Abstract 11618: Low Antithrombin Level Correlates With Failing Fontan Circulation and Predicts the Mortality
Background: Antithrombin is one of natural anticoagulants produced by the liver and the activity (AT) decreases as a result of liver dysfunction. Fontan pathophysiology includes heart failure-related hepatopathy that accompanies coagulation abnormality.
Purpose: To clarify clinical significance of AT in Fontan patients.
Methods and Results: We prospectively measured AT in consecutive 303 Fontan patients (17±9 years) and compared with the clinical variables, including hemodynamics, peak oxygen uptake (PVO2), liver function, plasma brain natriuretic peptide (BNP), and unscheduled hospitalization (USH), including all-cause mortality. The AT was 109±14 (%, normal range: 80-120%) and it correlated inversely with age, central venous pressure (CVP), and plasma levels of BNP and creatinine, and positively with arterial oxygen saturation (Sat) and PVO2 (p < 0.001 for all). Patient with heterotaxy syndrome (HS) showed a low AT (p < 0.001). The lower plasma albumin level and platelet count and high total bilirubin level were associated with the lower AT (p < 0.05-0.0001). Multivariate analysis revealed that HS, older age, high levels of CVP, total bilirubin and BNP, and low Sat independently associated with the low AT. During the follow-up of 26 months, 53 USH, including 14 deaths, occurred. Low AT predicted USH (HR: 0.72 per 10 %, 95%CI: 0.60-0.88, p = 0.0012), especially the mortality (HR: 0.40 per 10 %, 95%CI: 0.28-0.56, p < 0.0001).
Conclusions: Low AT was an ominous clinical manifestation that closely associated with failing Fontan circulation in adults, especially those with HS, and predicted the morbidity and morbidity.
Author Disclosures: N. Tsujii: None. H. Ohuchi: None. Y. Hayama: None. J. Negishi: None. K. Noritake: None. O. Sasaki: None. A. Miyazaki: None. O. Yamada: None.
- © 2014 by American Heart Association, Inc.