Lloyd Kenneth Scott
Methodist DeBakey Heart & Vascular Center, Houston, Texas, USA.
Methodist Debakey Cardiovasc J. 2009;5(2):20-3. doi: 10.14797/mdcj-5-2-20.
Pulmonary vascular hypertension in general is a progressive, nearly always fatal condition that until recently has had very few treatment options. Our understanding of the pulmonary vascular disease process has opened the window to earlier screening techniques, diagnosis, and treatment options. However, all current treatment options are complex and expensive and therefore require clinical support strategies often necessitating specialized pulmonary hypertension treatment centers. Whether idiopathic or secondary, pulmonary arterial hypertension is characterized by the deregulated proliferation of pulmonary artery endothelial cells and intimal smooth muscle cells, both resistant to cellular apoptosis. Early recognition of such disregulation may lead to earlier diagnosis and treatment and thus alteration in the disease process. Screening of high-risk populations such as those with connective tissue disorders, HIV disease, congenital heart disease, portal hypertension, and those exposed to certain drugs and toxins such as methamphetamines and the diet drugs Dexfenfluramine and Fenfluramine is of utmost importance. Similarly, early symptom recognition in these high-risk groups is essential to earlier diagnosis and treatment.
一般而言，肺动脉高压是一种进行性疾病，几乎总是致命的，直到最近其治疗选择都非常有限。我们对肺血管疾病进程的理解为早期筛查技术、诊断和治疗选择打开了一扇窗。然而，目前所有的治疗选择都很复杂且昂贵，因此需要临床支持策略，这往往需要专门的肺动脉高压治疗中心。无论是特发性还是继发性肺动脉高压，其特征都是肺动脉内皮细胞和内膜平滑肌细胞增殖失控，且两者都对细胞凋亡有抗性。尽早识别这种失调可能会导致更早的诊断和治疗，从而改变疾病进程。对高危人群进行筛查至关重要，比如患有结缔组织病、艾滋病、先天性心脏病、门静脉高压的人群，以及接触过某些药物和毒素（如甲基苯丙胺、减肥药右芬氟拉明和芬氟拉明）的人群。同样，在这些高危人群中尽早识别症状对于早期诊断和治疗至关重要。
