Diagnosis of cardiac sarcoidosis: histological evidence vs. imaging

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.

KEYWORDS:

• Cardiac sarcoidosis
• diagnosis
• imaging
• prognosis
• heart failure

Article highlights

• Early and accurate diagnosis of cardiac sarcoidosis is crucial.

• Most diagnostic factors for HRS and JCS criteria are similar, but there are some significant differences, each with advantages and disadvantages in terms of sensitivity and specificity.

• Phenotyping based on clinical manifestations such as heart failure, conduction abnormality or ventricular arrhythmia, and extracardiac abnormalities is clinically significant.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial relationships or otherwise to disclose.

Additional information

Funding

This paper was not funded.