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A 55-year-old Caucasian male without cardiac history presented with a broad-complex tachycardia of a right ventricular origin (Fig. 1a). After successful electrocardioversion, echocardiography and coronary angiogram showed no significant abnormalities. Electrophysiological examination suggested a right ventricular mid-septal origin of the arrythmia, closely to the HIS bundle. Cardiac MRI (Fig. 1b,c) showed extensive late enhancement at the right ventricular part of the interventricular septum (Fig. 1b) and a focal lesion in the epicardial inferolateral wall (Fig. 1c), both showing high uptake on FDG PET-CT (Fig. 1d). In the laboratory findings, soluble interleukin-2 receptor (sIL-2R), a marker for sarcoidosis, was elevated (5276 pg/ml, normal value <3000 pg/ml), making cardiac sarcoidosis highly likely. FDG PET-CT and examination of skin and eyes showed no signs of extracardiac sarcoidosis and pulmonary sarcoidosis was excluded by high-resolution CT and bronchoalveolar lavage, suggesting a case of isolated cardiac sarcoidosis. The prevalence of cardiac involvement in sarcoidosis varies from 5 % in symptomatic patients to up to 30 % in autopsies [1]. Isolated cardiac sarcoidosis is rare and only described in case series [2]. For diagnosis, multimodality imaging is recommended, including MRI and FDG PET [3]. FDG PET is the modality of choice to examine (extra)cardiac sarcoidosis [4]. Giant cell myocarditis can be considered in the differential diagnosis. However the scan results, elevated sIL-2R, young age and dysrhythmia are typical for cardiac sarcoidosis. A two-chamber implantable cardioverter defibrillator was implanted and prednisolone treatment was started. FDG PET-CT after 3 months showed complete normalisation. Although extremely rare, this case illustrates the possible occurrence of mono-organ localisation of sarcoidosis.
References
Smedema JP, Zondervan PE, Hagen P van, et al. Cardiac sarcoidosis: Case-studies and a brief review of the literature. Neth Heart J. 2002;10:318–25.
Pampaloni MH, Nazar B, Botvinick E. Isolated right ventricular cardiac sarcoidosis demonstrated by 18FDG positron emission tomography. J Nucl Cardiol. 2014;21:652–4.
Joyce E, Delgado V, Ninaber MK, Marsan NA. The invisible made visible: multi-modality imaging in the evaluation of cardiac sarcoidosis. Eur Heart J. 2013;34:1278.
Adams H, Keijsers RG, Korenromp IH, Grutters JC. FDG PET for gauging of sarcoid disease activity. Semin Respir Crit Care Med. 2014;35:352–61.
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Huitema, M.P., Swaans, M.J., Grutters, J.C. et al. A case highly suspicious of isolated cardiac sarcoidosis. Neth Heart J 24, 433–434 (2016). https://doi.org/10.1007/s12471-016-0837-3
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DOI: https://doi.org/10.1007/s12471-016-0837-3