Clinical Department of Respiratory Diseases, University Hospitals of Leuven, Belgium (M.D., R.Q.).
Laboratory of Respiratory Diseases and Thoracic Surgery, Department of Chronic Diseases and Metabolism, University of Leuven, Belgium (M.D., R.Q.).
Circulation. 2024 Oct 22;150(17):1354-1365. doi: 10.1161/CIRCULATIONAHA.124.068610. Epub 2024 Sep 17.
The European Chronic Thromboembolic Pulmonary Hypertension (CTEPH) registry, conducted between 2007 and 2012, reported the major impact of pulmonary endarterectomy (PEA) on the long-term survival of patients with CTEPH. Since then, 2 additional treatments for inoperable CTEPH have become available: balloon pulmonary angioplasty (BPA), and an approved oral drug therapy with the guanylate cyclase stimulator riociguat. The current registry aimed to evaluate the effect of these new therapeutic approaches in a worldwide context.
Participation in this international global registry included 34 centers in 20 countries. Between February 2015 and September 2016, 1009 newly diagnosed, consecutive patients were included and followed until September 2019.
Overall, 605 patients (60%) underwent PEA and 185 (18%) underwent BPA; 76% of the 219 remaining patients not receiving mechanical intervention (ie, neither PEA nor BPA) were treated with pulmonary hypertension drugs. Of patients undergoing PEA and BPA, 38% and 78% also received drugs for pulmonary hypertension, respectively. Median age at diagnosis was higher in the BPA and No PEA/BPA groups than in the PEA group: 66 and 69, respectively, versus 60 years. Pulmonary vascular resistance (PVR) was similar in all groups, with an average of 643 dynes.s.cm. During the observation period (>3 years; ≤5.6 years), death was reported in 7%, 11%, and 27% of patients treated by PEA and BPA, and those receiving no mechanical intervention (<0.001). In Kaplan-Meier analysis, 3-year survival was 94%, 92%, and 71% in the 3 groups, respectively. PEA 3-year survival improved by 5% from that observed between 2007 and 2012. There was no survival difference in patients receiving vitamin K antagonists and non-vitamin K oral anticoagulants (=0.756). In Cox regression, reduced mortality was associated with: PEA and BPA in the global cohort; history of venous thromboembolism and lower PVR in the PEA group; lower right atrial pressure in the BPA group; and use of pulmonary hypertension drugs, oxygen therapy, and lower right atrial pressure, as well as functional class in the group receiving no mechanical intervention.
This second international CTEPH registry reveals important improvement in patient survival since the introduction of BPA and an approved drug for pulmonary hypertension. The type of anticoagulation regimen did not influence survival.
URL: https://www.clinicaltrials.gov; Unique identifier: NCT02656238.
欧洲慢性血栓栓塞性肺动脉高压（CTEPH）注册研究于 2007 年至 2012 年进行，报告了肺动脉内膜切除术（PEA）对 CTEPH 患者长期生存的重大影响。此后，又出现了两种治疗无法手术的 CTEPH 的新方法：球囊肺动脉成形术（BPA）和批准的口服药物治疗用鸟苷酸环化酶刺激剂 riociguat。本注册研究旨在评估这些新治疗方法在全球范围内的效果。
参与这项国际全球注册研究的包括 20 个国家的 34 个中心。2015 年 2 月至 2016 年 9 月期间，共纳入了 1009 例新诊断的连续患者，并随访至 2019 年 9 月。
总体而言，605 例患者（60%）接受了 PEA，185 例（18%）接受了 BPA；219 例未接受机械干预（即未行 PEA 或 BPA）的患者中，76%接受了肺动脉高压药物治疗。接受 PEA 和 BPA 的患者中，分别有 38%和 78%还接受了肺动脉高压药物治疗。BPA 组和无 PEA/BPA 组的中位诊断年龄高于 PEA 组，分别为 66 和 69 岁，而 PEA 组为 60 岁。所有组的肺血管阻力（PVR）相似，平均为 643 达因·秒·厘米。在观察期（>3 年；≤5.6 年）内，PEA 和 BPA 治疗组的患者死亡分别为 7%、11%和 27%，而未接受机械干预的患者为 27%（<0.001）。在 Kaplan-Meier 分析中，3 年生存率分别为 94%、92%和 71%。与 2007 年至 2012 年相比，PEA 组的 3 年生存率提高了 5%。接受维生素 K 拮抗剂和非维生素 K 口服抗凝剂的患者之间无生存差异（=0.756）。在 Cox 回归分析中，死亡率降低与以下因素相关：全球队列中的 PEA 和 BPA；PEA 组的静脉血栓栓塞史和较低的 PVR；BPA 组的较低的右心房压力；以及在未接受机械干预的患者中，肺动脉高压药物治疗、氧疗和较低的右心房压力以及功能分级。
这项第二次国际 CTEPH 注册研究显示，自 BPA 和一种批准用于肺动脉高压的药物问世以来，患者的生存状况有了显著改善。抗凝方案的类型并不影响生存。
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