Division of Pulmonary and Critical Care, Department of Internal Medicine, Mayo Clinic, Rochester, MN.
Division of Pulmonary and Critical Care, Department of Internal Medicine, Mayo Clinic, Jacksonville, FL.
Mayo Clin Proc. 2022 Dec;97(12):2236-2247. doi: 10.1016/j.mayocp.2022.08.020. Epub 2022 Nov 3.
To explore clinical characteristics, risk profiles, and outcomes of patients with portopulmonary hypertension (PoPH) who have contraindications to liver transplant (LT).
From the largest US single-institution registry of patients with PoPH, we analyzed 160 patients who did not receive LT between 1988 to 2019. Pulmonary arterial hypertension (PAH)-pertinent characteristics, hemodynamic features, treatments, and risk stratification were compared at baseline, first follow-up visit, and censor/death time.
Median survival for the entire cohort was 27.5 months from the diagnosis of PoPH. Overall survival was 89%, 77%, 51%, and 38% at 6 months, 1 year, 3 years, and 5 years, respectively. Survival was significantly affected by the severity of liver disease (P<.001). Most patients received PAH-specific therapies (136 [85%]), predominantly monotherapy (123 [77%)]. With treatment, significant improvements were noted in World Health Organization functional class (P=.04), 6-minute walk distance (P<.001), right ventricular function (P<.001), pulmonary vascular resistance (P<.001), and Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) Lite 2 score (P=.02) univariately. Per European Society of Cardiology risk stratification, no patient met full criteria for low risk at baseline or at follow-up. In a multivariate Cox risk model, 6-minute walk distance, right atrial pressure, pulmonary capillary wedge pressure, bilirubin level, and Model for End-Stage Liver Disease-sodium score of 15 or higher were associated with increased risk of death.
Patients with PoPH who did not undergo LT had a poor prognosis. This persisted despite use of PAH-specific therapies and significant improvements in hemodynamics, echocardiography parameters of right ventricle function, 6-minute walk distance, and World Health Organization functional class.
探讨因存在肝移植（LT）禁忌而未接受 LT 的门脉高压性肺高血压（PoPH）患者的临床特征、风险概况和结局。
我们分析了 1988 年至 2019 年间来自美国最大的单中心 PoPH 患者登记处的 160 例未接受 LT 的患者。比较了基线时、首次随访时和截止/死亡时的肺动脉高压（PAH）相关特征、血流动力学特征、治疗和风险分层。
整个队列的中位生存时间为 PoPH 诊断后的 27.5 个月。总体生存率分别为 6 个月时的 89%、1 年时的 77%、3 年时的 51%和 5 年时的 38%。生存率显著受肝病严重程度的影响（P<.001）。大多数患者接受了 PAH 特异性治疗（136 [85%]），主要是单药治疗（123 [77%]）。随着治疗，世界卫生组织功能分级（P=.04）、6 分钟步行距离（P<.001）、右心室功能（P<.001）、肺血管阻力（P<.001）和登记以评估早期和长期肺动脉高压疾病管理（REVEAL）Lite 2 评分（P=.02）均有显著改善。根据欧洲心脏病学会风险分层，没有患者在基线或随访时符合低危的全部标准。在多变量 Cox 风险模型中，6 分钟步行距离、右心房压、肺毛细血管楔压、胆红素水平和 Model for End-Stage Liver Disease-sodium 评分 15 或更高与死亡风险增加相关。
未接受 LT 的 PoPH 患者预后较差。尽管使用了 PAH 特异性治疗，血流动力学、右心室功能超声心动图参数、6 分钟步行距离和世界卫生组织功能分级均有显著改善，但这种情况仍持续存在。
