Diagnosis of isolated cardiac sarcoidosis based on new guidelines

Abstract

Aims In the updated guidelines for cardiac sarcoidosis (CS) proposed by the Japanese Circulation Society (JCS), the definition of isolated CS (iCS) was established for the first time. This prompted us to examine the characteristics of patients with CS including iCS according to them by reviewing patients undergoing 18 F-fluoro-2-deoxyglucose positron-emission tomography/computerized tomography (FDG-PET/CT), compared with those with CS determined by the conventional international criteria. Methods and results From 2013 to 2019, 94 patients (61 ± 15 years, 50 female patients) with suspected CS underwent whole-body and cardiac FDG-PET/CT scanning. In contrast to 22 patients with CS based on the international criteria, 34 [27 with systemic sarcoidosis including cardiac involvement (sCS) and 7 with definitive iCS] were diagnosed with CS according to the new JCS guidelines (P = 0.012), and 60 were not (4 suspected iCS, 13 systematic sarcoidosis without cardiac involvement, and 43 no sarcoidosis). In addition to 26 of 34 patients with CS, corticosteroids were also started in 6 of 60 without CS according to clinical need. Conclusions Diagnostic yield with the new JCS guidelines was higher, with approximately 1.5-fold of the patients diagnosed with CS compared with the previous international criteria and definitive iCS accounting for approximately 20% of the whole CS cohort. In addition to 75% of the patients with sCS or definitive iCS in the updated guidelines, 10% in whom CS was not documented were also started on corticosteroids for clinical indications such as reduced cardiac function or arrhythmia.

POINT: Should Isolated Cardiac Sarcoidosis Be Considered a Significant Manifestation of Sarcoidosis? Yes

“…In the most recent study, seven of 34 CS patients fulfilling the current Japanese diagnostic criteria 2 had definitive isolated CS (21%), and four had suspected isolated CS (12%), by whole-body PET scan and a comprehensive clinical assessment. 9 In certain clinical scenarios, cardiologists should be particularly alert to the possibility of isolated CS. One is the phenotype of severe chronic heart failure akin to nonischemic dilated cardiomyopathy.…”

Isolated Cardiac Sarcoidosis with High-Grade Heart Block: Utilization of New Diagnostic Guidelines

“…These new guidelines proposed the diagnosis of CS via major and minor criteria to classify cardiac involvement that includes analysis of ECG, ECHO, CMR, and FDG PET findings ( Table 1 ) [ 5 ]. These guidelines have been shown to capture more patients with CS and ICS, allowing them to proceed to recommended therapies such as steroid treatment and cardiac devices for primary prevention [ 6 ].…”

The Importance of Multimodality Imaging in the Diagnosis and Management of Patients with Infiltrative Cardiomyopathies: An Update

“…No pathognomonic findings for cardiac sarcoidosis can be revealed by cardiac magnetic resonance imaging, but the extent of late gadolinium enhancement, mainly located in the basal segments of the interventricular septum and lateral wall, typically in the epicardium and mid myocardium, emerged as an important prognostic factor [ 125 , 126 , 127 ].…”