Cardiac sarcoidosis presenting as sustained ventricular tachycardia

N. Cotrim, Beatriz Vargas Andrade, Sofia Carralas Antunes, Miguel Rodrigues, Sílvia Aguiar Rosa, Marisa Peres, V. Martins
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Abstract

Introduction: Sarcoidosis has many possible clinical presentations since it can affect any organ, most commonly the lungs. The hallmark of the disease consists of the formation of non-necrotising granulomas. Pathogenesis is thought to rely on the interplay of genetic, environmental and epigenetic factors. This case highlights the importance of a thorough clinical history and physical examination, and the correlation with imaging findings in the diagnostic work-up of the non-ischaemic cardiomyopathy. Case description: A 57-year-old woman was admitted due to the sudden onset of malaise, dizziness, and chest discomfort. Sustained monomorphic ventricular tachycardia was evidenced and the patient rapidly evolved with haemodynamic instability; she underwent successful electrical cardioversion. The electrocardiogram afterwards showed a high-risk electrocardiographic pattern. Invasive coronary angiography excluded obstructive epicardial coronary lesions. Physical examination revealed skin lesions on the lower limbs which raised suspicion for erythema nodosum and therefore a biopsy was performed. Transthoracic echocardiography and cardiac magnetic resonance imaging revealed features consistent with an inflammatory cardiomyopathy, and an implantable cardioverter-defibrillator was placed. The histologic examination of the cutaneous lesions showed a non-necrotising granulomatous inflammatory process. Radionuclide imaging was inconclusive. The patient underwent an endomyocardial biopsy, which confirmed the diagnosis of systemic sarcoidosis with cardiac involvement. Conclusions: Systemic sarcoidosis with cardiac involvement is a challenging diagnosis. The role of imaging techniques such as transthoracic echocardiography, cardiac magnetic resonance imaging and radionuclide imaging is essential in raising suspicion and diagnosing this pathology. Endomyocardial biopsy is the ‘gold standard’ for its diagnosis; however, it has a low diagnostic yield.
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表现为持续性室性心动过速的心脏肉瘤病
导言肉样瘤病的临床表现多种多样,因为它可以影响任何器官,最常见的是肺部。该病的特征是形成非坏死性肉芽肿。发病机制被认为取决于遗传、环境和表观遗传因素的相互作用。本病例强调了全面临床病史和体格检查的重要性,以及在诊断非缺血性心肌病时与影像学检查结果的相关性。病例描述一名 57 岁的女性因突然出现乏力、头晕和胸部不适而入院。患者出现持续性单形性室性心动过速,并迅速发展为血流动力学不稳定。随后的心电图显示出高危心电图模式。侵入性冠状动脉造影排除了阻塞性心外膜冠状动脉病变。体格检查发现她下肢皮肤有病变,怀疑是结节性红斑,因此进行了活组织检查。经胸超声心动图和心脏磁共振成像显示其特征与炎症性心肌病一致,并放置了植入式心律转复除颤器。皮肤病变的组织学检查显示为非坏死性肉芽肿炎症过程。放射性核素成像没有得出结论。患者接受了心内膜活检,确诊为累及心脏的全身性肉样瘤病。结论:心脏受累的系统性肉样瘤病是一项具有挑战性的诊断。经胸超声心动图、心脏磁共振成像和放射性核素成像等成像技术在提高怀疑和诊断这种病变方面起着至关重要的作用。心内膜活检是诊断的 "金标准",但诊断率较低。
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来源期刊
CiteScore
2.10
自引率
0.00%
发文量
166
审稿时长
8 weeks
期刊介绍: The European Journal of Case Reports in Internal Medicine is an official journal of the European Federation of Internal Medicine (EFIM), representing 35 national societies from 33 European countries. The Journal''s mission is to promote the best medical practice and innovation in the field of acute and general medicine. It also provides a forum for internal medicine doctors where they can share new approaches with the aim of improving diagnostic and clinical skills in this field. EJCRIM welcomes high-quality case reports describing unusual or complex cases that an internist may encounter in everyday practice. The cases should either demonstrate the appropriateness of a diagnostic/therapeutic approach, describe a new procedure or maneuver, or show unusual manifestations of a disease or unexpected reactions. The Journal only accepts and publishes those case reports whose learning points provide new insight and/or contribute to advancing medical knowledge both in terms of diagnostics and therapeutic approaches. Case reports of medical errors, therefore, are also welcome as long as they provide innovative measures on how to prevent them in the current practice (Instructive Errors). The Journal may also consider brief and reasoned reports on issues relevant to the practice of Internal Medicine, as well as Abstracts submitted to the scientific meetings of acknowledged medical societies.
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