Cardiac sarcoidosis presenting with syncope and rapidly progressive atrioventricular block: a case report

Abstract

BackgroundCardiac sarcoidosis (CS) accounts for a substantial morbidity and mortality. Early recognition of CS is important to prevent such detrimental consequences. A definite diagnosis of cardiac sarcoidosis remains challenging. Even after the diagnosis of CS is established, the appropriate dose and duration of corticosteroids in the treatment of CS have not been well-defined.Case summaryIn this report, we discuss a case of a 50-year-old man who presented with recurrent syncope. Electrocardiogram revealed sinus rhythm with left bundle branch block. Telemetry captured high-grade atrioventricular block. Coronary angiogram showed no coronary artery disease. Left ventriculography revealed left ventricular ejection fraction (LVEF) of 35–40%. A dual-chamber pacemaker was implanted. Cardiac magnetic resonance revealed mid-myocardial scarring suggestive of sarcoidosis. Computed tomography of the chest showed lymphadenopathy. Transbronchial biopsy was unrevealing; however, mediastinoscopy and lymph node biopsy showed non-caseating granulomas diagnostic of sarcoidosis. He became pacemaker dependent as noted in outpatient pacemaker interrogations. A biventricular implantable cardioverter-defibrillator upgrade was performed for primary prevention of sudden cardiac death. He was started on prednisone taper over the course of 6 months. After 1-year, his LVEF improved to 55% and native atrioventricular (AV) conduction had recovered as noted in outpatient device interrogations.DiscussionThis case highlights the importance to include CS in the differential diagnosis of a young patient with conduction system disease and non-ischaemic cardiomyopathy for appropriate treatment. Patients with left ventricular systolic dysfunction and AV nodal disease could potentially benefit from a slow prednisone taper over the course of 6 months.

A case of cardiac sarcoidosis with concurrent myocardial ischemia

“…Notably, more common pathologies such as hypertensive heart disease, vascular disease, diabetes mellitus, and thyroid-related disease must be considered. 4 While the definitive diagnosis for CS is endomyocardial biopsy showing noncaseating granulomas, this is rarely done in practice, as the procedure carries significant risk 5 with a low sensitivity (w20%). 6 In lieu of this, imaging plays a crucial role in diagnosis.…”

“…7 The discussed CMR findings support the diagnosis of CS in this patient; however, LGE on CMR is a nonspecific finding also seen in cardiac amyloidosis, myocarditis, systemic sclerosis, and dilated cardiomyopathy. 4 To further clarify the diagnosis, the Heart Rhythm Society proposed that clinical diagnosis should include all 3 of the following: histologically confirmed extracardiac sarcoidosis, exclusion of other causes of cardiac dysfunction, and at least 1 imaging, ECG, or functional finding consistent with CS. 8 This definition leaves ambiguity in the definitive diagnosis of this patient.…”

“…Notably, more common pathologies such as hypertensive heart disease, vascular disease, diabetes mellitus, and thyroid-related disease must be considered. 4 …”

Dilated Cardiomyopathy Compatible With Sarcoidosis Presenting with Syncope Due to Torsades de Pointes: a Case Report

“…Basic pathogenesis involves resolving inflammation areas that tend to evolve to myocardial scarring tissue, providing the substrate for reentry circuits 2 . Upon this scenario, the inflammatory affection of myocardial tissue stablishes cardiac sarcoidosis (CS), that might present with electrical abnormalities, including ventricular arrhythmias and atrioventricular (AV) blocks 3 , but also syncope [4][5][6][7] , heart failure and even cardiac tamponade 8 .…”

“…The exact prevalence of isolated cardiac sarcoidosis is uncertain 9 . It is estimated that up to a quarter of patients with sarcoidosis might present CS 10 , but only 5% manifest cardiac involvement clinically 4 . In parallel, up to 20% of CS patients present a clinically silent disease 11 with asymptomatic cardiac involvement, not identified by diagnostic criteria.…”