Novel hepatic function-based prognostic prediction model for pulmonary arterial hypertension

BackgroundHepatic dysfunction is increasingly recognized as a contributor to adverse outcomes in patients with pulmonary arterial hypertension (PAH). However, current prognostic models for PAH do not incorporate hepatic function parameters.ObjectivesTo develop and validate a novel risk prediction model that integrates hepatic function indicators with established PAH severity markers, and to compare its performance against the COMPERA 2.0 model.DesignProspective cohort study.MethodsThree liver fibrosis scores (LFSs)-FIB-4, NFS, and HUI-were evaluated for their prognostic significance. A total of 715 PAH patients from Fuwai Hospital were used to develop the FW-HP model. The model's predictive performance was assessed and compared with COMPERA 2.0. Risk stratification of the PAH cohort was subsequently conducted based on model scores.ResultsAll three LFSs were independently associated with mortality in PAH. The FW-HP model, incorporating albumin, total bilirubin, N-terminal pro-B-type natriuretic peptide, and World Health Organization functional class, provided superior risk prediction with area under the receiver operating characteristic curve of 0.86, 0.89, and 0.83 at 1-, 2-, and 3-year intervals, respectively-markedly exceeding COMPERA 2.0. The FW-HP score demonstrated higher net clinical benefit and enabled earlier identification of non-low-risk patients requiring more aggressive management.ConclusionsThe FW-HP model offers improved prognostic accuracy by incorporating hepatic function into PAH risk stratification. It represents a practical tool for early risk identification with potential to guide clinical decision-making in PAH patients.