Section of Pulmonary, Critical Care and Sleep Medicine, Yale School of Medicine, 300 Cedar Street, PO Box 208057, New Haven, CT 06520-8057, USA.
Section of Pulmonary, Critical Care and Sleep Medicine, Yale School of Medicine, 300 Cedar Street, PO Box 208057, New Haven, CT 06520-8057, USA.
Clin Chest Med. 2018 Sep;39(3):595-603. doi: 10.1016/j.ccm.2018.04.005.
Pulmonary hypertension (PH) is divided into varied pathophysiological and etiologic groupings, as classified by the World Health Organization (WHO). Pulmonary arterial hypertension (PAH), which falls under WHO group 1 PH, is a progressive and potentially fatal disease characterized by a vasoconstrictive, proliferative, and thrombotic phenotype, which leads to increased pulmonary artery pressure, right heart failure, and death. Pathologically, in situ thromboses are found in the small distal pulmonary arteries. Dysregulation of coagulation, platelet function, and endothelial cells may contribute to a prothrombotic state. There is mixed evidence for the use of anticoagulation or antiplatelet therapy in PAH patients.
肺动脉高压(PH)被世界卫生组织(WHO)分为多种病理生理学和病因学分组。肺动脉高压(PAH)属于 WHO 第 1 组 PH,是一种进行性和潜在致命的疾病,其特征为血管收缩、增殖和血栓形成表型,导致肺动脉压升高、右心衰竭和死亡。病理学上,在小的远端肺动脉中发现原位血栓形成。凝血、血小板功能和内皮细胞的失调可能导致血栓形成状态。在 PAH 患者中使用抗凝或抗血小板治疗的证据混杂不一。