Hughes R J, Jais X, Bonderman D, Suntharalingam J, Humbert M, Lang I, Simonneau G, Pepke-Zaba J
Pulmonary Vascular Diseases Unit, Papworth Hospital, Cambridgeshire, CB3 8RE, UK.
Eur Respir J. 2006 Jul;28(1):138-43. doi: 10.1183/09031936.06.00135905. Epub 2006 Apr 12.
The treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH) is pulmonary endarterectomy (PEA). However, many patients develop a severe progressive small vessel pulmonary arteriopathy that is inaccessible to surgical intervention and is associated with poor survival. The purpose of the present study was to evaluate the medium-term efficacy and safety of the dual endothelin receptor antagonist, bosentan, in inoperable CTEPH. Forty-seven patients with inoperable CTEPH (distal disease or persistent pulmonary hypertension following PEA) underwent evaluation after 1 yr of bosentan therapy. Outcomes included assessment of 6-min walk test (6MWT), haemodynamics and World Health Organization functional classification. Monitoring of serious adverse effects and changes in therapy was undertaken. Patients showed sustained improvements in 6MWT (49+/-8 m), functional classification, cardiac index (+0.2+/-0.07 L.min(-1).m(-2)) and total pulmonary resistance (-139+/-42 dyn.s.cm(-5)). Those patients with persisting pulmonary hypertension following PEA showed the greatest improvement. One-yr survival was 96%, and bosentan was well tolerated with only one patient developing deranged liver function. Although all patients with chronic thromboembolic pulmonary hypertension should be considered for pulmonary endarterectomy, bosentan provides an alternative medical therapy to improve function and delay the progression of this devastating disease in those in whom surgery is not suitable.
慢性血栓栓塞性肺动脉高压（CTEPH）的首选治疗方法是肺动脉内膜剥脱术（PEA）。然而，许多患者会出现严重的进行性小血管肺动脉病变，这种病变无法通过手术干预，且与生存率低相关。本研究的目的是评估双重内皮素受体拮抗剂波生坦对无法进行手术的CTEPH患者的中期疗效和安全性。47例无法进行手术的CTEPH患者（远端病变或PEA术后持续性肺动脉高压）在接受波生坦治疗1年后接受评估。结果包括6分钟步行试验（6MWT）、血流动力学和世界卫生组织功能分级评估。对严重不良反应和治疗变化进行监测。患者的6MWT（49±8米）、功能分级、心脏指数（+0.2±0.07升·分钟-1·米-2）和总肺阻力（-139±42达因·秒·厘米-5）持续改善。那些PEA术后仍有持续性肺动脉高压的患者改善最为明显。1年生存率为96%，波生坦耐受性良好，只有1例患者出现肝功能紊乱。虽然所有慢性血栓栓塞性肺动脉高压患者都应考虑进行肺动脉内膜剥脱术，但波生坦提供了一种替代药物治疗方法，可改善功能并延缓这种毁灭性疾病在那些不适合手术的患者中的进展。
