BACKGROUND & AIMS Long-term outcomes in portopulmonary hypertension (PoPH) are poorly studied in the current management era of pulmonary hypertension. We analysed the effect of pulmonary arterial hypertension (PAH)-targeted therapies, the survival and the predictors of death in a large contemporary cohort of patients with PoPH. METHODS Data from patients with PoPH consecutively enrolled in the French Pulmonary Hypertension Registry between 2007 and 2017 were collected. Effect of initial treatment strategies on functional class, exercise capacity and cardiopulmonary haemodynamics were analysed. Survival and its association with PAH- and hepatic-related characteristics were also examined. RESULTS Six hundred and thirty-seven patients (mean age 55±10 years; 58% male) were included. Fifty seven percent had mild cirrhosis, i.e. Child-Pugh (CP) stage A. The median MELD score was 11 (Q1-Q3; 9-15). Most patients (n=474; 74%) were initiated on monotherapy, either with a phosphodiesterase-5 inhibitor (n=336) or with an endothelin-receptor antagonist (n=128); 95 (15%) were initiated on double oral combination therapy and 5 (1%) on triple therapy. After a median treatment time of 4.5 months, there were significant improvements in functional class (p<0.001), 6-minute walk distance (6MWD) (p<0.0001) and pulmonary vascular resistance (p<0.0001). Overall survival rates were 84%, 69% and 51% at 1, 3 and 5 years, respectively. Baseline 6MWD, sex, age and MELD Score or CP stage were identified as independent prognostic factors. Survival from PoPH diagnosis was significantly better in the subgroup of patients who underwent liver transplantation (92%, 83% and 81% at 1, 3 and 5 years, respectively) CONCLUSION: Survival of patients with PoPH is strongly associated with the severity of liver disease. Patients who underwent liver transplantation had the best long-term outcomes.

中文翻译：

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当前肺动脉高压管理时代的门静脉高压

背景和目的 在当前的肺动脉高压管理时代，门静脉高压 (PoPH) 的长期结果研究很少。我们在一个大型当代 PoPH 患者队列中分析了肺动脉高压 (PAH) 靶向治疗的效果、生存率和死亡预测因素。方法 收集了 2007 年至 2017 年连续入组法国肺动脉高压登记处的 PoPH 患者的数据。分析了初始治疗策略对功能等级、运动能力和心肺血流动力学的影响。还检查了存活率及其与 PAH 和肝脏相关特征的关联。结果 包括 637 名患者（平均年龄 55±10 岁；58% 为男性）。57% 有轻度肝硬化，即 Child-Pugh (CP) A 阶段。 MELD 评分中位数为 11（Q1-Q3；9-15）。大多数患者（n=474；74%）开始单药治疗，使用磷酸二酯酶 5 抑制剂（n=336）或内皮素受体拮抗剂（n=128）；95 人 (15%) 开始接受双重口服联合治疗，5 人 (1%) 接受三联疗法。中位治疗时间为 4.5 个月后，功能等级 (p<0.001)、6 分钟步行距离 (6MWD) (p<0.0001) 和肺血管阻力 (p<0.0001) 均有显着改善。1、3 和 5 年的总生存率分别为 84%、69% 和 51%。基线 6MWD、性别、年龄和 MELD 评分或 CP 分期被确定为独立的预后因素。在接受肝移植的患者亚组中，PoPH 诊断的存活率显着提高（1 时分别为 92%、83% 和 81%，3 年和 5 年） 结论：PoPH 患者的存活率与肝病的严重程度密切相关。接受肝移植的患者的长期预后最好。