Background: The 6-minute walk test (6MWT) and cardiopulmonary exercise test (CPET) are exercise tests associated with physical function, quality of life and hemodynamic data in patients with pulmonary arterial hypertension (PAH). This study was conducted to assess correlations between exercise capacity, quality of life and disease functional classification, and to analyze the value of comprehensive assessments in predicting mortality in patientswith PAH. Methods: Fifty-four patients with PAH were enrolled. Comprehensive assessments including exercise capacity evaluated using the 6MWT and CPET, and health-related quality of life evaluated using the Short Form 36 (SF-36) questionnaire were performed in all participants. The patients were followed for 2 years with the end point of mortality. Results: The survivors had a longer 6-minute walking distance, higher peak oxygen uptake and higher physical component score of the SF-36 than the non-survivors. In addition, exercise capacity combined with SF-36 predicted 2-year mortality in the patients with PAH. The patients with lower peak oxygen uptake (peak VO2 < 11.03 mL/kg/ min) and lower physical component score (score < 44.54) had a higher mortality rate than those with a higher peak VO2 and higher physical component score (adjusted hazard ratio = 19.95, p = 0.011). Conclusions: Comprehensive assessments of exercise capacity and quality of life can be used to predict 2-year mortality in patients with PAH.