Malenfant Simon, Neyron Anne-Sophie, Paulin Roxane, Potus François, Meloche Jolyane, Provencher Steeve, Bonnet Sébastien
Pulmonary Hypertension Research Group of the Institut universitaire de cardiologie et de pneumologie de Quebec Research Center, Laval University, Quebec City, Canada.
Pulm Circ. 2013 Apr;3(2):278-93. doi: 10.4103/2045-8932.114752.
Pulmonary arterial hypertension (PAH) is a unique disease. Properly speaking, it is not a disease of the lung. It can be seen more as a microvascular disease occurring mainly in the lungs and affecting the heart. At the cellular level, the PAH paradigm is characterized by inflammation, vascular tone imbalance, pulmonary arterial smooth muscle cell proliferation and resistance to apoptosis and the presence of in situ thrombosis. At a clinical level, the aforementioned abnormal vascular properties alter physically the pulmonary circulation and ventilation, which greatly influence the right ventricle function as it highly correlates with disease severity. Consequently, right heart failure remains the principal cause of death within this cohort of patients. While current treatment modestly improve patients' conditions, none of them are curative and, as of today, new therapies are lacking. However, the future holds potential new therapies that might have positive influence on the quality of life of the patient. This article will first review the clinical presentation of the disease and the different molecular pathways implicated in the pathobiology of PAH. The second part will review tomorrow's future putative therapies for PAH.
肺动脉高压（PAH）是一种独特的疾病。确切地说，它并非肺部疾病。它更可被视为一种主要发生在肺部并影响心脏的微血管疾病。在细胞水平上，PAH的模式特征为炎症、血管张力失衡、肺动脉平滑肌细胞增殖以及对细胞凋亡的抵抗和原位血栓形成。在临床层面，上述异常的血管特性会在物理上改变肺循环和通气，这对右心室功能有很大影响，因为它与疾病严重程度高度相关。因此，右心衰竭仍然是这类患者的主要死因。虽然目前的治疗能适度改善患者状况，但无一具有治愈性，而且截至目前，仍缺乏新的治疗方法。然而，未来可能会有对患者生活质量产生积极影响的潜在新疗法。本文将首先综述该疾病的临床表现以及PAH病理生物学中涉及的不同分子途径。第二部分将综述未来PAH可能的治疗方法。
