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副神经节瘤所致心源性休克和肠缺血中的机械循环支持:一个复杂病例的经验教训及叙述性综述

Mechanical Circulatory Support in Paraganglioma Induced Cardiogenic Shock and Intestinal Ischemia: Lessons from a Complex Case and Narrative Review.

作者信息

Giordano Alessio, Canu Letizia, Mastronardi Manuela, Petrone Luisa, Sparano Clotilde, Marzano Mauro, Bergamini Carlo, Prosperi Paolo

机构信息

Emergency Surgery Unit, Department of Emergency and Acceptance, Careggi University Hospital, 50134 Florence, Italy.

Department of Experimental and Clinical Biomedical Sciences "Mario Serio", University of Florence, 50134 Florence, Italy.

出版信息

J Clin Med. 2025 Aug 20;14(16):5882. doi: 10.3390/jcm14165882.

Abstract

: The catecholamine-induced hypertensive crisis is a rare, life-threatening condition caused by excessive catecholamine release, often resulting in cardiogenic shock and multiorgan failure. Management is challenging, especially when hemodynamic instability persists despite standard medical therapy. : We conducted a narrative review of published articles between 2013 and 2025. The search was conducted in MEDLINE (PubMed, Scholar and Embase). We also presented a case managed at our reference center. : Overall, 42 studies including 69 patients were included. ECMO was the most commonly used modality, often serving as a bridge to surgery. The overall hospital mortality rate was 17.4%. Timing of adrenalectomy varied, with no clear consensus on the optimal approach. We report also a case of a 43-year-old woman with neurofibromatosis type 1 who developed acute cardiogenic shock due to an adrenal paraganglioma. She was supported with ECMO and underwent emergency bowel resection for intestinal ischemia, followed by adrenalectomy. Despite aggressive treatment, the patient died from progressive multiorgan failure. : This case highlights the complexity of managing paraganglioma crisis, the potential role of ECMO as a bridge to surgery, and the importance of individualized, multidisciplinary care. Early recognition and referral to specialized centers are essential, though further studies are needed to guide optimal management strategies.

摘要

儿茶酚胺诱发的高血压危象是一种罕见的、危及生命的疾病,由儿茶酚胺过度释放引起,常导致心源性休克和多器官功能衰竭。治疗具有挑战性,尤其是在尽管采用了标准药物治疗但血流动力学仍不稳定的情况下。

我们对2013年至2025年发表的文章进行了叙述性综述。检索在MEDLINE(PubMed、Scholar和Embase)中进行。我们还介绍了在我们参考中心管理的一个病例。

总体而言,纳入了42项研究,共69例患者。体外膜肺氧合(ECMO)是最常用的方式,常作为手术的桥梁。总体医院死亡率为17.4%。肾上腺切除术的时机各不相同,对于最佳方法没有明确的共识。我们还报告了一例43岁1型神经纤维瘤病女性患者,因肾上腺副神经节瘤发生急性心源性休克。她接受了ECMO支持,并因肠缺血接受了急诊肠切除术,随后进行了肾上腺切除术。尽管进行了积极治疗,患者仍死于进行性多器官功能衰竭。

该病例凸显了管理副神经节瘤危象的复杂性、ECMO作为手术桥梁的潜在作用以及个体化多学科护理的重要性。早期识别并转诊至专科中心至关重要,不过还需要进一步研究来指导最佳管理策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2692/12387319/fd073d2dc246/jcm-14-05882-g001.jpg

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