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心脏结节病

Cardiac sarcoidosis.

作者信息

Kim Jessica S, Judson Marc A, Donnino Robert, Gold Michael, Cooper Leslie T, Prystowsky Eric N, Prystowsky Stephen

机构信息

New York University School of Medicine, New York, NY 10016, USA.

出版信息

Am Heart J. 2009 Jan;157(1):9-21. doi: 10.1016/j.ahj.2008.09.009. Epub 2008 Nov 12.

Abstract

Cardiac sarcoidosis (CS) is a rare but potentially fatal condition that may present with a wide range of clinical manifestations including congestive heart failure, conduction abnormalities, and most notably, sudden death. Recent advances in imaging technology allow easier detection of CS, but the diagnostic guidelines with inclusion of these techniques have yet to be written. It has become clear that minimally symptomatic or asymptomatic cardiac involvement is far more prevalent than previously thought. Because of the potential life-threatening complications and potential benefit of treatment, all patients diagnosed with sarcoidosis should be screened for cardiac involvement. Patients with CS and symptoms such as syncope need an aggressive workup for a potentially life-threatening etiology, and often require implantable cardioverter-defibrillator therapy. CS patients without arrhythmic symptoms are still at risk for sudden death and may warrant an implantable cardioverter-defibrillator for primary prevention reasons. Although corticosteroids are regarded as the first-line drug of choice, therapy for CS is not yet standardized, and it is unclear at this point whether asymptomatic patients require therapy. Randomized clinical trials are clearly warranted to answer these very important patient care questions, and are endorsed fully by the authors.

摘要

心脏结节病(CS)是一种罕见但可能致命的疾病,其临床表现多样,包括充血性心力衰竭、传导异常,最显著的是猝死。成像技术的最新进展使CS的检测更加容易,但包含这些技术的诊断指南尚未制定。目前已经明确,症状轻微或无症状的心脏受累远比之前认为的更为普遍。鉴于潜在的危及生命的并发症以及治疗可能带来的益处,所有被诊断为结节病的患者都应接受心脏受累情况的筛查。患有CS且有晕厥等症状的患者需要积极检查以寻找可能危及生命的病因,并且通常需要植入式心律转复除颤器治疗。没有心律失常症状的CS患者仍然有猝死风险,出于一级预防的原因,可能也需要植入式心律转复除颤器。尽管皮质类固醇被视为一线首选药物,但CS的治疗尚未标准化,目前尚不清楚无症状患者是否需要治疗。显然需要进行随机临床试验来回答这些非常重要的患者护理问题,作者完全支持这一点。

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