最常與肺高壓相關的肺部疾病，包括慢性阻塞性肺病，特發性肺纖維化及合併肺纖維化與肺氣腫。 肺部疾病如果併生肺高壓，常導致運動耐受力變差，低血氧及存活率降低。肺部疾病相關之肺高壓嚴重 程度，往往與肺部疾病之嚴重度沒有太大的相關性。肺部疾病產生肺高壓，常導致一氧化氮擴散指數 (DLco)與動脈血二氧化碳分壓(PaCO2)不成比例地下降。在治療方面，長期氧氣治療曾被發現可減緩慢性 阻塞性肺病患者肺高壓之進展。在這些病患並不被建議使用傳統血管擴張劑(如鈣離子阻斷劑)。目前肺動 脈高壓的標靶治療藥物臨床試驗的結果，尚未能證實在這些病患有顯著療效。慢性血栓性肺動脈高壓(簡 稱 CTEPH)是由大血管栓塞引起的阻塞性肺動脈重塑疾病。在發生有症狀的肺栓塞事件後兩年內，CTEPH 的累計發生率為 0.1%-9.1%。診斷標準包含肺部通氣/灌注(V/Q)掃描顯示，至少有一個肺小節有不匹配 (mismatched)灌注缺陷的現象，以及經由電腦斷層血管造影、磁振造影或傳統肺部血管攝影檢查，顯示有 CTEPH 特定肺動脈血管病徵。肺動脈內膜切除手術 (PEA) 是 CTEPH 的治療選擇，絕大多數的病患術 後症狀有明顯改善。病患應終生使用抗凝劑。Riociguat 是目前唯一被核准治療CTEPH 的肺動脈高壓藥 物。介入性肺動脈氣球擴張術(BPA)是發展中的技術，但必須在有經驗的中心才能執行。

The most common lung diseases associated with PH are COPD, interstitial lung disease and combined pulmonary fibrosis and emphysema (CPFE). In any lung disease, the development of PH is accompanied by a deterioration of exercise capacity, worsening of hypoxemia and shorter survival. The severity of PH is usually poorly associated with the severity of the underlying lung disease. The most common indicators for the presence of PH in these patients are a disproportionally low DLCO and a low PaCO2. Currently there is no specific therapy for PH associated with lung diseases. Long-term O2 administration has been shown to partially reduce the progression of PH in COPD. Published experience with targeted PAH drug therapy is scarce, and so far there is no evidence from RCTs suggesting that PAH drugs result in improved symptoms or outcomes in patients with lung disease. Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease of obstructive pulmonary arterial remodelling as a consequence of major vessel thromboembolism. CTEPH has been reported with a cumulative incidence of 0.1–9.1% within the first 2 years after a symptomatic pulmonary embolism event. For the diagnosis of CTEPH, patients must meet the hemodynamic criteria of PAH, with mismatched perfusion defects on lung scans. Specific diagnostic may be seen by CT angiography, MR imaging or selective pulmonary angiography. Pulmonary endarterectomy (PEA) is the treatment of choice. The majority of patients experience substantial relief from symptoms after PEA. Lifelong anticoagulation is recommended. Medical treatment with targeted therapy may be justified in non-operable patients. The only approved PAH specific drug for CTEPH is riociguat. Balloon pulmonary angioplasty (BPA) is rapidly gaining attention worldwide. It should only be performed in experienced and high-volume CTEPH centers.