Diagnosis of cardiac sarcoidosis: histological evidence vs. imaging

Abstract

ABSTRACT Introduction The prognosis for cardiac sarcoidosis (CS) remains unfavorable. Although early and accurate diagnosis is crucial, the low detection rate of endomyocardial biopsy makes accurate diagnosis challenging. Areas covered The Heart Rhythm Society (HRS) consensus statement and the Japanese Circulation Society (JCS) guidelines are two major diagnostic criteria for the diagnosis of CS. While the requirement of positive histology for the diagnosis in the HRS criteria can result in overlooked cases, the JCS guidelines advocate for a group of ‘clinical’ diagnoses based on advanced imaging, including cardiovascular magnetic resonance and 18F-fluorodeoxyglucose positron emission tomography, which do not require histological evidence. Recent studies have supported the usefulness of clinical diagnosis of CS. However, other evidence suggests that clinical CS may sometimes be inaccurate. This article describes the advantages and disadvantages of the current diagnostic criteria for CS, and typical imaging and clinical courses. Expert opinion The diagnosis of clinical CS has been made possible by recent developments in multimodality imaging. However, it is still crucial to look for histological signs of sarcoidosis in other organs in addition to the endomyocardium. Additionally, phenotyping based on clinical manifestations such as heart failure, conduction abnormality or ventricular arrhythmia, and extracardiac abnormalities is clinically significant.