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Cardiac sarcoidosis concealed by arrhythmogenic right ventricular dysplasia/cardiomyopathy

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Abstract

Background A 37-year-old male with a history of palpitations and ventricular tachycardia was diagnosed with arrhythmogenic right ventricular dysplasia/cardiomyopathy on the basis of clinical assessment, electrocardiography and echocardiography. Over the following 3 years the patient progressed to end-stage heart failure and eventually underwent heart transplantation. Histological analysis of the explanted heart revealed the presence of numerous noncaseating granulomas.

Investigations Electrocardiography, echocardiography, 24 h Holter monitoring, cardiac MRI, coronary angiography, endomyocardial biopsy, exercise testing, electrophysiological study, laboratory examinations and histological examination of the explanted heart.

Diagnosis Cardiac sarcoidosis.

Management Immunosupressive and corticosteroid therapy. Routine endomyocardial biopsy is planned.

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Figure 1: Freeze frames from a functional MRI study performed on the patient at presentation.
Figure 2: Typical electrocardiograms recorded from the patient 3 years after his initial presentation.
Figure 3: Echocardiograms recorded from the patient 4 years and 9 months after his initial presentation—8 months before he underwent cardiac transplantation.
Figure 4: Examination of the explanted heart revealed findings that were typical of cardiac sarcoidosis.

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Acknowledgements

P Petrakopoulou and M Greif contributed equally to the preparation of this article.

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Correspondence to Paraskevi Petrakopoulou.

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Greif, M., Petrakopoulou, P., Weiss, M. et al. Cardiac sarcoidosis concealed by arrhythmogenic right ventricular dysplasia/cardiomyopathy. Nat Rev Cardiol 5, 231–236 (2008). https://doi.org/10.1038/ncpcardio1146

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