Abstract 13244: Prothrombotic Endothelial Dysfunction in Pre-Fontan Subjects is Similar to Pulmonary Arterial Hypertension Associated with the Eisenmenger Syndrome (PAH-Eisenmenger)
Background: Prothrombotic endothelial changes have been reported in patients with congenital heart disease presenting as Eisenmenger syndrome, but less extensively investigated in subjects undergoing total cavopulmonary anastomosis (so-called Fontan-type operation, non-pulsatile pulmonary blood flow). Purpose: We comparatively analyzed the circulating levels of endothelial activation/prothrombotic markers in PAH-Eisenmenger patients with Glenn operation undergoing Fontan completion. Patients/
Methods: Thirty-five patients were enrolled, 25 classified as PAH-Eisenmenger (age 4 to 52 years, median 31 years) and 10 as Fontan candidates (age 4 to 19 years, median 10.7 years). Plasma von Willebrand factor antigen (VWF:Ag), tissue-type plasminogen activator (t-PA) and thrombomodulin were measured by enzyme-linked immunoassays.
Results: In both patient groups, the circulating levels of VWF:Ag and t-PA were increased very similarly (Table). Levels of these markers were positively related in pre-Fontan patients (r=0.66, p=0.03). Thrombomodulin, a naturally occurring endothelial anticoagulant proteoglycan was significantly depressed in both patient groups, particularly in the pre-Fontan conditions (Table).
Conclusions: The data suggest that endothelial dysfunction with a shift toward a protrombotic and procoagulant state is also present in Fontan candidates, with a potential role in the so-called failure of the Fontan-type circulation. As in PAH in general, development of specific therapeutic strategies seems warranted.
- © 2012 by American Heart Association, Inc.