Diagnosis and Prognosis of Isolated Cardiac Sarcoidosis: Multidisciplinary Diagnosis Versus Japanese Circulation...

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. We aimed to study the prognosis of ICS according to 2 different diagnostic strategies. We prospectively included patients with a high suspicion for cardiac sarcoidosis 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. The median follow-up time was 2.4 years (twenty-fifth to seventy-fifth percentile: 1.2 to 3.7). The prevalence of ICS was 7% (14 of 198) per the JCS criteria and 29% (57 of 198) per our MDD criteria. Compared with patients with non-ICS, patients with ICS per either criterion had similar rates of major cardiovascular outcomes (progression of atrioventricular block, worsening ejection fraction ≥10%, appropriate implantable cardioverter-defibrillator therapies, heart transplant, and death). The Kaplan-Meier analyses showed similar prognosis for ICS and non-ICS in all outcomes, except for an association between ICS by MDD criteria and a shorter time to cardiac hospitalization. The 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 patients with ICS and patients with non-ICS, regardless of which criteria were used.