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以危及生命的心律失常为表现的未确诊心肌结节病

Undiagnosed myocardial sarcoidosis presenting as life threatening cardiac dysrhythmia.

作者信息

Davis Stacy A, Moore Walter J, Oliver Alyce M

机构信息

Section of Rheumatology, Department of Medicine, Medical College of Georgia, Augusta, Georgia, USA.

出版信息

BMJ Case Rep. 2011 Apr 13;2011:bcr0620103092. doi: 10.1136/bcr.06.2010.3092.

Abstract

Sarcoidosis is a systemic disorder of unknown aetiology characterised by its pathological hallmark of non-caseating granuloma. Definitive diagnosis requires compatible clinical and imaging features as well as pathogenic identification of non-caseating granulomas in at least one organ. The disease has a wide variety of clinical and radiological manifestations but is associated with low mortality. However, cardiac involvement which is clinically only identified in 5% significantly worsens prognosis due to complications such as congestive heart failure, ventricular tachyarrhythmia, pulmonary hypertension or conduction disturbance leading to sudden death. Cardiac involvement is implicated in 77-85% of deaths directly related to sarcoidosis. Autopsy series in sarcoidosis patients show cardiac involvement with sarcoidosis in up to 79% cases. This case details the clinical course of a 56-year-old female who had experienced refractory cardiac dysrhythmias for many years in the context of also having biopsy proven pulmonary sarcoidosis. She had failed multiple antiarrhythmics as well as pacemaker and implantable cardioverter defibrillator placement. It was not until she presented as a potentially fatal ventricular tachycardia that the possibility of cardiac involvement from her sarcoidosis was entertained as the aetiology of her cardiac problems. Confirmation of myocardial sarcoidosis with PET CT imaging and subsequent treatment with prednisone resulted in her clinical improvement.

摘要

结节病是一种病因不明的全身性疾病,其病理特征为非干酪样肉芽肿。明确诊断需要符合临床表现和影像学特征,以及在至少一个器官中发现非干酪样肉芽肿的病理学证据。该疾病有多种临床和放射学表现,但死亡率较低。然而,临床上仅有5%的患者会出现心脏受累,由于充血性心力衰竭、室性快速心律失常、肺动脉高压或传导障碍等并发症,会显著恶化预后,甚至导致猝死。心脏受累在与结节病直接相关的死亡中占77-85%。结节病患者的尸检系列显示,高达79%的病例存在心脏结节病。本病例详细介绍了一名56岁女性的临床病程,她多年来一直患有难治性心律失常,同时经活检证实患有肺部结节病。她使用多种抗心律失常药物均无效,且已植入起搏器和植入式心律转复除颤器。直到她出现潜在致命的室性心动过速,心脏结节病累及心脏才被认为是其心脏问题的病因。PET CT成像证实心肌结节病,随后使用泼尼松治疗后,她的临床症状有所改善。

相似文献

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Ventricular Arrhythmias in Cardiac Sarcoidosis.心脏结节病中的室性心律失常。
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