University of Giessen and Marburg Lung Center (UGMLC), member of the German Center for Lung Research, Max-Planck Institute for Heart and Lung Research, Giessen/Bad Nauheim, Germany.
Pulmonary Division, Lady Davis Carmel Medical Center, Faculty of Medicine, The Technion, Institute of Technology, Haifa, Israel.
J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D109-16. doi: 10.1016/j.jacc.2013.10.036.
Chronic obstructive lung disease (COPD) and diffuse parenchymal lung diseases (DPLD), including idiopathic pulmonary fibrosis (IPF) and sarcoidosis, are associated with a high incidence of pulmonary hypertension (PH), which is linked with exercise limitation and a worse prognosis. Patients with combined pulmonary fibrosis and emphysema (CPFE) are particularly prone to the development of PH. Echocardiography and right heart catheterization are the principal modalities for the diagnosis of COPD and DPLD. For discrimination between group 1 PH patients with concomitant respiratory abnormalities and group 3 PH patients (PH caused by lung disease), patients should be transferred to a center with expertise in both PH and lung diseases for comprehensive evaluation. The task force encompassing the authors of this article provided criteria for this discrimination and suggested using the following definitions for group 3 patients, as exemplified for COPD, IPF, and CPFE: COPD/IPF/CPFE without PH (mean pulmonary artery pressure [mPAP] <25 mm Hg); COPD/IPF/CPFE with PH (mPAP ≥25 mm Hg); PH-COPD, PH-IPF, and PH-CPFE); COPD/IPF/CPFE with severe PH (mPAP ≥35 mm Hg or mPAP ≥25 mm Hg with low cardiac index [CI <2.0 l/min/m(2)]; severe PH-COPD, severe PH-IPF, and severe PH-CPFE). The "severe PH group" includes only a minority of chronic lung disease patients who are suspected of having strong general vascular abnormalities (remodeling) accompanying the parenchymal disease and with evidence of an exhausted circulatory reserve rather than an exhausted ventilatory reserve underlying the limitation of exercise capacity. Exertional dyspnea disproportionate to pulmonary function tests, low carbon monoxide diffusion capacity, and rapid decline of arterial oxygenation upon exercise are typical clinical features of this subgroup with poor prognosis. Studies evaluating the effect of pulmonary arterial hypertension drugs currently not approved for group 3 PH patients should focus on this severe PH group, and for the time being, these patients should be transferred to expert centers for individualized patient care.
慢性阻塞性肺疾病（COPD）和弥漫性实质性肺疾病（DPLD），包括特发性肺纤维化（IPF）和结节病，与肺动脉高压（PH）的发生率高相关，后者与运动受限和预后较差有关。合并肺纤维化和肺气肿（CPFE）的患者尤其容易发生 PH。超声心动图和右心导管检查是诊断 COPD 和 DPLD 的主要方式。为了区分伴有呼吸异常的 1 型 PH 患者和 3 型 PH 患者（由肺部疾病引起的 PH），应将患者转至同时具备 PH 和肺部疾病专业知识的中心进行全面评估。本文作者组成的专家组提供了这种区分的标准，并建议对 3 型患者使用以下定义，以 COPD、IPF 和 CPFE 为例：无 PH 的 COPD/IPF/CPFE（平均肺动脉压[mPAP]＜25mmHg）；有 PH 的 COPD/IPF/CPFE（mPAP≥25mmHg）；PH-COPD、PH-IPF 和 PH-CPFE）；COPD/IPF/CPFE 合并严重 PH（mPAP≥35mmHg 或 mPAP≥25mmHg 伴低心指数[CI＜2.0 l/min/m2]；严重 PH-COPD、严重 PH-IPF 和严重 PH-CPFE）。“严重 PH 组”仅包括少数慢性肺部疾病患者，这些患者疑似伴有实质性疾病的全身性血管异常（重塑）和循环储备衰竭的证据，而不是运动能力受限的通气储备衰竭。与肺功能测试不成比例的运动性呼吸困难、一氧化碳扩散能力低和运动时动脉氧合迅速下降是这种预后不良的亚组的典型临床特征。评估目前未批准用于 3 型 PH 患者的肺动脉高压药物的效果的研究应集中在这个严重 PH 组，目前这些患者应转至专家中心进行个体化的患者护理。
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