Cardiac sarcoidosis presenting with complex conduction abnormalities as the first manifestation of widespread systemic sarcoidosis: a case report

Maximilian L Müller; Wolfgang Poller; Carsten Skurk; Denis Poddubnyy; Britta Siegmund; Thomas Schneider; Ulf Landmesser; Bettina Heidecker

doi:10.1093/ehjcr/ytad017

Cardiac sarcoidosis presenting with complex conduction abnormalities as the first manifestation of widespread systemic sarcoidosis: a case report

Eur Heart J Case Rep. 2023 Jan 11;7(1):ytad017. doi: 10.1093/ehjcr/ytad017. eCollection 2023 Jan.

Authors

Maximilian L Müller¹, Wolfgang Poller¹, Carsten Skurk¹, Denis Poddubnyy², Britta Siegmund², Thomas Schneider², Ulf Landmesser¹, Bettina Heidecker¹

Affiliations

¹ Department of Cardiology, Campus Benjamin Franklin, Charité-Universitätsmedizin Berlin corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Hindenburgdamm 30, 12203 Berlin, Germany.
² Department of Gastroenterology, Infectious Diseases and Rheumatology, Campus Benjamin Franklin, Charité-Universitätsmedizin Berlin corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Hindenburgdamm 30, 12203 Berlin, Germany.

Abstract

Background: Sarcoidosis is a granulomatous multi-organ disease of unknown aetiology. Despite being relatively rare, cardiac sarcoidosis constitutes a very important manifestation of sarcoidosis, as its symptoms regularly precede or occur in isolation of more prevalent ones, and as it is the main driver of mortality in systemic sarcoidosis.

Case summary: We present the case of a 37-year-old woman, in which clinically isolated cardiac sarcoidosis revealed widespread systemic sarcoidosis. Apart from constitutional symptoms and strong recurrent dizziness (i.e. near-syncopes), which persisted for multiple years already, our patient initially presented with complex conduction abnormalities, including a right bundle branch block, left anterior hemi-block, and atrioventricular block °1. Following inconclusive endomyocardial biopsies, performed due to detection of focal septal scarring on cardiac magnetic resonance imaging, an ¹⁸F-FDG-PET-CT, performed upon admission to our clinic, showed distinct hypermetabolic lesions indicative of active inflammation in various organs and raised suspicion of systemic sarcoidosis. Eventually, histopathological evidence of non-caseating granulomas in affected lymph nodes, extracted by bronchoscopy, confirmed the diagnosis of systemic sarcoidosis after reasonable exclusion of other granulomatous diseases. Immediate initiation of long-term immunosuppressive therapy led to almost complete remission, as monitored by consequential ¹⁸F-FDG-PET-CT scans.

Discussion: Unexplained complex conduction abnormalities in young patients may be a sign of sarcoidosis, even in isolation of more prevalent symptoms. Correct interpretation and prompt initiation of a structured interdisciplinary diagnostic workup, including ¹⁸F-FDG-PET-CT as the imaging modality of choice, are essential to initiate specific treatment and obviate the major risk of mortality resulting from cardiac sarcoidosis.

Keywords: Cardiac magnetic resonance imaging; Cardiac sarcoidosis; Case report; Heart arrhythmia; Positron emission tomography; Sarcoidosis.

Publication types

Case Reports