Division of Pulmonary and Critical Care Medicine, University of California, San Diego, La Jolla, CA 92037-1300, USA.
Eur Respir Rev. 2010 Mar;19(115):68-71. doi: 10.1183/09059180.00007909.
Although pulmonary endarterectomy remains the treatment of choice for patients with chronic thromboembolic pulmonary hypertension (CTEPH), not all patients will benefit from or receive this highly specialised surgery. Patients whose CTEPH is deemed inoperable by an experienced centre and patients with persistent pulmonary hypertension after surgery are candidates for trial of pulmonary arterial hypertension (PAH) specific pharmacotherapies. However, the currently available PAH specific pharmacotherapies have not demonstrated a clear benefit in either of these patient groups. Accordingly, PAH therapies remain off-label for use in CTEPH. Riociguat (BAY 63-2521) is a stimulator of soluble guanylate cyclase, and may represent a novel agent in the treatment of select patients with CTEPH. Pre-clinical and human phase II studies with riociguat have reported promising results, and a multinational, randomised, controlled, double-blinded phase III study is currently underway to investigate the effect of riociguat in patients with inoperable CTEPH and those with persistent or recurrent pulmonary hypertension following pulmonary endarterectomy.
虽然肺血管内膜切除术仍然是慢性血栓栓塞性肺动脉高压（CTEPH）患者的首选治疗方法，但并非所有患者都能从这种高度专业化的手术中受益。经验丰富的中心认为 CTEPH 无法手术的患者和手术后持续性肺动脉高压的患者是肺动脉高压（PAH）特定药物治疗试验的候选者。然而，目前可用的 PAH 特定药物治疗在这两个患者群体中均未显示出明显获益。因此，PAH 治疗在 CTEPH 中的应用仍属超适应证用药。利奥西呱（BAY 63-2521）是可溶性鸟苷酸环化酶的激动剂，可能是治疗特定 CTEPH 患者的一种新型药物。利奥西呱的临床前和 II 期人体研究报告了有前景的结果，目前正在进行一项多中心、随机、对照、双盲 III 期研究，以评估利奥西呱在无法手术的 CTEPH 患者以及肺血管内膜切除术后持续性或复发性肺动脉高压患者中的疗效。
