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

Abstract 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 any MACE 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.