DuBrock Hilary M, Jose Arun, Arendse Sarah, Martin Nicolas, Studer Sean, Rosenberg Daniel
Division of Pulmonary, Critical Care and Sleep Medicine, Mayo Clinic, Rochester, MN.
Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati, Cincinnati, OH.
Transplant Direct. 2024 Feb 21;10(3):e1586. doi: 10.1097/TXD.0000000000001586. eCollection 2024 Mar.
Portopulmonary hypertension (PoPH) occurs in patients with advanced liver disease and can be a contraindication to liver transplant (LT). Improvement of hemodynamic parameters with pulmonary arterial hypertension (PAH) therapies (including endothelin receptor antagonists [ERAs]) may help some patients to become eligible for LT.
We conducted a retrospective secondary data analysis to describe the clinical course and management of PoPH in patients on a US registry LT waitlist and outcomes in patients receiving an ERA.
At the time of LT waitlist entry (1996-2019), patient characteristics and disease severity were similar in the 685 patients with PoPH enrolled overall (LT waitlist data set) and the 420 of them who underwent LT (LT data set). Most patients (92.0%) had a model for end-stage liver disease exception granted before entering the LT waitlist. Patients spent a median of 8.9 mo (interquartile range, 3.7-19.7) on the LT waitlist before undergoing LT. Overall, 77.1% of patients received PAH treatment at LT waitlist entry (ERAs, 30.1%). Hemodynamic parameters improved in ≥95% of patients between the first assessment versus the second (median interval, 9 mo) and last assessments (median interval, 14 mo). At the first assessment, 49.6% of patients had mean pulmonary arterial pressure ≥45 mm Hg versus 2.6% and 1.8% of patients at the second and last assessments, respectively; 47.5% of patients had pulmonary vascular resistance >450 dynes·s/cm versus 0.9% and 0.2% of patients at the second and last assessments. One-year survival was 90.6% (95% confidence interval [CI], 87.6-92.9) following LT waitlist entry and was 86.4% (95% CI, 82.6-89.5) after LT; 5-y survival was 67.4% (95% CI, 60.0-73.8) while on the LT waitlist (before LT) and was 75.6% (95% CI, 70.4-80.0) following LT.
This large US study of patients with PoPH on an LT waitlist confirms that effective PAH treatments can help patients achieve acceptable hemodynamics, providing the opportunity to undergo LT.
门肺高压（PoPH）发生于晚期肝病患者，可能是肝移植（LT）的禁忌证。采用肺动脉高压（PAH）治疗（包括内皮素受体拮抗剂[ERA]）改善血流动力学参数可能有助于部分患者符合LT的条件。
我们进行了一项回顾性二次数据分析，以描述美国肝移植等候名单上PoPH患者的临床病程和管理情况，以及接受ERA治疗患者的结局。
在进入肝移植等候名单时（1996 - 2019年），总体纳入的685例PoPH患者（肝移植等候名单数据集）和其中接受肝移植的420例患者（肝移植数据集）的患者特征和疾病严重程度相似。大多数患者（92.0%）在进入肝移植等候名单前获得了终末期肝病模型例外资格。患者在接受肝移植前在肝移植等候名单上的中位时间为8.9个月（四分位间距，3.7 - 19.7个月）。总体而言，77.1%的患者在进入肝移植等候名单时接受了PAH治疗（ERA，30.1%）。≥95%的患者在首次评估与第二次评估（中位间隔9个月）以及末次评估（中位间隔14个月）之间血流动力学参数得到改善。在首次评估时，49.6%的患者平均肺动脉压≥45 mmHg，而在第二次和末次评估时分别为2.6%和1.8%；47.5%的患者肺血管阻力>450 dynes·s/cm，而在第二次和末次评估时分别为0.9%和0.2%。进入肝移植等候名单后1年生存率为90.6%（95%置信区间[CI]，87.6 - 92.9），肝移植后为86.4%（95% CI，82.6 - 89.5）；在肝移植等候名单上（肝移植前）5年生存率为67.4%（95% CI，60.0 - 73.8），肝移植后为75.6%（95% CI，70.4 - 80.0）。
这项针对美国肝移植等候名单上PoPH患者的大型研究证实，有效的PAH治疗可帮助患者实现可接受的血流动力学状态，从而获得接受肝移植的机会。
