Ferns Sunita Juliana, Wehrmacher William H, Serratto Maria
Department of Pediatrics, University of Illinois at Chicago, 840 St, Wood St, Chicago IL 60612, USA.
Compr Ther. 2009 Summer;35(2):81-90.
Pulmonary arterial hypertension (PAH) afflicts thousands of children worldwide. The pathophysiology involves intravascular proliferation and remodeling leading to an increase in pulmonary vascular resistance which if left untreated results in right heart failure and death. Signs and symptoms are subtle as the disease progresses to irreversible lung damage. There is no cure for PAH, however newer methods of treatment can successfully manage these patients and delay progression of the disease process.
肺动脉高压（PAH）困扰着全球数千名儿童。其病理生理学涉及血管内增殖和重塑，导致肺血管阻力增加，若不治疗，会导致右心衰竭和死亡。随着疾病进展至不可逆转的肺损伤，体征和症状较为隐匿。PAH无法治愈，然而，更新的治疗方法能够成功治疗这些患者并延缓疾病进程。
