LaRaia Anne V, Waxman Aaron B
Pulmonary Critical Care Unit and the Critical Care Unit, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
South Med J. 2007 Apr;100(4):393-9. doi: 10.1097/SMJ.0b013e31802f2ff1.
Pulmonary arterial hypertension (PAH), a rare disease involving the pulmonary vascular circuit, is defined as an elevation in pulmonary arterial pressures and is characterized by symptoms of dyspnea, chest pain, and syncope. If left untreated, the disease carries a high mortality rate, with the most common cause of death being decompensated right heart failure. Over the past 5 years, there have been significant advances in this field in regards to understanding the pathogenesis, diagnosis, and classification of PAH. The availability of newer drugs has resulted in a radical change in the management of this disease with significant improvement in both quality of life and mortality. Ongoing research promises to lead to a more comprehensive understanding of the genetics, etiology, and pathogenesis of pulmonary arterial hypertension, which may ultimately translate into more effective therapeutic options.
肺动脉高压（PAH）是一种累及肺血管系统的罕见疾病，定义为肺动脉压力升高，其特征为呼吸困难、胸痛和晕厥等症状。若不治疗，该病死亡率很高，最常见的死亡原因是失代偿性右心衰竭。在过去5年里，该领域在肺动脉高压的发病机制、诊断和分类的认识方面取得了重大进展。新型药物的出现给这种疾病的治疗带来了根本性变化，生活质量和死亡率都有显著改善。正在进行的研究有望带来对肺动脉高压的遗传学、病因和发病机制更全面的认识，这最终可能转化为更有效的治疗选择。
