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体外膜肺氧合支持下计算机断层扫描血管造影诊断马方综合征合并暴发性心肌炎:一例报告

Computed tomography angiography diagnosis of Marfan syndrome complicated with fulminant myocarditis under extracorporeal membrane oxygenation support: a case report.

作者信息

Zhou Jing, Yang Yuqiong, Mei Jing, Zhao Shibing, Xu Jiali

机构信息

Department of Radiology, The First Affiliated Hospital of Bengbu Medical University, No. 287 Changhuai Road, Bengbu, Anhui Province 233004, China.

School of Graduate, Bengbu Medical University, No. 2600 Donghai Avenue, Longzihu District, Bengbu, Anhui Province 233030, China.

出版信息

Eur Heart J Case Rep. 2025 Aug 21;9(9):ytaf411. doi: 10.1093/ehjcr/ytaf411. eCollection 2025 Sep.

DOI:10.1093/ehjcr/ytaf411
PMID:40918213
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12412438/
Abstract

BACKGROUND

Fulminant myocarditis (FM) is a rare but serious inflammatory disease of the heart that should be considered for extracorporeal membrane oxygenation (ECMO) supportive therapy when it occurs. The diagnosis of FM is made more difficult in the context of Marfan's syndrome combined with aortic root dilation. We report a case of a patient on ECMO support and with comorbid Marfan's syndrome who was finally diagnosed with FM after computed tomography angiography (CTA) differentiated between FM, coronary artery disease, and aortic root dilation.

CASE SUMMARY

An 18-year-old male with suspected Marfan's syndrome presented to our hospital with sudden onset of anterior chest pain without obvious trigger with nausea and vomiting for 10 h, supported by ECMO. His laboratory tests showed leucocytosis, elevated troponin, and creatine kinase. The electrocardiogram showed acute high lateral and extensive anterior wall myocardial infarction. On the second day of admission, he underwent CTA to rule out extensive aortic coarctation and coronary stenosis and found inhomogeneous enhancement of the myocardium and abnormal patchy enhancement of the epicardium, which was considered to be FM. The patient was then treated with methylprednisolone and human immunoglobulin, and his symptoms and laboratory markers improved markedly after a few days.

DISCUSSION

The diagnostic process in this case highlights the challenge of recognizing FM in the context of complex cardiovascular disease. Extracorporeal membrane oxygenation-supported CTA of the aorta combined with computed tomography (CT) cardiac coronary imaging provided an important basis for diagnosis, helped rule out other potential aetiologies, suggested the possibility of myocarditis, and contributed to the patient's optimal therapy.

摘要

背景

暴发性心肌炎(FM)是一种罕见但严重的心脏炎症性疾病,发病时应考虑采用体外膜肺氧合(ECMO)支持治疗。在马凡综合征合并主动脉根部扩张的情况下,FM的诊断更加困难。我们报告一例接受ECMO支持且合并马凡综合征的患者,经计算机断层扫描血管造影(CTA)鉴别FM、冠状动脉疾病和主动脉根部扩张后,最终确诊为FM。

病例摘要

一名疑似马凡综合征的18岁男性因无前明显诱因突然出现胸痛并伴有恶心呕吐10小时入院,接受ECMO支持。他的实验室检查显示白细胞增多、肌钙蛋白升高和肌酸激酶升高。心电图显示急性高侧壁和广泛前壁心肌梗死。入院第二天,他接受CTA检查以排除广泛主动脉缩窄和冠状动脉狭窄,结果发现心肌不均匀强化以及心外膜片状异常强化,考虑为FM。随后该患者接受甲泼尼龙和人免疫球蛋白治疗,几天后症状和实验室指标明显改善。

讨论

该病例的诊断过程凸显了在复杂心血管疾病背景下识别FM的挑战。主动脉的ECMO支持下CTA联合心脏冠状动脉CT成像为诊断提供了重要依据,有助于排除其他潜在病因,提示心肌炎的可能性,并有助于患者的最佳治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a4c5/12412438/e4acde3f63df/ytaf411f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a4c5/12412438/2aaa74e8822e/ytaf411f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a4c5/12412438/e4acde3f63df/ytaf411f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a4c5/12412438/2aaa74e8822e/ytaf411f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a4c5/12412438/e4acde3f63df/ytaf411f2.jpg

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经早期活检证实的暴发性心肌炎及其结局。
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What Is Marfan Syndrome?什么是马凡氏综合征?
JAMA. 2023 May 9;329(18):1618. doi: 10.1001/jama.2023.3826.
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Case Report: Effects of Sars-CoV-2 on Marfan syndrome with resulting acute aortic dissection.病例报告:Sars-CoV-2 对马凡综合征的影响导致急性主动脉夹层。
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Acute Myocarditis Mimicking Hypertrophic Cardiomyopathy in Marfan Syndrome and Morphologically Abnormal Mitral Valve.马凡综合征中酷似肥厚型心肌病且形态异常二尖瓣的急性心肌炎
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