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Brain & Heart





                                        REVIEW ARTICLE
                                        Characteristics and prognosis of patients

                                        with cardiac sarcoidosis presenting with
                                        atrioventricular block and ventricular

                                        arrhythmias



                                        Caleb Carver *     and Raheel Ahmed 2
                                                   1
                                        1 Department of Medicine, Gold Coast University Hospital, Southport, Queensland, Australia
                                        2 Department of Cardiology, Royal Brompton and Harefield Hospitals, London, United Kingdom



                                        Abstract

                                        Cardiac sarcoidosis (CS) may initially present with life-threatening arrhythmias such
                                        as atrioventricular block (AVB) and ventricular arrhythmia (VA), including ventricular
                                        tachycardia and ventricular fibrillation. Diagnosing CS is challenging due to subtle
                                        or absent findings in routine tests and the low sensitivity of the definitive diagnostic
                                        tool, endomyocardial biopsy. CS can also mimic other cardiac conditions such as
                                        dilated cardiomyopathy or arrhythmogenic right ventricular cardiomyopathy, and it
                                        may occur without any systemic involvement. Consequently, delays in presentation,
                                        accurate  diagnosis,  and  initiation  of  appropriate  treatment  are  frequent.  Greater
                                        awareness of the condition could improve outcomes for patients with CS. Non-
                                        invasive advanced imaging techniques, such as 18F-fluorodeoxyglucose positron
                                        emission tomography and cardiac magnetic resonance imaging are essential for
            *Corresponding author:
            Caleb Carver                diagnosis, although they may not be accessible in all clinical settings. Management of
            (calebcarver@yahoo.co.uk)   arrhythmias in CS involves conventional therapies in the utilization of antiarrhythmics
                                        and non-pharmacological therapy (e.g., implantable devices), but with notable
            Citation: Carver C, Ahmed R.
            Characteristics and prognosis of   divergences in the use of immunosuppression and a preference for implantable
            patients with cardiac sarcoidosis   cardioverter defibrillators over permanent pacemakers. This narrative review aimed
            presenting with atrioventricular   to outline the clinical features of patients with CS presenting with AVB or VA and
            block and ventricular arrhythmias.
            Brain & Heart. 2024;2(4):3515.   examine diagnostic investigations, imaging techniques, treatment approaches, and
            doi: 10.36922/bh.3515       prognosis based on current research and international guidelines.
            Received: April 27, 2024
            Accepted: August 19, 2024   Keywords: Cardiac sarcoidosis; Sarcoidosis; Atrioventricular block; Ventricular tachycardia;
                                        Ventricular fibrillation; Sudden cardiac death
            Published Online: October 8, 2024
            Copyright: © 2024 Author(s).
            This is an Open-Access article
            distributed under the terms of the
            Creative Commons Attribution   1. Introduction
            License, permitting distribution,
            and reproduction in any medium,   Sarcoidosis is a multisystem disease characterized by granuloma formation, inflammation,
            provided the original work is   and subsequent scarring. Cardiac involvement is clinically evident in approximately 5%
            properly cited.             of cases, although imaging and postmortem studies have revealed that this may be an
                                                     1,2
            Publisher’s Note: AccScience   underestimation.  Cardiac sarcoidosis (CS) has a worse prognosis than other organ
            Publishing remains neutral with   involvement, contributing to death in up to 85% of Japanese patients with sarcoidosis.
                                                                                                             2
            regard to jurisdictional claims in
            published maps and institutional   The manifestations of CS vary and include atrioventricular block (AVB), ventricular
            affiliations.               arrhythmias (VAs), left ventricular systolic dysfunction, and even sudden cardiac death,
            Volume 2 Issue 4 (2024)                         1                                doi: 10.36922/bh.3515
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