Division of Pulmonary and Critical Care Medicine, Department of Medicine, Mayo Clinic, Rochester, MN.
Division of Pulmonary and Critical Care Medicine, Department of Medicine, Mayo Clinic, Scottsdale, AZ.
Chest. 2021 Jan;159(1):328-336. doi: 10.1016/j.chest.2020.07.081. Epub 2020 Aug 13.
Portopulmonary hypertension (POPH), pulmonary arterial hypertension that develops in the setting of portal hypertension, can lead to right-sided heart failure and death. Being female is a known risk factor for POPH, but little is known about the effect of sex on clinical manifestations, hemodynamics, treatment response, and survival.
We sought to characterize sex differences in clinical characteristics, pulmonary hemodynamics, treatment response, and survival in patients with POPH.
We performed a retrospective cohort study of adult candidates for liver transplant (LT) who had POPH within the Organ Procurement and Transplantation Network database. Females and males were compared. Multivariate regression was performed to assess the association between sex and pulmonary vascular resistance (PVR) and survival. Patients were also stratified by age (50 years) to determine how age modifies the relationship between sex and hemodynamics and survival.
We included 190 adults (103 male, 87 female). Compared with men, women had a lower model for end-stage liver disease (MELD) score (12.1± 4.2 vs 13.8 ± 4.9; P = .01) and were more likely to have autoimmune liver disease. Women had a higher baseline PVR (610.6 ± 366.6 vs 461.0 ± 185.3 dynes-s-cm; P < .001) and posttreatment PVR (244.6 ± 119.5 vs 202.0 ± 87.7 dynes-s-cm; P = .008) and a greater treatment response (ΔPVR) (-359.3 ± 381.9 vs -260.2 ± 177.3 dynes-s-cm; P = .03). In multivariate analysis, female sex (or gender) remained associated with a higher baseline PVR (P = .008). Women and men had overall similar survival (P > .05). When patients were stratified by age, being female was independently associated with worse waiting list survival after adjusting for MELD and PVR in younger patients (HR, 6.61; 95% CI, 1.25-35.08; P = .03) but not in older patients.
Compared with male candidates, female candidates for LT who had POPH had a higher PVR and lower MELD score and were more likely to have autoimmune liver disease. Women and men had similar overall survival, but female sex (or gender) was associated with worse survival in younger patients.
门脉高压症（POPH）是指在门脉高压症的背景下发生的肺动脉高压，可导致右侧心力衰竭和死亡。女性是 POPH 的已知危险因素，但对于性别对临床表现、血流动力学、治疗反应和生存率的影响知之甚少。
我们旨在描述 POPH 患者的临床特征、肺血流动力学、治疗反应和生存率的性别差异。
我们对器官获取和移植网络数据库中患有 POPH 的成人肝移植（LT）候选者进行了回顾性队列研究。比较了女性和男性。采用多元回归评估性别与肺血管阻力（PVR）和生存率之间的关系。还根据年龄（50 岁）对患者进行分层，以确定年龄如何改变性别与血流动力学和生存率之间的关系。
我们纳入了 190 名成年人（103 名男性，87 名女性）。与男性相比，女性的终末期肝病模型（MELD）评分较低（12.1 ± 4.2 与 13.8 ± 4.9；P =.01），更可能患有自身免疫性肝病。女性的基线 PVR 较高（610.6 ± 366.6 与 461.0 ± 185.3 dynes-s-cm；P <.001）和治疗后 PVR 较高（244.6 ± 119.5 与 202.0 ± 87.7 dynes-s-cm；P =.008），治疗反应更大（ΔPVR）（-359.3 ± 381.9 与-260.2 ± 177.3 dynes-s-cm；P =.03）。多元分析中，女性（或性别）仍与基线 PVR 较高相关（P =.008）。女性和男性的总体生存率相似（P >.05）。当根据年龄对患者进行分层时，在校正 MELD 和 PVR 后，年轻患者的女性独立与等待名单生存率较差相关（HR，6.61；95%CI，1.25-35.08；P =.03），而老年患者则不然。
与男性候选者相比，患有 POPH 的 LT 女性候选者的 PVR 更高，MELD 评分更低，更可能患有自身免疫性肝病。女性和男性的总体生存率相似，但女性（或性别）与年轻患者的生存率较差相关。
