Department of Clinical Pharmacology, Aarhus University Hospital, Denmark.
Int J Cardiol. 2013 Oct 3;168(3):1795-804. doi: 10.1016/j.ijcard.2013.06.033. Epub 2013 Jul 11.
The purpose of the present review is to summarize the current knowledge on PH in relation to COPD and ILD from a clinical perspective with emphasis on diagnosis, biomarkers, prevalence, impact, treatment, and practical implications. PH in COPD and ILD is associated with a poor prognosis, and is considered one of the most frequent types of PH. However, the prevalence of PH among patients with COPD and ILD is not clear. The diagnosis of PH in chronic lung disease is often established by echocardiographic screening, but definitive diagnosis requires right heart catheterization, which is not systematically performed in clinical practice. Given the large number of patients with chronic lung disease, biomarkers to preclude or increase suspicion of PH are needed. NT-proBNP may be used as a rule-out test, but biomarkers with a high specificity for PH are still required. It is not known whether specific treatment with existent drugs effective in pulmonary arterial hypertension (PAH) is beneficial in lung disease related PH. Studies investigating existing PAH drugs in animal models of lung disease related PH have indicated a positive effect, and so have case reports and open label studies. However, treatment with systemically administered pulmonary vasodilators implies the risk of worsening the ventilation-perfusion mismatch in patients with lung disease. Inhaled vasodilators may be better suited for PH in lung disease, but new treatment modalities are also required.
本文旨在从临床角度总结目前关于 COPD 和ILD 相关 PH 的知识，重点介绍诊断、生物标志物、患病率、影响、治疗和实际意义。COPD 和 ILD 中的 PH 与预后不良相关，被认为是最常见的 PH 类型之一。然而，COPD 和 ILD 患者中 PH 的患病率尚不清楚。慢性肺部疾病中 PH 的诊断通常通过超声心动图筛查确定，但明确诊断需要进行右心导管检查，但在临床实践中并未系统进行。鉴于患有慢性肺部疾病的患者数量众多，需要生物标志物来排除或增加对 PH 的怀疑。NT-proBNP 可作为排除测试，但仍需要具有高特异性的 PH 生物标志物。目前尚不清楚在与肺部疾病相关的 PH 中使用对肺动脉高压（PAH）有效的现有药物进行特定治疗是否有益。研究表明，在与肺部疾病相关的 PH 的动物模型中，现有的 PAH 药物具有积极作用，病例报告和开放标签研究也表明了这一点。然而，全身给予肺血管扩张剂治疗可能会增加肺部疾病患者通气-灌注不匹配的风险。吸入性血管扩张剂可能更适合肺部疾病中的 PH，但也需要新的治疗方法。
