Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, China.
Int J Clin Pract. 2022 Feb 3;2022:7021178. doi: 10.1155/2022/7021178. eCollection 2022.
To observe different roles of direct bilirubin (Dbil) on portopulmonary hypertension (POPH) and idiopathic pulmonary arterial hypertension (IPAH).
Thirty 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.
Compared to IPAH, the POPH group had better hemodynamics including PVR (7.08 ± 3.95 vs. 14.89 ± 7.11, < 0.001) and higher total bilirubin (Tbil) and Dbil. Tbil and Dbil had a negative correlation with PVR in the POPH group ( = -0.394, =0.031; = -0.364, =0.048, respectively) but positive correlation in the IPAH group ( = 0.218, =0.003; = 0.178, =0.018, respectively). Increased neutrophil counts ( = 0.394, =0.031) and elevated NT-proBNP ( = 0.433, < 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, =0.009), and the ability was elevated when taking aspartate aminotransferase together (AUC = 0.835, < 0.001). The overall survival was better in POPH than in IPAH (7 dead cases of POPH and 96 of IPAH, =0.002). Survival was better in POPH than in IPAH in the group of Dbil ≥7 mol/L (=0.001) but showed no significant difference between POPH and IPAH in the group of Dbil <7 mol/L (=0.192).
The 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 mol/L, but limited dead cases of POPH should be noted.
观察直接胆红素（Dbil）在门脉性肺动脉高压（POPH）和特发性肺动脉高压（IPAH）中的不同作用。
纳入 2010 年 3 月至 2020 年 12 月期间的 30 例新发 POPH 患者和 180 例 IPAH 患者（按 WHO 功能分类以 1:6 的比例匹配）。应用受试者工作特征曲线和 Kaplan-Meier 法分别估计区分两者和生存的能力。采用单因素和向前逐步多元回归分析评估肺血管阻力（PVR）与临床指标的关系。
与 IPAH 相比，POPH 组具有更好的血流动力学特征，包括更低的 PVR（7.08 ± 3.95 比 14.89 ± 7.11，<0.001）和更高的总胆红素（Tbil）和 Dbil。Tbil 和 Dbil 与 POPH 组的 PVR 呈负相关（ = -0.394，=0.031； = -0.364，=0.048，分别），但与 IPAH 组的 PVR 呈正相关（ = 0.218，=0.003； = 0.178，=0.018，分别）。增加中性粒细胞计数（ = 0.394，=0.031）和升高 NT-proBNP（ = 0.433，<0.001）有助于预测 POPH 和 IPAH 组中 PVR 的升高，且与 Dbil 无关。Dbil 可区分 POPH 和 IPAH（AUC=0.799，=0.009），当与天门冬氨酸氨基转移酶联合使用时，其区分能力升高（AUC=0.835，<0.001）。POPH 的总体生存率优于 IPAH（POPH 死亡 7 例，IPAH 死亡 96 例，=0.002）。在 Dbil≥7μmol/L 的组中，POPH 的生存率优于 IPAH（=0.001），但在 Dbil<7μmol/L 的组中，POPH 和 IPAH 之间的生存率无显著差异（=0.192）。
POPH 组具有比 IPAH 更好的血流动力学特征。Dbil 与 POPH 和 IPAH 中 PVR 的升高呈相反关系。在总队列和 Dbil≥7μmol/L 的组中，POPH 患者的生存率优于 IPAH 患者，但应注意 POPH 患者的死亡人数有限。
