• Yuan Li, Hongling Qiu, Qinhua Zhao, Jing He, Rong Jiang, Wenhui Wu, Cijun Luo, Huiting Li, Lan Wang, Jinming Liu, and Sugang Gong.
• Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, China.
• Int. J. Clin. Pract. 2022 Jan 1; 2022: 7021178.

BackgroundTo observe different roles of direct bilirubin (Dbil) on portopulmonary hypertension (POPH) and idiopathic pulmonary arterial hypertension (IPAH).MethodsThirty incident patients with POPH and 180 with IPAH (matched by the WHO functional classification in a 1 : 6 ratio) between March 2010 and December 2020 were included. The receiver operating curve and Kaplan-Meier method were applied to estimate the ability to distinguish between the two and survival, respectively. Univariate and forward multiple stepwise regression analyses were performed to access the relationship between pulmonary vascular resistance (PVR) and clinical indices.ResultsCompared to IPAH, the POPH group had better hemodynamics including PVR (7.08 ± 3.95 vs. 14.89 ± 7.11, P < 0.001) and higher total bilirubin (Tbil) and Dbil. Tbil and Dbil had a negative correlation with PVR in the POPH group (r = -0.394, P=0.031; r = -0.364, P=0.048, respectively) but positive correlation in the IPAH group (r = 0.218, P=0.003; r = 0.178, P=0.018, respectively). Increased neutrophil counts (r = 0.394, P=0.031) and elevated NT-proBNP (r = 0.433, P < 0.001) would help predict the elevation of PVR in POPH and IPAH groups independent of Dbil, respectively. Dbil could distinguish POPH from IPAH (AUC = 0.799, P=0.009), and the ability was elevated when taking aspartate aminotransferase together (AUC = 0.835, P < 0.001). The overall survival was better in POPH than in IPAH (7 dead cases of POPH and 96 of IPAH, P=0.002). Survival was better in POPH than in IPAH in the group of Dbil ≥7 μmol/L (P=0.001) but showed no significant difference between POPH and IPAH in the group of Dbil <7 μmol/L (P=0.192).ConclusionsThe POPH group had a better hemodynamic profile than IPAH. Dbil was associated oppositely with the elevation of PVR in POPH and IPAH. Patients with POPH had better survival than those with IPAH in the total cohort and in the group of Dbil ≥7 μmol/L, but limited dead cases of POPH should be noted.Copyright © 2022 Yuan Li et al.

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