Abstract 11038: Late Gadolinium Enhancement Cardiac Magnetic Resonance in Detection of Cardiac Involvement and its Prognostic Implication in Patients with Extracardiac Sarcoidosis

Abstract

PURPOSE: In patients with sarcoidosis, sudden cardiac death is a leading cause of mortality, and unrecognized cardiac involvement (CI) is often noted. Recent advances in late gadolinium enhancement cardiovascular magnetic resonance (LGE-CMR) studies enable detection of minute amounts of myocardial damage. We sought to investigate the usefulness of LGE-CMR for the early identification of CI and its prognostic implication in patients with extracardiac sarcoidosis in Japan.

METHODS: Sixty-one consecutive patients with biopsy and/or clinically-proven extracardiac sarcoidosis without clinical signs of cardiac sarcoidosis were prospectively recruited for LGE-CMR study. Patients were followed for a median of 31 months (IQR 26-38 months) for major adverse outcomes (primary; death from cardiac cause, secondary; death from any cause, symptomatic arrythmia or heart failure required admission).

RESULTS: Patients were predominantly middle-aged (57 ± 15 years), female (66%) and had relatively prolonged course of sarcoidosis (median, 38 months) mostly in stable condition with free from immunosuppressants (89%). LGE-CMR identified CI in 8 patients (13%). Multivariate analysis showed thinning of interventricular septum on echocardiogram was an independent determinant for CI (odds ratio 11.7, 95% confidence interval 1.49-92.0; p=0.019). During the follow-up period, no patients experienced cardiac death, one patient with CI required pacemaker for advanced atrio-ventricular block, and one with CI had the resolution of LGE-CMR with steroid therapy and no adverse events. Four patients with CI denied steroid therapy, but had no LGE progression by follow-up CMR. Two patients without CI had non-cardiac death and there were no significant differences in event free survival between the patients with and without CI (87.5% vs 96.2%; p=0.42).

CONCLUSIONS: In Japanese patients with extracardiac sarcoidosis, LGE-CMR, associated with echocardiographic thinning of interventricular septum, was useful for early detection of CI. However, the crude event rate in our relatively latent cases of sarcoidosis were lower compared with previous studies, indicating ethnical consideration for their outcome prediction.