Hoeper M M, Ghofrani H A, Grimminger F, Rosenkranz S
Abteilung Pneumologie, Medizinische Hochschule Hannover.
Dtsch Med Wochenschr. 2008 Oct;133 Suppl 6:S191-5. doi: 10.1055/s-0028-1091236. Epub 2008 Sep 23.
The treatment algorithm that has recently been developed at the 4th World Conference on Pulmonary Hypertension (PH) in Dana Point will contain a number of important innovations for patients with pulmonary arterial hypertension (PAH), but also for those with other forms of PH. In PAH patients, a targeted therapy with ERA or PDE5 inhibitors is now recommended for patients in functional class II. Combination therapy (ERA and/or PDE5i and/or prostanoids) is proposed if the clinical response to monotherapy is not adequate. In addition, supervised training programs are recommended for patients with PAH. For other forms of PH such as PH due to left heart disease or chronic lung disease, it remains valid that the underlying disease should be treated as efficiently as possible. However, a targeted PAH therapy may be beneficial in selected patients with "out of proportion PH", but these treatments should exclusively be initiated in expert centers. Pulmonary endarterectomy (PEA) remains the treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH). If patients are inoperable and/or if surgical treatment is not an option, targeted PAH therapy may be considered, but these patients should at present be included into clinical trials. Since there is currently no cure for PH/PAH, further development and progress in medical treatment are highly desirable. A number of promising novel compounds are currently under investigation. These include sGC stimulators, tyrosine kinase inhibitors, and serotonin antagonists.
最近在达纳角举行的第四届世界肺动脉高压会议上制定的治疗算法,将包含一些针对肺动脉高压(PAH)患者的重要创新,也适用于其他形式肺动脉高压患者。对于PAH患者,目前推荐对功能分级为II级的患者使用ERA或PDE5抑制剂进行靶向治疗。如果单药治疗的临床反应不足,则建议联合治疗(ERA和/或PDE5i和/或前列腺素)。此外,建议为PAH患者提供监督训练计划。对于其他形式的肺动脉高压,如左心疾病或慢性肺部疾病所致的肺动脉高压,尽可能有效地治疗基础疾病仍然有效。然而,靶向PAH治疗可能对部分“肺动脉高压程度与基础疾病不相称”的患者有益,但这些治疗应仅在专家中心启动。肺动脉内膜切除术(PEA)仍然是慢性血栓栓塞性肺动脉高压(CTEPH)的首选治疗方法。如果患者无法手术和/或手术治疗不是一种选择,可以考虑靶向PAH治疗,但目前这些患者应纳入临床试验。由于目前尚无治愈肺动脉高压/PAH的方法,因此非常需要在医学治疗方面取得进一步的发展和进步。目前有一些有前景的新型化合物正在研究中。这些包括可溶性鸟苷酸环化酶刺激剂、酪氨酸激酶抑制剂和5-羟色胺拮抗剂。