Plasma level of big endothelin-1 predicts the prognosis in patients with hypertrophic cardiomyopathy

Background: Cardiac remodeling is one of major pathological process in hypertrophic cardiomyopathy (HCM). Endothelin-1 has been linked to cardiac remodeling. Big endothelin-1 is the precursor of endothelin-1. Methods: A total of 245 patients with HCM were enrolled from 1999 to 2011 and partitioned to low, middle and high level groups according to their plasma big endothelin-1 levels. Results: At baseline, significant associations were found between high level of big endothelin-1 and left atrium size, heart function and atrial fibrillation. Big endothelin-1 was positively correlated with N-terminal B-type natriuretic peptide (r= 0.291, p < 0.001) and late gadoliniumenhancement (LGE) on magnetic resonance imaging (r= 0.222, p= 0.016). During a follow-up of 3 (range, 2-5) years, big endothelin-1 level was positively associated with the risks of all-causemortality, cardiovascular death and progression to NYHA class 3 or 4 (p= 0.020, 0.044 and 0.032, respectively). The rate of above events in the highest tertile were 18.1%, 15.7%, 24.2%, respectively. After adjusting for multiple factors related to survival and cardiac function, the significance remained in the association of big endothelin-1with the risk of all-causemortality (hazard ratio (HR)= 4.94, 95% confidence interval (CI) 1.07-22.88; p= 0.041) and progression to NYHA class 3 or 4 (HR= 4.10,95% CI 1.32-12.75, p= 0.015). Conclusion: Our study showed that high level of plasma big endothelin-1 predicted prognosis for patients with HCM and it can be added to the marker panel in stratifying HCM patients for giving treatment priority to those at high risk. (C) 2017 Published by Elsevier Ireland Ltd.