Cargando…

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

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 dri...

Descripción completa

Detalles Bibliográficos
Autores principales: Müller, Maximilian L, Poller, Wolfgang, Skurk, Carsten, Poddubnyy, Denis, Siegmund, Britta, Schneider, Thomas, Landmesser, Ulf, Heidecker, Bettina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9887667/
https://www.ncbi.nlm.nih.gov/pubmed/36733684
http://dx.doi.org/10.1093/ehjcr/ytad017
_version_ 1784880385220411392
author Müller, Maximilian L
Poller, Wolfgang
Skurk, Carsten
Poddubnyy, Denis
Siegmund, Britta
Schneider, Thomas
Landmesser, Ulf
Heidecker, Bettina
author_facet Müller, Maximilian L
Poller, Wolfgang
Skurk, Carsten
Poddubnyy, Denis
Siegmund, Britta
Schneider, Thomas
Landmesser, Ulf
Heidecker, Bettina
author_sort Müller, Maximilian L
collection PubMed
description 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 (18)F-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 (18)F-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 (18)F-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.
format Online
Article
Text
id pubmed-9887667
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher Oxford University Press
record_format MEDLINE/PubMed
spelling pubmed-98876672023-02-01 Cardiac sarcoidosis presenting with complex conduction abnormalities as the first manifestation of widespread systemic sarcoidosis: a case report Müller, Maximilian L Poller, Wolfgang Skurk, Carsten Poddubnyy, Denis Siegmund, Britta Schneider, Thomas Landmesser, Ulf Heidecker, Bettina Eur Heart J Case Rep Grand Round 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 (18)F-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 (18)F-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 (18)F-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. Oxford University Press 2023-01-11 /pmc/articles/PMC9887667/ /pubmed/36733684 http://dx.doi.org/10.1093/ehjcr/ytad017 Text en © The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology. https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Grand Round
Müller, Maximilian L
Poller, Wolfgang
Skurk, Carsten
Poddubnyy, Denis
Siegmund, Britta
Schneider, Thomas
Landmesser, Ulf
Heidecker, Bettina
Cardiac sarcoidosis presenting with complex conduction abnormalities as the first manifestation of widespread systemic sarcoidosis: a case report
title Cardiac sarcoidosis presenting with complex conduction abnormalities as the first manifestation of widespread systemic sarcoidosis: a case report
title_full Cardiac sarcoidosis presenting with complex conduction abnormalities as the first manifestation of widespread systemic sarcoidosis: a case report
title_fullStr Cardiac sarcoidosis presenting with complex conduction abnormalities as the first manifestation of widespread systemic sarcoidosis: a case report
title_full_unstemmed Cardiac sarcoidosis presenting with complex conduction abnormalities as the first manifestation of widespread systemic sarcoidosis: a case report
title_short Cardiac sarcoidosis presenting with complex conduction abnormalities as the first manifestation of widespread systemic sarcoidosis: a case report
title_sort cardiac sarcoidosis presenting with complex conduction abnormalities as the first manifestation of widespread systemic sarcoidosis: a case report
topic Grand Round
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9887667/
https://www.ncbi.nlm.nih.gov/pubmed/36733684
http://dx.doi.org/10.1093/ehjcr/ytad017
work_keys_str_mv AT mullermaximilianl cardiacsarcoidosispresentingwithcomplexconductionabnormalitiesasthefirstmanifestationofwidespreadsystemicsarcoidosisacasereport
AT pollerwolfgang cardiacsarcoidosispresentingwithcomplexconductionabnormalitiesasthefirstmanifestationofwidespreadsystemicsarcoidosisacasereport
AT skurkcarsten cardiacsarcoidosispresentingwithcomplexconductionabnormalitiesasthefirstmanifestationofwidespreadsystemicsarcoidosisacasereport
AT poddubnyydenis cardiacsarcoidosispresentingwithcomplexconductionabnormalitiesasthefirstmanifestationofwidespreadsystemicsarcoidosisacasereport
AT siegmundbritta cardiacsarcoidosispresentingwithcomplexconductionabnormalitiesasthefirstmanifestationofwidespreadsystemicsarcoidosisacasereport
AT schneiderthomas cardiacsarcoidosispresentingwithcomplexconductionabnormalitiesasthefirstmanifestationofwidespreadsystemicsarcoidosisacasereport
AT landmesserulf cardiacsarcoidosispresentingwithcomplexconductionabnormalitiesasthefirstmanifestationofwidespreadsystemicsarcoidosisacasereport
AT heideckerbettina cardiacsarcoidosispresentingwithcomplexconductionabnormalitiesasthefirstmanifestationofwidespreadsystemicsarcoidosisacasereport