Diagnosis and Prognosis of Isolated Cardiac Sarcoidosis: Multidisciplinary Diagnosis vs Japanese Circulation Society Criteria.

Abstract

The prognosis of isolated cardiac sarcoidosis (ICS) has not been fully elucidated. The only diagnostic criteria available for ICS, the Japanese Circulation Society (JCS) criteria, have not been validated. Our objective was to study the prognosis of ICS, according to 2 different diagnostic strategies. We prospectively included patients with high suspicion for cardiac sarcoidosis (CS) by a multidisciplinary sarcoidosis team from November 2016 to June 2021. We included only incident cases. We applied the JCS ICS criteria and our multidisciplinary diagnosis (MDD) ICS criteria. We included 198 incident patients who had a final diagnosis of cardiac sarcoidosis. Median follow up time was 2.4 years (p25-p75 1.2-3.7). The prevalence of ICS was 7% (14/198) per the JCS criteria, and 29% (57/198) per our MDD criteria. Compared to non-isolated CS, patients with ICS per either criteria had similar rates of major cardiovascular outcomes (progression of atrioventricular block, worsening EF ≥ 10%, appropriate implantable cardioverter defibrillator [ICD] therapies, heart transplant, and death). Kaplan-Meier analyses showed similar prognosis for ICS and non-isolated CS in all outcomes, except for an association between ICS by MDD criteria and a shorter time to cardiac hospitalization. Multivariable Cox regression analyses showed that ICS was not an independent predictor of death, heart transplant, or clinical worsening. In conclusion, our MDD ICS criteria were more inclusive than the JCS ICS criteria. The prognosis was similar between ICS and non-isolated CS patients, regardless of which criteria were used.