Increased vascular endothelial growth factor in patients with cyanotic congenital heart diseases may not be normalized after a Fontan type operation

To determine the change of serum concentration of vascular endothelial growth factor (VEGF) in patients with cyanotic congenital heart disease (C-CHD).Patients comprised four groups: group A, 19 patients without cyanosis; group B, 24 patients with C-CHD; group C, 17 patients who had C-CHD and underwent biventricular repair; and group D, 15 patients who had single ventricle and underwent a Fontan type operation. Blood samples were obtained from upper arm veins and serum VEGF was determined. We determined correlation between serum VEGF and arterial oxygen saturation and compared levels of serum VEGF among groups. In addition, age and hemodynamic variables derived from cardiac catheterization were analyzed in terms of correlation with serum VEGF.Serum VEGF significantly negatively correlated with arterial oxygen saturation (r = -0.62, p0.0001). Serum VEGF in B and D were significantly higher than those in A and C (median = 443 pg/mL in B and 302 pg/mL in D vs 180 pg/mL in A and 216 pg/mL in C, respectively). Serum VEGF was significantly negatively correlated with patient's age (p = 0.54, p0.04) in D, but significantly positively correlated with systemic venous pressure (p = 0.45, p0.01) in pooled data.Patients with C-CHD have increased serum VEGF in parallel with the degree of cyanosis. With biventricular repair, cyanosis and serum VEGF are normalized. However, with a Fontan type operation, cyanosis disappears but serum VEGF may not be normalized because of elevated venous pressure in association with younger age.