Wiedenroth Christoph B, Jenkins David, Brenot Philippe, Lang Irene M, Matsubara Hiromi, Pepke-Zaba Joanna, Channick Richard, Jais Xavier, Simonneau Gérald, Delcroix Marion, Kerr Kim, de Perrot Marc, Mayer Eckhard, Pretorius Victor, Mahmud Ehtisham, Poch David, Shimokawahara Hiroto, Steinberg Zachary L, Madani Michael
Department of Thoracic Surgery, Kerckhoff Heart and Thorax Center, Bad Nauheim, Germany.
Cardiothoracic Surgery, Royal Papworth Hospital, Cambridge, UK.
J Heart Lung Transplant. 2025 Jul;44(7S):S8-S14. doi: 10.1016/j.healun.2025.02.1691.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe and progressive disease. Three treatment modalities are available: pulmonary endarterectomy, balloon pulmonary angioplasty, and pulmonary hypertension (PH) medical therapy. Both mechanical therapies may also be considered in patients with chronic thromboembolic pulmonary disease without PH. Patients should be referred to expert centers, as full surgical evaluation is the primary target and further evaluation for additional or alternative treatment strategies by an experienced multidisciplinary team is mandatory. Surgery remains the treatment of choice for CTEPH. PH medical therapies are available for inoperable patients, or those with residual PH postprocedure. Furthermore, interventional therapy is recommended in inoperable patients with appropriate target lesions. Multimodal treatment strategies are common in inoperable patients. In recent years, there is a growing expertise in combining all 3 modalities in carefully selected patients. In the current era, the peri-interventional mortality risks are low (<3% for pulmonary endarterectomy and <1% for balloon pulmonary angioplasty in expert centers), and outcomes are excellent for the vast majority of patients with CTEPH.
慢性血栓栓塞性肺动脉高压（CTEPH）是一种严重的进行性疾病。有三种治疗方式可供选择：肺动脉内膜剥脱术、球囊肺动脉成形术和肺动脉高压（PH）药物治疗。对于无PH的慢性血栓栓塞性疾病患者，也可考虑这两种机械治疗方法。患者应转诊至专家中心，因为全面的手术评估是主要目标，由经验丰富的多学科团队进行进一步评估以确定额外或替代治疗策略是必不可少的。手术仍然是CTEPH的首选治疗方法。PH药物治疗适用于无法手术的患者或术后有残余PH的患者。此外，对于有合适靶病变的无法手术的患者，建议采用介入治疗。多模式治疗策略在无法手术的患者中很常见。近年来，在精心挑选的患者中联合使用这三种治疗方式的专业知识越来越多。在当前时代，围介入期死亡风险较低（在专家中心，肺动脉内膜剥脱术的死亡率<3%，球囊肺动脉成形术的死亡率<1%），并且绝大多数CTEPH患者的治疗效果良好。
