Background

It is unknown whether pulmonary arterial hypertension (PAH) risk stratification instruments could be helpful to support the decision to list a patient for lung transplantation (LT). Our aim was to evaluate contemporary risk assessment tools in a cohort of PAH patients listed for LT.

Methods

Consecutive PAH patients (without pulmonary veno-occlusive disease or unrepaired congenital heart disease) listed for LT at the French Pulmonary Hypertension Reference Center between January 2006 and December 2018 were included. At the time of listing, risk stratification was assessed using the ESC/ERS criteria, the REVEAL Lite 2 score and the COMPERA 2.0 method. The primary end point was overall survival after LT listing. Secondary outcome measures were mortality on waiting list and posttransplant survival.

Results

One hundred and two patients were enrolled (mean age 38 ± 13 years, 69% females). Overall survival after listing was 72%, 58% and 46% at 1, 3 and 5 years respectively. Survival after LT listing was lower in “high-risk” patients according to the ESC/ERS criteria (p = 0.0001) and the REVEAL Lite 2 score (p = 0.04). The COMPERA 2.0 method discriminated post-listing survival of patients at high-risk, intermediate-high and intermediate-low risk (p = 0.04). The proportion of patients requiring urgent transplantation and extracorporeal life support as a bridge to transplantation was higher in the “high-risk” patients. Posttransplant survival was significantly lower in “high-risk” patients according to the ESC/ERS criteria (p = 0.0004).

Conclusions

High-risk PAH patients at the time of LT listing have poor outcomes, suggesting that LT should be considered earlier in the course of PAH remaining refractory to triple combination therapy with a parenteral prostacyclin.

中文翻译：

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肺移植上市时肺动脉高压患者的风险分层

背景

尚不清楚肺动脉高压 (PAH) 风险分层工具是否有助于支持将患者列入肺移植 (LT) 的决定。我们的目的是评估列于 LT 的 PAH 患者队列中的当代风险评估工具。

方法

包括 2006 年 1 月至 2018 年 12 月在法国肺动脉高压参考中心列入 LT 的连续 PAH 患者（无肺静脉闭塞性疾病或未修复的先天性心脏病）。在上市时，使用 ESC/ERS 标准、REVEAL Lite 2 评分和 COMPERA 2.0 方法评估风险分层。主要终点是 LT 上市后的总生存期。次要结局指标是等待名单上的死亡率和移植后存活率。

结果

招募了 102 名患者（平均年龄 38 ± 13 岁，69% 为女性）。上市后 1 年、3 年和 5 年的总生存率分别为 72%、58% 和 46%。根据 ESC/ERS 标准 ( p  = 0.0001) 和 REVEAL Lite 2 评分 ( p  = 0.04) ，“高危”患者的 LT 上市后存活率较低。COMPERA 2.0 方法区分了高风险、中高风险和中低风险患者的上市后生存率 ( p  = 0.04)。在“高危”患者中，需要紧急移植和体外生命支持作为移植过渡的患者比例较高。根据 ESC/ERS 标准（ p  = 0.0004），“高危”患者的移植后存活率显着降低。

结论

在 LT 上市时的高危 PAH 患者预后较差，这表明在 PAH 与肠外前列环素三联疗法仍难以治疗的过程中，应更早考虑 LT。