Betkier-Lipińska Katarzyna, Ryczek Robert, Cwetsch Andrzej
Military Institute of Medicine in Warsaw, Poland, Central Clinical Hospital of the Ministry of National Defense, Department of Cardiology and Internal Medicine.
Pol Merkur Lekarski. 2015 Jan;38(223):34-8.
Pulmonary arterial hypertension (PAH) is a chronic, serious disease caused by remodeling of small pulmonary vessels, which leads to increase of pulmonary resistance, right heart failure and death. The 1990ths of XX century are the beginning of dynamic research into the pathophysiology and treatment of this disease. Actually, the goal oriented therapy based on three main metabolic pathways includes: prostacyclin's analogues, endothelin receptor antagonists and phosphodiesterase-5 inhibitors. Applying this therapy, according to ESC guidelines has prolonged significantly the survival in the group of patients with PAH. Due to the high cost of the therapy, there has been created the national therapeutic program in Poland. It assures the real possibility of therapy for patients with PAH from the third FC WHO.
肺动脉高压（PAH）是一种由肺小血管重塑引起的慢性严重疾病，可导致肺阻力增加、右心衰竭和死亡。20世纪90年代是对该疾病病理生理学和治疗进行动态研究的开端。实际上，基于三种主要代谢途径的靶向治疗包括：前列环素类似物、内皮素受体拮抗剂和磷酸二酯酶-5抑制剂。根据欧洲心脏病学会（ESC）指南应用这种治疗方法，显著延长了PAH患者群体的生存期。由于治疗费用高昂，波兰制定了国家治疗计划。这确保了世界卫生组织功能分级（FC）为三级的PAH患者真正有可能接受治疗。
