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一例疑似致心律失常性双心室心肌病的心脏结节病的磁共振成像与病理学相关性病例报告

An magnetic resonance imaging-pathology correlation case report of cardiac sarcoidosis mimicking arrhythmogenic biventricular cardiomyopathy.

作者信息

Kim B Michelle, Bois Melanie C, Del-Carpio Munoz Freddy, Rosenbaum Andrew N, Chang Ian C

机构信息

Mayo Clinic Alix School of Medicine, 200 First St SW, Rochester, MN 55905, USA.

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.

出版信息

Eur Heart J Case Rep. 2024 Aug 5;8(8):ytae395. doi: 10.1093/ehjcr/ytae395. eCollection 2024 Aug.

DOI:10.1093/ehjcr/ytae395
PMID:39156954
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11328533/
Abstract

BACKGROUND

Cardiac sarcoidosis (CS) is a granulomatous disease that can manifest as conduction defects, ventricular arrhythmias, and heart failure. The diagnosis of CS is inherently difficult due to variable presentations; as such, endomyocardial biopsy is often required but lacks sensitivity due to patchy myocardial involvement. Moreover, the diagnostic criteria of CS and arrhythmogenic cardiomyopathy overlap, particularly in right-side dominant or biventricular presentations, which further complicates an already challenging differential diagnosis.

CASE SUMMARY

A 53-year-old man with no prior chronic medical conditions presented with ventricular tachycardia (VT) and heart failure with reduced ejection fraction. He was found to have biventricular cardiomyopathy and late gadolinium enhancement on cardiac magnetic resonance imaging, resulting in an initial diagnosis of arrhythmogenic cardiomyopathy. Implantable cardioverter-defibrillator was placed, but he was readmitted for recurrent VT 2 months later. Despite an aggressive VT therapy (combination of antiarrhythmic drugs, epicardial and endocardial ablation, and stellate ganglion block), he continued with refractory VT and developed cardiogenic shock. Extra-corporeal membrane oxygenation was initiated as a bridge to heart transplantation. Pathology of the explanted heart revealed the underlying disease to be CS.

DISCUSSION

Cardiac sarcoidosis can mimic arrhythmogenic biventricular cardiomyopathy and may be difficult to distinguish by the proposed diagnostic criteria. High clinical suspicion and thorough investigation are necessary for an earlier diagnosis and initiation of treatment.

摘要

背景

心脏结节病(CS)是一种肉芽肿性疾病,可表现为传导缺陷、室性心律失常和心力衰竭。由于临床表现多样,CS的诊断本质上具有难度;因此,通常需要进行心内膜活检,但由于心肌受累呈斑片状,该检查缺乏敏感性。此外,CS和致心律失常性心肌病的诊断标准存在重叠,尤其是在右心室优势型或双心室表现中,这使得本就具有挑战性的鉴别诊断更加复杂。

病例摘要

一名53岁男性,既往无慢性病史,因室性心动过速(VT)和射血分数降低的心力衰竭就诊。他被发现患有双心室心肌病,心脏磁共振成像显示钆延迟强化,初步诊断为致心律失常性心肌病。植入了植入式心律转复除颤器,但2个月后他因复发性VT再次入院。尽管采取了积极的VT治疗(抗心律失常药物、心外膜和心内膜消融以及星状神经节阻滞联合应用),他仍持续出现难治性VT并发展为心源性休克。开始进行体外膜肺氧合作为心脏移植的过渡。切除心脏的病理检查显示潜在疾病为CS。

讨论

心脏结节病可模仿致心律失常性双心室心肌病,根据现有的诊断标准可能难以区分。高度的临床怀疑和全面的检查对于早期诊断和开始治疗是必要的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eeef/11328533/ac5079a63ae6/ytae395f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eeef/11328533/801d12fcd6b5/ytae395f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eeef/11328533/0bb645a92021/ytae395f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eeef/11328533/c6878f1031ad/ytae395f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eeef/11328533/ac5079a63ae6/ytae395f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eeef/11328533/801d12fcd6b5/ytae395f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eeef/11328533/0bb645a92021/ytae395f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eeef/11328533/c6878f1031ad/ytae395f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eeef/11328533/ac5079a63ae6/ytae395f4.jpg

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本文引用的文献

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Cardiac sarcoidosis: diagnosis and management.心肌结节病:诊断与管理。
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