Catastrophic Events of Cardiac Sarcoidosis: A Case Report

Abstract

Cardiac sarcoidosis (CS) can be silent in most patients with extrapulmonary sarcoidosis. Atrioventricular (AV) block is the most common clinical presentation, but it can also present as fatal ventricular arrhythmias and sudden cardiac death. Endomyocardial biopsy is the gold standard; however, it is not sensitive since CS can involve the myocardium in a patchy distribution. Our case depicts a female who presented with syncope; however, her hospital course was complicated by multiple cardiac arrests. Her initial laboratory tests, including an autoimmune workup, were unremarkable. Cardiac magnetic resonance and fluorodeoxyglucose (FDG) positron emission tomography (PET) imaging revealed intramyocardial delayed enhancement of the basal anteroseptal (non-ischemic distribution) and patchy foci of increased uptake in the anteroseptal and inferior myocardial region, respectively. The patient was started on intravenous methylprednisolone, and her condition slowly improved. Post-discharge, the patient followed in the outpatient clinic with a repeat FDG-PET scan revealing resolution of myocardial FDG uptake. She also underwent bronchoscopy with lymph node biopsy showing granulomas and endobronchial biopsy confirming pulmonary sarcoidosis.

Real-Life Utilization of Criteria Guidelines for Diagnosis of Cardiac Sarcoidosis (CS)

“…Our results are in line with other case reports and case series of CS, in which most CS cases present with AV conduction abnormalities, with a noted prevalence of up to 62% in the literature [35], and with many cases occurring as initial presenting events in systemic sarcoidosis [18][19][20][21]36]. Furthermore, in accordance with the literature, we found fewer cases of tachyarrhythmias and heart failure and only one case of symptomatic pericardial involvement, which has rarely been reported in the literature [18][19][20][21].…”

“…As in our case series, most CS cases in the literature were treated with a GC taper, with MTX used as the chief GC-sparing agent [ 18 , 19 , 20 , 21 ]. Prednisone has been shown retrospectively to reduce conduction abnormalities, although it was not shown in a study by Banba et al to be effective in reducing ventricular tachyarrhythmias [ 36 ].…”

“…Along these lines, CMR and PET-FDG were both used as complementary imaging modalities in our case series, not only for CS diagnosis but also to determine the medical and device therapeutic management of patients with CS, with the added advantage of the utilization of both imaging modalities over a single modality especially in determining disease activity. As reported in the literature, most of our CS cases were treated with GC tapers, with MTX used as the chief GC-sparing agent [ 18 , 19 , 20 , 21 ]. The need for and duration of immunosuppressive therapy in our CS cases were determined by cardiac imaging.…”

“…Because diagnosis remains a challenge and no consensus exists on the detection, monitoring, and treatment of CS, the literature on CS mostly consists of single case reports or small case series [ 18 , 19 , 20 , 21 ]. The diagnosis and management of CS ultimately depend on expert consensus, with no recommendations validated by prospective data or clinical trials [ 22 , 23 ].…”

“…Indeed, three sets of expert consensus criteria are utilized currently for the diagnosis of CS. These include the World Association of Sarcoidosis and other Granulomatous Disorders (WASOG) organ assessment tool, published in 2014 and updated in 2020 by the American Thoracic Society Guidelines for Sarcoidosis Diagnosis [ 24 , 25 ]; the Japanese Ministry of Health & Welfare (JMHW) 2006 criteria revised by the Japanese Circulation Society (JCS) 2016 criteria [ 26 ]; and the 2014 Heart Rhythm Society (HRS) criteria [ 5 , 27 ], which are thought to have greater sensitivity, as they include both cardiac PET-FDG and CMR as acceptable imaging modalities, as well as responsiveness to glucocorticosteroid (GC) treatment [ 5 , 19 , 24 , 25 , 27 , 28 , 29 , 30 ].…”