介绍

本文报告一例因反复头晕和胸闷入院的高血压患者。治疗期间,患者突然出现胸闷、气短,随后出现难治性心源性休克,最终被诊断为rTTS。患者经气管插管机械通气、体外膜肺氧合(ECMO)及手术等治疗后逐渐康复并成功出院。

结论

游离血浆甲氧基肾上腺素的存在及心脏超声观察到的特定变化对rTTS的诊断有显著帮助。在严重rTTS的治疗中,ECMO可作为关键的生命支持技术。在VA-ECMO支持下加速术前准备后早期切除PGL可能是一种可行的方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2d31/11471588/caf07646c3e3/fcvm-11-1468415-g001.jpg

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