Ghigna Maria Rosa, Mooi Wolter J, Grünberg Katrien
Service d'Anatomie et de Cytologie Pathologiques, Hôpital Marie Lannelongue, Le Plessis Robinson, France
Dept of Pathology, VU University Medical Center, Amsterdam, The Netherlands.
Eur Respir Rev. 2017 Jun 28;26(144). doi: 10.1183/16000617.0003-2017. Print 2017 Jun 30.
Pulmonary hypertension (PH) with complicating chronic lung diseases and/or hypoxia falls into group 3 of the updated classification of PH. Patients with chronic obstructive lung disease (COPD), diffuse lung disease (such as idiopathic pulmonary fibrosis (IPF)) and with sleep disordered breathing are particularly exposed to the risk of developing PH. Although PH in such a context is usually mild, a minority of patients exhibit severe haemodynamic impairment, defined by a mean pulmonary arterial pressure (mPAP) of ≥35 mmHg or mPAP values ranging between 25 mmHg and 35 mmHg with a low cardiac index (<2 L·min·m). The overlap between lung parenchymal disease and PH heavily affects life expectancy in such a patient population and complicates their therapeutic management. In this review we illustrate the pathological features and the underlying pathophysiological mechanisms of pulmonary circulation in chronic lung diseases, with an emphasis on COPD, IPF and obstructive sleep apnoea syndrome.
合并慢性肺部疾病和/或缺氧的肺动脉高压（PH）属于PH更新分类中的第3组。慢性阻塞性肺疾病（COPD）、弥漫性肺疾病（如特发性肺纤维化（IPF））以及睡眠呼吸障碍患者尤其容易发生PH。尽管在这种情况下的PH通常较轻，但少数患者表现出严重的血流动力学损害，定义为平均肺动脉压（mPAP）≥35 mmHg或mPAP值在25 mmHg至35 mmHg之间且心脏指数较低（<2 L·min·m）。肺实质疾病与PH之间的重叠严重影响了这类患者群体的预期寿命，并使其治疗管理变得复杂。在本综述中，我们阐述了慢性肺部疾病中肺循环的病理特征和潜在的病理生理机制，重点关注COPD、IPF和阻塞性睡眠呼吸暂停综合征。
