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Background: The severity of pulmonary arterial hypertension (PAH) is classified based on mean pulmonary artery pressure (mPAP) levels. However, other markers have not been elucidated. Fibrinolytic markers, such as total plasminogen activator inhibitor-1 (tPAI-1) and thrombomodulin (TM), are known to reflect arterial endothelial function. However, the relationship between serum tPAI-1, TM and pulmonary circulation has not been completely determined. Methods: This study included 100 consecutive patients (38 men), with a mean age of 68.9±12.0 years, with cardiac diseases who underwent right heart catheterization. Serum coagulation and fibrinolytic marker levels were measured.Results: The average mPAP value was 25.1±13.1 mmHg for all patients. The mPAP levels revealed a significant positive correlation with serum tPAI-1 (ρ=0.24, p=0.042) and uric acid (ρ=0.29, p=0.0031) levels. In the group with mPAP levels less than 25 mmHg (n=58, ave. 17.3±4.3 mmHg), mPAP levels showed a significant positive correlation with serum tPA-1 (ρ=0.34, p=0.034) and TM (ρ=0.34, p=0.043) values. The mean tPAI-1 (29.8±23.3 ng/ml, p=0.047) and uric acid (5.7±1.8 mg/dl, p=0.026) levels were significantly less in those with lower mPAP levels. A multivariate analysis revealed that tPAI-1 alone was a significant independent characteristic marker of PAH (odds ratio 1.02, 95%CI 1.000-1.036, p=0.034).Conclusions: These results indicate that serum tPAI-1 and TM may be useful predictors of severity, similar to mPAP in patients with PAH. They could be beneficial in predicting PAH among patients in the early stage of the disease.