Diagnosis and treatment of cardiac sarcoidosis

Abstract

Sarcoidosis is a systemic granulomatous disease of unknown aetiology. The frequency of cardiac involvement (cardiac sarcoidosis (CS)) varies in the different geographical regions, but it has been reported that it is an absolutely important prognostic factor in this disease. Complete atrioventricular block is the most common, and ventricular tachycardia/ventricular fibrillation the second most common arrhythmia in this disease, both of which are associated with cardiac sudden death. Diagnosing CS is sometimes difficult because of the non-specific ECG and echocardiographic findings, and CS is sometimes misdiagnosed as dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy or an idiopathic ventricular aneurysm, and therefore, endomyocardial biopsy is important, but has a low sensitivity. Another problem is the recognition of isolated types of CS. Recently, MRI and (18)F-fluorodeoxyglucose positron emission tomography have been demonstrated to be useful tools for the non-invasive diagnosis of CS as well as therapeutic evaluation tools, but are still unsatisfactory. Treatment of CS is usually done by corticosteroid therapy to control inflammation, prevent fibrosis and protect from any deterioration of the cardiac function, but the long-term outcome is still in debate. Despite the advancement of non-pharmacological approaches for CS (pacing, defibrillators and catheter ablation) to improve the prognosis, there are still many issues remaining to resolve diagnosing and managing CS. Here, we attempt a review of the clinical evidence, with special focus on the current understanding of this disease and showing the current strategies and remaining problems of diagnosing and managing CS.

Cardiac sarcoidosis diagnosed by multimodality imaging

“…Our patient fulfilled one major criterion (ventricular wall thinning at the basal septum by echocardiogram and CMR) and four minor criteria (1) ECG showed frequent PVCs; (2) echocardiography showed localized akinesis; (3) PET showed abnormal myocardial perfusion; (4) CMR showed late gadolinium enhancement) . Active sarcoid lesions manifest increased 18F‐FDG uptake on PET imaging due to high utilization of glucose by inflammatory cells …”

“…The sensitivity of endomyocardial biopsy is <20% because sarcoid is usually locally distributed . Multimodality imaging can be used to guide the diagnose of cardiac sarcoidosis in conjunction with clinical signs . Our patient fulfilled one major criterion (ventricular wall thinning at the basal septum by echocardiogram and CMR) and four minor criteria (1) ECG showed frequent PVCs; (2) echocardiography showed localized akinesis; (3) PET showed abnormal myocardial perfusion; (4) CMR showed late gadolinium enhancement) .…”

Japanese Antibacterial Drug Management for Cardiac Sarcoidosis (J‐ACNES): A multicenter, open‐label, randomized, controlled study

“…Sarcoidosis is a systemic inflammatory and noncaseating granulomatous disease, involving various organs such as the lungs, eyes, heart, skin, lymph node, and nerves. 1 Although the etiology of sarcoidosis remains unknown, a hypersensitivity of immune reaction has been suspected as the main cause of sarcoidosis. 2 Cardiac sarcoidosis (CS) has been reported more frequently in Japan compared with other countries.…”

Cardiac sarcoidosis – management and prognosis (RCD code: III-3A.3)

“…The main goal is to reduce the area of active inflammation and prevent transformation of sarcoid granulomas to fibrosis. These reduce the risk of sudden cardiac death and protect from deterioration of the cardiac function [2,13]. Medications used to treat CS are presented in Table 1.…”