Introduction: Left ventricular systolic dysfunction (LVD) occurs in progressive cardiac sarcoidosis (CS) and is associated with increased cardiovascular morbidity and mortality. Due to the unpredictable course of CS, the degree of LVD required to substantially increase the risk of death or transplant remains incompletely understood. We sought to establish this threshold and hypothesized that a lesser degree of LVD in CS patients compared to other heart failure (HF) cohorts would be associated with increased risk of death or transplant.
Methods: We identified consecutive patients with CS diagnosed per HRS guidelines or suspected isolated CS. LVEF was examined as a continuous variable and also in a priori defined groups of LVEF ≥ 40% and LVEF < 40%. Cox regression models compared endpoints of (1) death and (2) death or transplant between LVEF groups, adjusting for demographics, comorbidities, and medication use. Receiver operating curves were generated for each endpoint, and to determine the point of maximal discrimination by LVEF.
Results: In 110 patients with CS, LVEF < 40% (n=39) was associated with decreased NYHA class and increased use of HF medications compared to LVEF ≥ 40% (n=71, p < 0.05). LVEF < 40% did not predict mortality alone in either unadjusted (HR 1.90, p = 0.52) or adjusted analyses (HR 4.85, p = 0.39). However, LVEF < 40% was strongly associated with death or transplant prior to adjustment (HR 6.43, p = 0.007), with a persistent trend toward increased risk after adjustment (HR 8.31, p = 0.051). Receiver operating curves provided a c-statistic of 0.67 for predicting death or cardiac transplant with a maximal discriminatory point of LVEF = 40.4%.
Conclusion: Similar to other HF cohorts, decreased LV systolic function in CS is associated with an increased risk of transplant or death. This risk appears to manifest at a higher LVEF threshold (40.4%) than expected in other HF cohorts, highlighting the need for additional risk stratification beyond LVEF in CS patients.