Prasad Jyotika D, Williams Trevor J, Whitford Helen M
Department of Respiratory Medicine, Alfred Hospital, Melbourne, Victoria, Australia.
Department of Respiratory Medicine, Royal Melbourne Hospital, Melbourne, Victoria, Australia.
Intern Med J. 2024 Dec;54(12):1944-1951. doi: 10.1111/imj.16515. Epub 2024 Oct 23.
Pulmonary arterial hypertension (PAH) is a rare condition for which a remarkable change has been witnessed in the epidemiology, assessment and treatment landscape over the last three decades. Well-established registries from the Western world have not only highlighted the shift in the epidemiology to an older, more comorbid cohort but have also identified markers of prognosis that have been validated as part of risk stratification scores in multiple cohorts. The emphasis on early identification through a systematic assessment pathway and the option of upfront combination therapy with serial risk stratification assessment has laid the foundation for the standard of care and improved prognosis. This review provides an update on the assessment and newer therapies for PAH.
肺动脉高压（PAH）是一种罕见疾病，在过去三十年里，其流行病学、评估和治疗领域都发生了显著变化。西方世界完善的登记系统不仅突出了流行病学向年龄更大、合并症更多的人群的转变，还确定了预后标志物，这些标志物已在多个队列中作为风险分层评分的一部分得到验证。通过系统评估途径强调早期识别以及采用序贯风险分层评估的初始联合治疗方案，为护理标准和改善预后奠定了基础。本综述提供了PAH评估和新疗法的最新情况。
