Abstract

BACKGROUND: Risk assessment in cardiac sarcoidosis remains challenging. OBJECTIVES: This study explored the prognostic value of myocardial late gadolinium enhancement (LGE) in sarcoidosis patients. METHODS: The study cohort included 324 patients with biopsy-proven sarcoidosis. LGE extent, pattern, and location were analyzed. The primary endpoint was ventricular tachycardia (VT) or ventricular fibrillation (VF) or appropriate device therapy. Secondary endpoints were hospitalization for heart failure (HF) or heart transplantation (HTx) and all-cause mortality. RESULTS: Over a 4.6-year follow-up, 30 patients (9.3%) reached the primary endpoint. HF/HTx occurred in 15 patients (4.6%) and all-cause mortality in 41 (12.7%). LGE extent was independently predictive of the primary endpoint (per SD change: HR: 1.03 [95% CI: 1.00-1.06]; P = 0.047), but not of HF/HTx (P = 0.30) or all-cause mortality (P = 0.50). Further to LGE extent, LGE on the right ventricular (RV) septum (HR: 5.43 [95% CI: 2.61-11.30]; P < 0.001), RV free wall (HR: 4.30 [95% CI: 1.99-9.27]; P < 0.001), and multifocal LGE (HR: 4.62 [95% CI: 2.19-9.72]; P < 0.001) were strongly predictive of the arrhythmia endpoint. Based on these findings, we propose an algorithm that identifies 4 patient subgroups and stratifies well the arrhythmia risk in biopsy-proven sarcoidosis patients (cumulative event rates: 1%, 11%, 23%, and 44%, respectively; chi-square = 44.7; P = 1.084 × 10-9). Compared with the Heart Rhythm Society classification system, this approach significantly enhanced model performance (chi-square = 8.02; P = 0.046) and risk discrimination (ΔAUC = 0.082; P = 0.019), and reclassified 43% of the population (9% to higher and 34% to lower risk categories). CONCLUSIONS: The authors propose a new risk stratification approach based on LGE features for assessing the risk of life-threatening ventricular arrhythmias in patients with biopsy-proven sarcoidosis.