Cardiac sarcoidosis

Research Reports in Clinical Cardiology Dovepress open access to scientific and medical research Review Open Access Full Text Article Research Reports in Clinical Cardiology downloaded from https://www.dovepress.com/ by 54.37.163.172 on 01-Dec-2018 For personal use only. Cardiac sarcoidosis This article was published in the following Dove Press journal: Research Reports in Clinical Cardiology 15 April 2016 Number of times this article has been viewed Benedict T Costello 1,2 James Nadel 3 Andrew J Taylor 1,2 Department of Cardiovascular Medicine, The Alfred Hospital, 2Baker IDI Heart and Diabetes Research Institute, Melbourne, VIC, 3School of Medicine, University of Notre Dame, Sydney, NSW, Australia 1 Clinical vignette Correspondence: Andrew J Taylor Heart Centre, Third Floor Phillip Block, Commercial Road, Melbourne 3004,VIC, Australia Tel +61 3 9076 2363 Email A 34-year-old man presented with fever following a period of overseas travel and underwent computed tomography of the chest. In this study, there were signs suspicious for pulmonary sarcoidosis with small bilateral noncalcified mediastinal and hilar lymph nodes, yet he was lost to follow-up. The following year, the same patient presented with acute shortness of breath following an aeroplane flight. Clinical examination revealed tachycardia (120 bpm), hypotension (100/70 mmHg), and signs of congestive cardiac failure. An electrocardiogram showed evidence of conducting system disease with a first-degree heart block and left anterior hemiblock (Figure 1). The patient was referred for echocardiography (echo), which showed severe left ventricular (LV) systolic dysfunction, and an echodensity in the LV apex was suspicious for thrombus. An angiotensin-converting enzyme level was within normal limits, and serum calcium was slightly elevated at 2.53 mmol/L (normal range: 2.14–2.50 mmol/L). Urinary calcium levels were also within normal limits. He was referred for cardiac magnetic resonance (CMR) imaging, which confirmed severely reduced LV function and also demonstrated widespread late contrast enhancement in a noncoronary distribution (Figure 2). On the basis of these findings, the clinical diagnosis of cardiac sarcoidosis (CS) was highly likely. A positron emission tomo graphy (PET) scan was also supportive of this diagnosis (Figure 3), with heterogeneous 18 F-fluorodeoxyglucose (18F-FDG) uptake corresponding to areas with late gadolinium enhancement (LGE) on CMR and reduced perfusion in the inflamed segments. The patient was subsequently referred for cardiac biopsy. Despite an attempt to attain cardiac tissue from the areas of the heart corresponding to the contrast enhancement on the CMR images, there was no evidence of sarcoidosis on the cardiac biopsy specimens. However, an endobronchial biopsy was then performed, which demonstrated the 27 submit your manuscript | www.dovepress.com Research Reports in Clinical Cardiology 2016:7 27–33 Dovepress © 2016 Costello et al. This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms. php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php). http://dx.doi.org/10.2147/RRCC.S72080 Powered by TCPDF (www.tcpdf.org) Abstract: Cardiac sarcoidosis is a rare but life-threatening condition, requiring a high degree of clinical suspicion and low threshold for investigation to make the diagnosis. The cardiac manifestations include heart failure, conducting system disease, and arrhythmias predisposing to sudden cardiac death. A number of investigations are available to assist in making the diagnosis. The diagnosis may be made from the clinical history and evidence of inflammation on imaging modalities in the active phase and evidence of myocardial scarring in the chronic phase. Keywords: cardiac magnetic resonance, positron emission tomography, sarcoidosis, sudden cardiac death Research Reports in Clinical Cardiology downloaded from https://www.dovepress.com/ by 54.37.163.172 on 01-Dec-2018 For personal use only. Costello et al Figure 1 Electrocardiogram in a patient with CS demonstrating first-degree heart block and left anterior hemiblock. Abbreviation: CS, cardiac sarcoidosis. typical noncaseating granulomas consistent with sarcoidosis (Figure 4). The combination of this finding and the abnormalities on cardiac imaging allowed a histologic diagnosis of CS. Based on the diagnosis and with a reduced ejection fraction, he was referred for implantation of an automatic implantable cardiovertor defibrillator (AICD). There has been no therapy from the AICD in the 6 months since it was implanted. At last clinical review, he was stable, breathless on moderate levels of exertion, and has had no significant change in ejection fraction despite initial aggressive steroid therapy. Sarcoidosis is a multisystem granulomatous disorder of unclear etiology with a significant prevalence of cardiac involvement. CS can be a life-threatening condition, requiring a high level of clinical suspicion and a low threshold for investigation to make the diagnosis. The patient provided written informed consent for the use of the information and images in this review. The Alfred Hospital ethical review Figure 3 PET images of a patient with CS. Notes: (A) PET image of left ventricle in short axis: resting perfusion imaging (top row) show defects in the anterolateral wall corresponding to areas of inflammation on 18 F-FDG images (bottom row). (B) Image taken from whole-body PET demonstrating heterogeneous 18F-FDG uptake consistent with CS. Abbreviations: PET, positron emission tomography; 18F-FDG, 18F-fluorodeoxyglucose; CS, cardiac sarcoidosis. board deemed ethics approval unnecessary for this study under their Institutional guidelines, as patient images were anonymized and could not be traced back to patients. Incidence and pathophysiology Systemic sarcoidosis Systemic sarcoidosis is a disease characterized by noncaseating granulomas in involved organs (Figure 1). Sarcoidosis Figure 2 CMR images in a patient with CS, demonstrating the widespread contrast enhancement (LGE) typical of granuloma, extending throughout the myocardium and not constrained to a coronary artery distribution. Notes: (A) Apical three-chamber view. (B) Apical four-chamber view. (C) Basal short axis view of the LV. Abbreviations: CMR, cardiac magnetic resonance; CS, cardiac sarcoidosis; LGE, late gadolinium enhancement; LV, left ventricle. 28 Powered by TCPDF (www.tcpdf.org) Dovepress submit your manuscript | www. (...truncated)