Pharmacological therapy for patients with chronic thromboembolic pulmonary hypertension.
Pharmacological therapy for patients with chronic thromboembolic pulmonary hypertension.
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DOI：
10.1183/16000617.00001015
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发表时间：
2015-06
影响因子：
7.5
通讯作者：
Hoeper, Marius M
中科院分区：
文献类型：
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作者：
Hoeper, Marius M
慢性血栓栓塞性肺动脉高压(CTEPH)是一种罕见但危及生命的疾病，由未解决的血栓栓塞性阻塞引起。肺内膜切除术(PEA)是金标准的治疗方法，因为它具有潜在的治愈能力；然而，并不是所有的患者都被认为是可以手术的，多达三分之一的患者在手术后持续或复发。肺动脉高压(PAH)和CTEPH具有相似的临床表现和组织病理学特征，因此，在缺乏有效治疗方法的情况下，经常给CTEPH患者开出治疗PAH有效的药物。然而，这一策略的临床证据并不令人信服。一些小型非对照试验研究了CTEPH中的内皮素受体拮抗剂、前列环素类似物和磷酸二酯酶5型抑制剂，结果喜忧参半，内皮素受体拮抗剂波生坦的III期研究只满足其两个共同主要终点中的一个。然而，最近，可溶性鸟苷环化酶刺激剂riociguat在第三阶段胸腔研究(慢性血栓栓塞性肺高压可溶性鸟苷环化酶刺激剂试验)取得阳性结果后，在美国和欧洲被批准用于治疗不可手术或持续性/复发性CTEPH。本文综述了目前药物治疗CTEPH的证据。不能手术或持续性/复发性慢性前列腺炎的药物治疗及未来的护理标准http://ow.ly/KMUQV
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but life-threatening disease resulting from unresolved thromboembolic obstructions. Pulmonary endarterectomy (PEA) surgery is the gold-standard treatment as it is potentially curative; however, not all patients are deemed operable and up to one-third have persistent or recurrent CTEPH after the procedure. Pulmonary arterial hypertension (PAH) and CTEPH have similar clinical presentations and histopathological features, so agents shown to be effective in PAH have often been prescribed to patients with CTEPH in the absence of proven therapies. However, clinical evidence for this strategy is not compelling. A number of small uncontrolled trials have investigated endothelin receptor antagonists, prostacyclin analogues and phosphodiesterase type 5 inhibitors in CTEPH with mixed results, and a phase III study of the endothelin receptor antagonist bosentan met only one of its two co-primary end-points. Recently, however, the soluble guanylate cyclase stimulator, riociguat, was approved in the USA and Europe for the treatment of inoperable or persistent/recurrent CTEPH following positive results from the phase III CHEST study (Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase–Stimulator Trial). This article reviews the current evidence for the use of pharmacological therapies in CTEPH. A review of pharmacological treatment of inoperable or persistent/recurrent CTEPH and the future standard of care http://ow.ly/KMUQV