Risk assessment for pulmonary arterial hypertension (PAH) utilizing noninvasive prognostic variables could be more practical in real-world scenarios, especially at follow-up reevaluations. Patients who underwent comprehensive evaluations both at baseline and at follow-up visits were enrolled. The primary endpoint was all-cause mortality. Predictive variables identified by Cox analyses were further incorporated with the French noninvasive risk prediction approach. A total of 580 PAH patients were enrolled. During a median follow-up time of 47.0 months, 112 patients (19.3%) died. By multivariate Cox analyses, tricuspid annular plane systolic excursion (TAPSE), TAPSE/pulmonary arterial systolic pressure (PASP), and cardiopulmonary exercise testing-derived peak oxygen consumption (VO 2 ) remained independent predictors for survival. Regarding the French noninvasive risk prediction method, substituting N-terminal pro-b-type natriuretic peptide (NT-proBNP) with the newly derived low-risk criteria of a TAPSE ≥ 17 mm or a TAPSE/PASP > 0.17 mm/mmHg, or alternating 6-min walking distance with a peak VO 2 ≥ 44 %predicted retained the discrimination power. When recombining the low-risk criteria, the combination of World Health Organization functional class (WHO FC), TAPSE and peak VO 2 at baseline, and the combination of WHO FC, NT-proBNP, and peak VO 2 at follow-up showed better discriminative ability than the other combinations. In