Division of Biological Sciences, Department of Medicine, University of Chicago, 5841 S. Maryland Avenue, Chicago, IL, 60637, USA.
Division of Respiratory Disease and Tuberculosis, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkoknoi, Bangkok, 10700, Thailand.
Lung. 2022 Jun;200(3):283-299. doi: 10.1007/s00408-022-00539-w. Epub 2022 May 29.
Chronic thromboembolic pulmonary hypertension (CTEPH), defined as precapillary pulmonary hypertension (PH) by right heart catheterization and imaging consistent with chronic thromboembolism, is a long-term complication of pulmonary embolism (PE). Pathobiological mechanisms involve pulmonary artery occlusion from organized thromboembolic material despite at least three months of uninterrupted therapeutic anticoagulation following acute PE and secondary microvasculopathy. Delay in diagnosis and management of CTEPH is associated with poor outcomes. High clinical suspicion, comprehensive assessment of residual dyspnea or exercise intolerance in the aftermath of PE and accurate interpretation of computed tomography pulmonary angiography (CTPA) are pivotal steps in the diagnosis. Ventilation-perfusion (V/Q) scan is the preferred initial radiologic screening tool as normal V/Q essentially rules out CTEPH. Any mismatched perfusion defect on the V/Q scan in the setting of PH or any finding compatible with chronic thromboembolism on CTPA should prompt referral to an expert CTEPH center. Once the diagnosis is verified, all eligible patients should be offered pulmonary thromboendarterectomy (PTE). Pulmonary vasodilators or balloon pulmonary angioplasty are safe and effective in inoperable or post-PTE persistent/recurrent CTEPH. During the course of their disease, a patient may receive a combination of treatments, at times consisting of all three strategies. Lifelong therapeutic anticoagulation is recommended for CTEPH.
慢性血栓栓塞性肺动脉高压（CTEPH）定义为右心导管检查和影像学检查符合慢性血栓栓塞的前毛细血管性肺动脉高压（PH），是肺栓塞（PE）的长期并发症。发病机制涉及到尽管在急性 PE 后至少三个月不间断的抗凝治疗，仍有血栓栓塞物质导致的肺动脉闭塞，以及继发性微血管病变。CTEPH 的诊断和治疗延误与不良预后相关。在 PE 后出现持续性呼吸困难或运动耐量下降时，高度怀疑临床症状、全面评估以及准确解读计算机断层肺动脉造影（CTPA），是诊断的关键步骤。通气-灌注（V/Q）扫描是首选的初始影像学筛查工具，因为正常的 V/Q 基本上可以排除 CTEPH。在 PH 情况下 V/Q 扫描上存在任何不匹配的灌注缺损，或 CTPA 上存在任何符合慢性血栓栓塞的发现，都应提示转介到 CTEPH 专家中心。一旦确诊，所有符合条件的患者均应接受肺动脉血栓内膜切除术（PTE）。在不可手术或 PTE 后持续性/复发性 CTEPH 中，肺血管扩张剂或球囊肺动脉成形术是安全有效的。在疾病过程中，患者可能会接受多种治疗方法的联合治疗，有时甚至会同时使用这三种策略。对于 CTEPH，建议终身进行抗凝治疗。
