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Medical management of chronic thromboembolic pulmonary hypertension

机译：慢性血栓栓塞性肺动脉高压的医疗管理

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Chronic thromboembolic pulmonary hypertension (CTEPH) results from incomplete resolution of acute pulmonary emboli; organised into fibrotic material that obstructs large pulmonary arteries; and distal small-vessel arteriopathy. Pulmonary endarterectomy (PEA) is the treatment of choice for eligible patients with CTEPH; in expert centres; PEA has low in-hospital mortality rates and excellent long-term survival. Supportive medical therapy consists of lifelong anticoagulation plus diuretics and oxygen; as needed.An important recent advance in medical therapy for CTEPH is the arrival of medical therapies for patients with inoperable disease or persistent/recurrent pulmonary hypertension after PEA. The soluble guanylate cyclase stimulator riociguat is licensed for the treatment of CTEPH in patients with inoperable disease or with recurrent/persistent pulmonary hypertension after PEA. Clinical trials of this agent have shown improvements in patients' haemodynamics and exercise capacity. Phosphodiesterase-5 inhibitors; endothelin receptor antagonists and prostanoids have been used in the treatment of CTEPH; but evidence of benefit is limited. Challenges in the future development of medical therapy for CTEPH include better understanding of the underlying pathology; end-points to monitor the condition's progress; and the optimisation of pulmonary arterial hypertension therapies in relation to diverse patient characteristics and emerging options such as balloon pulmonary angioplasty.
机译：慢性血栓栓塞性肺动脉高压（CTEPH）是由急性肺栓塞的不完全解决导致的;组织成阻塞大肺动脉的纤维化物质;和远端小血管动脉病变。肺动脉内膜切除术（PEA）是合格的CTEPH患者的首选治疗方法;在专家中心; PEA的院内死亡率低，长期存活率高。支持性药物治疗包括终生抗凝，利尿剂和氧气。 CTEPH药物治疗的最新重要进展是，对于无法手术的疾病或PEA后持续性/复发性肺动脉高压患者的药物治疗已经到来。可溶性鸟苷酸环化酶刺激剂riociguat被许可用于患有不能手术的疾病或PEA后复发/持续性肺动脉高压的患者进行CTEPH的治疗。该药物的临床试验表明，患者的血流动力学和运动能力得到改善。磷酸二酯酶5抑制剂;内皮素受体拮抗剂和类前列腺素已用于治疗CTEPH;但是受益的证据有限。 CTEPH药物治疗未来发展中的挑战包括更好地了解潜在的病理学。监测病情进展的端点;以及针对各种患者特征和新出现的选择（例如球囊肺血管成形术）进行的肺动脉高压疗法的优化。

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