Epidemiology, Pathogenesis, and Diagnosis of Cardiac Sarcoidosis

Abstract

Cardiac sarcoidosis (CS) is a widely underdiagnosed yet clinically significant form of granulomatous myocarditis associated with significant morbidity and mortality. Clinical presentation ranges from silent cardiac involvement detected on imaging to cardiomyopathy or sudden cardiac death. Diagnosis of CS remains challenging due to the lack of sensitivity and specificity of any single diagnostic method, underscoring the importance of elevated clinical suspicion and the use of multimodality imaging to guide diagnosis and treatment. In this review, we discuss the epidemiology, pathogenesis, clinical features, and diagnosis of this clinically evading and enigmatic disease.

Impact of comorbid heart failure among hospitalized patients with sarcoidosis: A United States population-based cohort study

“…On the other hand, traditional risk factors for incident HF such as hypertension, diabetes, obesity, and CKD are more prevalent among black individuals compared to other race/ethnic groups [19] , [27] . In contrast to previous studies [28] , [29] , our analysis has shown that female patients with sarcoidosis and concomitant HF were less likely to be hospitalized compared to their male counterparts. This is likely related to some evidence suggesting that male sarcoidosis patients tend to have more cardiac manifestations [30] , [31] .…”

Challenges of cardiac sarcoidosis

“…Histologic proof of the epithelioid granulomas is part of the definition of sarcoidosis. For the heart involvement, the biopsy of endomyocardial tissue has a high specificity, but sampling errors and variable histology reduce the sensitivity down to 20 to 30% (12). Also, endomyocardial biopsies are usually obtained from the right side of the interventricular septum, while granulomas are typically located in the left ventricle (13).…”

Management of the arrhythmic manifestations of cardiac sarcoidosis

“…The majority of patients with sarcoidosis (90%–95%) have pulmonary and lymph node involvement, but isolated CS can occur. In CS, granulomas typically develop in the myocardium, often with endocardial or epicardial extension ( 9 ). The extent and distribution of these granulomas dictate the clinical manifestations.…”

“…Active myocardial inflammation can result in ventricular arrhythmias, ventricular dysfunction and sudden cardiac death. Furthermore, in the post-inflammatory phase, patchy fibrosis in the ventricular myocardium can develop, further predisposing to ventricular tachyarrhythmias ( 9 ).…”

“…Cardiac sarcoidosis may result in conduction abnormalities, tachyarrhythmias, sudden cardiac death and heart failure, with the manifestations dependent on the location, extent and activity of the disease ( 5 , 8 ). Cardiac involvement is responsible for a significant burden of morbidity and mortality in sarcoidosis ( 9 ). To date, management recommendations are primarily based upon consensus and expert opinion, given the lack of randomised controlled trial data ( 3 ).…”