Department of Pulmonary Medicine, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic-University of Barcelona, Barcelona, Spain.
Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain.
Semin Respir Crit Care Med. 2023 Dec;44(6):826-839. doi: 10.1055/s-0043-1770121. Epub 2023 Jul 24.
Pulmonary hypertension (PH) is a common complication of chronic lung diseases, particularly in chronic obstructive pulmonary disease (COPD) and interstitial lung diseases (ILD) and especially in advanced disease. It is associated with greater mortality and worse clinical course. Given the high prevalence of some respiratory disorders and because lung parenchymal abnormalities might be present in other PH groups, the appropriate diagnosis of PH associated with respiratory disease represents a clinical challenge. Patients with chronic lung disease presenting symptoms that exceed those expected by the pulmonary disease should be further evaluated by echocardiography. Confirmatory right heart catheterization is indicated in candidates to surgical treatments, suspected severe PH potentially amenable with targeted therapy, and, in general, in those conditions where the result of the hemodynamic assessment will determine treatment options. The treatment of choice for these patients who are hypoxemic is long-term oxygen therapy and pulmonary rehabilitation to improve symptoms. Lung transplant is the only curative therapy and can be considered in appropriate cases. Conventional vasodilators or drugs approved for pulmonary arterial hypertension (PAH) are not recommended in patients with mild-to-moderate PH because they may impair gas exchange and their lack of efficacy shown in randomized controlled trials. Patients with severe PH (as defined by pulmonary vascular resistance >5 Wood units) should be referred to a center with expertise in PH and lung diseases and ideally included in randomized controlled trials. Targeted PAH therapy might be considered in this subset of patients, with careful monitoring of gas exchange. In patients with ILD, inhaled treprostinil has been shown to improve functional ability and to delay clinical worsening.
肺动脉高压（PH）是慢性肺部疾病的常见并发症，特别是在慢性阻塞性肺疾病（COPD）和间质性肺疾病（ILD）中，尤其是在疾病晚期。它与更高的死亡率和更差的临床病程相关。鉴于一些呼吸道疾病的高患病率，并且由于肺实质异常可能存在于其他 PH 组中，因此正确诊断与呼吸疾病相关的 PH 代表了一项临床挑战。患有慢性肺部疾病且出现超出肺部疾病预期症状的患者应进一步通过超声心动图进行评估。对于接受手术治疗的候选者、疑似严重 PH （可能对靶向治疗有效）以及一般情况下，在那些血流动力学评估结果将决定治疗选择的情况下，应进行有创右心导管检查。对于这些低氧血症患者，选择的治疗方法是长期氧疗和肺康复以改善症状。肺移植是唯一的治愈性治疗方法，在适当的情况下可以考虑。对于轻度至中度 PH 患者，不建议使用常规血管扩张剂或批准用于肺动脉高压（PAH）的药物，因为它们可能会损害气体交换，并且随机对照试验显示其疗效不佳。应将严重 PH（定义为肺血管阻力>5 Wood 单位）的患者转介到具有 PH 和肺部疾病专业知识的中心，并理想地纳入随机对照试验中。对于这部分患者，可以考虑进行靶向 PAH 治疗，并仔细监测气体交换。在 ILD 患者中，吸入曲前列尼尔已被证明可改善功能能力并延迟临床恶化。
