Department of Gastroenterology and Hepatology, Ghent University-Ghent University Hospital, Ghent, Belgium.
Department of Gastroenterology, Hepatopancreatology and Digestive Oncology, C.U.B. Hôpital Erasme, Université Libre de Bruxelles, Bruxelles, Belgium.
Hepatology. 2020 Nov;72(5):1701-1716. doi: 10.1002/hep.31164. Epub 2020 Oct 19.
Untreated portopulmonary hypertension (PoPH) carries a poor prognosis. Previous reports have described vasomodulator (VM) therapy and liver transplantation (LT) as treatment options. We aimed to provide summary estimates on the endpoints of pulmonary hemodynamics and survival in patients with PoPH, treated with different modalities.
We performed a systematic review with meta-analysis of mainly observational and case-control studies describing no treatment, VM, LT, or VM + LT in patients with PoPH. Twenty-six studies (1,019 patients) were included. Both VM and VM + LT improve pulmonary hemodynamics. A substantial proportion of patients treated with VM become eligible for LT (44%; 95% confidence interval [CI], 31-58). Pooled estimates for 1-, and 3-year postdiagnosis survival in patients treated with VM were 86% (95% CI, 81-90) and 69% (95% CI, 50-84) versus 82% (95% CI, 52-95) and 67% (95% CI, 53-78) in patients treated with VM + LT. Of note, studies reporting on the effect of VM mainly included Child-Pugh A/B patients, whereas studies reporting on VM + LT mainly included Child-Pugh B/C. Seven studies (238 patients) included both patients who received VM only and patients who received VM + LT. Risk of death in VM-only-treated patients was significantly higher than in patients who could be transplanted as well (odds ratio, 3.5; 95% CI, 1.4-8.8); however, importantly, patients who proceeded to transplant had been selected very strictly. In 50% of patients, VM can be discontinued post-LT (95% CI, 38-62).
VM and VM + LT both improve pulmonary hemodynamics and prognosis in PoPH. In a strictly selected subpopulation of cases where LT is indicated based on severe liver disease and where LT is considered safe and feasible, treatment with VM + LT confers a better prognosis. Considering successful VM, 44% can proceed to LT, with half being able to postoperatively stop medication.
未经治疗的门脉高压性肺高血压（PoPH）预后不良。既往研究已报道血管扩张剂（VM）治疗和肝移植（LT）为治疗选择。本研究旨在汇总 PoPH 患者采用不同治疗方法时肺血流动力学和生存率的终点数据。
我们对主要为观察性和病例对照研究的系统综述进行了荟萃分析，这些研究描述了 PoPH 患者未治疗、VM、LT 或 VM+LT 的情况。共纳入 26 项研究（1019 例患者）。VM 和 VM+LT 均可改善肺血流动力学。相当一部分接受 VM 治疗的患者有资格接受 LT（44%；95%置信区间[CI]，31-58）。接受 VM 治疗的患者在诊断后 1 年和 3 年的估计生存率分别为 86%（95%CI，81-90）和 69%（95%CI，50-84），而接受 VM+LT 治疗的患者分别为 82%（95%CI，52-95）和 67%（95%CI，53-78）。值得注意的是，报告 VM 疗效的研究主要纳入了 Child-Pugh A/B 患者，而报告 VM+LT 的研究主要纳入了 Child-Pugh B/C 患者。有 7 项研究（238 例患者）同时纳入了仅接受 VM 治疗的患者和接受 VM+LT 治疗的患者。仅接受 VM 治疗的患者死亡风险明显高于可进行移植的患者（比值比，3.5；95%CI，1.4-8.8）；然而，重要的是，接受移植的患者是经过严格选择的。在 50%的患者中，LT 后 VM 可以停药（95%CI，38-62）。
VM 和 VM+LT 均可改善 PoPH 的肺血流动力学和预后。在根据严重肝脏疾病选择 LT 且 LT 被认为安全可行的严格选择亚群中，VM+LT 治疗可改善预后。考虑到 VM 治疗成功，44%的患者可进行 LT，其中一半患者术后可停止用药。
