High mass (>18 g) of late gadolinium enhancement on CMR imaging is associated with major cardiac events on long-term outcome in patients with biopsy-proven extracardiac sarcoidosis

Background: Cardiac involvement is the most important cause of mortality in patients with systemic sarcoidosis. Late gadolinium enhancement (LGE) on cardiovascular magnetic resonance imaging (CMR) has been shown to be a predictor of major cardiovascular adverse events (MACE) in the setting of systemic sarcoidosis. We sought to evaluate the relationship between LGE mass and adverse long-term outcome in patients with biopsy-proven extracardiac sarcoidosis. Methods: Between 2001 and 2013, 197 consecutive patients with suspected cardiac sarcoidosis were identified in our institution database. Of them, 56 patients have had biopsy-proven extracardiac sarcoidosis and represented our studied population. Patients were divided into two groups based on LGE mass by a median value (mild LGE < 18 g, high LGE > 18 g) for comparison of MACE. Results: Twenty-eight patients had a high mass of LGE. Of them, 15 (54%) experienced MACE (OR - 31.15, 95% CI 3.7-262). Except for 1 patient, no patient with mild LGE presented with anyMACE during follow-up (median of 32 months). Patients with high LGE had lower CMR-derived left (53.6 +/- 14.9 vs. 62.2 +/- 6.7, p < 0.01) and right (49.1 +/- 11.5 vs. 56.4 +/- 9.2, p < 0.05) ventricular ejection fractions. LGE mass of 18 g discriminated patients with and without MACE (93% sensitivity, 88% specificity, AUC = 0.972). LGE mass was the only independent predictor of MACE on multivariate Cox analysis adjusted (OR = 1.7, 95% CI 1.06 to 2.72, p = 0.03). Conclusion: In biopsy-proven extracardiac sarcoidosis patients, a high mass of LGE >18 g was associated with MACE. (C) 2016 Elsevier Ireland Ltd. All rights reserved.