We tested the hypothesis that Chinese patients have a coagulation profile that is less prothrombotic than that of Caucasian counterparts after the
Fontan procedure by determining the type and prevalence of
anticoagulant and procoagulant deficiencies in Chinese patients and comparing the findings to those previously reported in Caucasian series. The liver function and
coagulation factors were assessed in 21 ethnic Chinese patients, aged 17.0 +/- 5.6 years, at 10.7 +/- 4.0 years after the
Fontan procedure. The results were compared to those of 21 age-matched Chinese controls with minor
congenital heart disease. The prevalence of
coagulation factor deficiencies in our patients was further compared to that reported in Caucasian patients. When compared with controls, patients had significantly lower
protein C (p = 0.014), factors II (p = 0.024), V (p < 0.001), VII (p < 0.001), IX (p = 0.036), and X (p < 0.001), and higher
bilirubin (p = 0.001) levels. The prevalence of
protein C deficiency was 9.5%, whereas those of
factor II, V, VII, IX, and X deficiencies were 0, 66.7, 9.5, 0, and 57.1%, respectively. When compared with Caucasian data, our data showed a significantly lower prevalence of
protein C, total
protein S, antithorombin III,
factor II, and
factor VII deficiencies. Furthermore, the previously reported increase in
factor VIII levels was not found. In contrast, the prevalence of
factor X deficiency was higher in our patients. This study provides the first evidence of ethnic differences in
coagulation factor abnormalities after the
Fontan procedure. The imbalance between procoagulant and
anticoagulant pathways in Chinese patients favors a
bleeding, rather than a thrombotic, tendency.