Subias Pilar Escribano, Cano María José Ruiz, Flox Angela
Unidad de Hipertensión Pulmonar, Servicio de Cardiología, Hospital 12 de Octubre, Madrid, España.
Arch Bronconeumol. 2009 Jun;45 Suppl 6:35-9. doi: 10.1016/S0300-2896(09)73501-X.
Pulmonary thromboendarterectomy is the treatment of choice in patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, specific medical treatment of pulmonary hypertension (PH) can be an alternative or play a complementary role to surgery. Thus, in patients unsuitable for surgery due to distal thrombotic obstruction, residual or persistent PH after surgery or very severe PH and a high-risk hemodynamic profile, medical treatment may improve their clinical course and the outcome of thromboendarterectomy. Patients with distal obstruction in the pulmonary tree and those with residual PH after surgery show clinical and hemodynamic deterioration due to progression of the pulmonary vascular disease in the smallcaliber arterioles. Conventional treatment with diuretics, anticoagulants and oxygen therapy has been demonstrated to have little effectiveness. In the last decade, numerous drugs have been developed for the treatment of PH: prostacyclin analogs, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors acting principally in vascular remodelling of small-caliber arterioles. Although evidence of the effectiveness of these drugs in PH and the histological similarity of small-vessel vasculopathy in CTEPH to that of other forms of PH provide the main rationale for the use of these drugs in patients with CTEPH, the evidence from clinical trials is still limited.
肺动脉血栓内膜剥脱术是慢性血栓栓塞性肺动脉高压（CTEPH）患者的首选治疗方法。然而，肺动脉高压（PH）的特定药物治疗可以作为手术的替代方法或起到辅助作用。因此，对于因远端血栓阻塞、术后残留或持续存在PH或非常严重的PH以及高风险血流动力学特征而不适合手术的患者，药物治疗可能会改善他们的临床病程和血栓内膜剥脱术的结果。肺血管树远端阻塞的患者以及术后残留PH的患者，由于小口径小动脉中肺血管疾病的进展，会出现临床和血流动力学恶化。利尿剂、抗凝剂和氧疗的传统治疗已被证明效果甚微。在过去十年中，已经开发出许多用于治疗PH的药物：前列环素类似物、内皮素受体拮抗剂和主要作用于小口径小动脉血管重塑的磷酸二酯酶-5抑制剂。尽管这些药物在PH中的有效性证据以及CTEPH中小血管血管病变与其他形式PH的组织学相似性为在CTEPH患者中使用这些药物提供了主要理论依据，但临床试验的证据仍然有限。
