AP-HP, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Centre, Hôpital Bicêtre, DMU 5 Thorinno, Le Kremlin-Bicêtre, France; Université Paris-Saclay, Faculty of Medicine, Le Kremlin-Bicêtre, France; INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
Université Paris-Saclay, Faculty of Medicine, Le Kremlin-Bicêtre, France; INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Le Plessis Robinson, France; Service de Pneumologie et Transplantation Pulmonaire, Hôpital Marie-Lannelongue, Le Plessis-Robinson, France.
J Heart Lung Transplant. 2022 Sep;41(9):1285-1293. doi: 10.1016/j.healun.2022.06.001. Epub 2022 Jun 6.
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.
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.
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).
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.
目前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）名单的决策。我们的目的是评估 LT 患者名单中 PAH 患者的当代风险评估工具。
连续纳入 2006 年 1 月至 2018 年 12 月在法国肺动脉高压参考中心接受 LT 名单的 PAH 患者（无肺静脉阻塞性疾病或未修复的先天性心脏病）。在列入名单时，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 名单后的总生存率。次要结局指标是等待名单上的死亡率和移植后生存率。
共纳入 102 例患者（平均年龄 38±13 岁，69%为女性）。名单后 1、3 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 名单后的生存率较低。COMPERA 2.0 方法可区分高危、中高危和中低危患者的名单后生存率（p=0.04）。“高危”患者中需要紧急移植和体外生命支持作为移植桥接的患者比例较高。根据 ESC/ERS 标准，“高危”患者的移植后生存率显著较低（p=0.0004）。
LT 名单时的高危 PAH 患者预后不良，表明对于对三联合治疗仍有反应的 PAH 患者，LT 应更早考虑。
