Division of Cardiovascular Medicine, Brigham and Women's Hospital and Harvard Medical School, Harvard University, Boston, Massachusetts.
Section of Pulmonary Medicine, Children's Hospital Colorado and the University of Colorado Anschutz Medical Center, University of Colorado, Aurora, Colorado.
Am J Respir Crit Care Med. 2021 Jun 15;203(12):1472-1487. doi: 10.1164/rccm.202012-4317SO.
The diagnosis and management of pulmonary arterial hypertension (PAH) includes several advances, such as a broader recognition of extrapulmonary vascular organ system involvement, validated point-of-care clinical assessment tools, and focus on the early initiation of multiple pharmacotherapeutics in appropriate patients. Indeed, a principal goal in PAH today is an early diagnosis for prompt initiation of treatment to achieve a minimal symptom burden; optimize the patient's biochemical, hemodynamic, and functional profile; and limit adverse events. To accomplish this end, clinicians must be familiar with novel risk factors and the revised hemodynamic definition for PAH. Fresh insights into the role of developmental biology (i.e., perinatal health) may also be useful for predicting incident PAH in early adulthood. Emergent or underused approaches to PAH management include a novel TGF-β ligand trap pharmacotherapy, remote pulmonary arterial pressure monitoring, next-generation imaging using inert gas-based magnetic resonance and other technologies, right atrial pacing, and pulmonary arterial denervation. These and other PAH state of the art advances are summarized here for the wider pulmonary medicine community.
肺动脉高压（PAH）的诊断和管理包括多个方面的进展，例如对外周肺血管器官系统受累的更广泛认识、经过验证的即时临床评估工具，以及关注在适当的患者中尽早启动多种药物治疗。事实上，PAH 目前的主要目标是早期诊断，以便及时开始治疗，以实现最小的症状负担；优化患者的生化、血液动力学和功能状况；并限制不良事件。为此，临床医生必须熟悉新的危险因素和 PAH 的修订血液动力学定义。发育生物学（即围产期健康）的新见解也可能有助于预测成年早期发生 PAH。PAH 管理的新兴或未充分利用的方法包括新型 TGF-β配体陷阱药物治疗、远程肺动脉压力监测、使用惰性气体基于磁共振和其他技术的下一代成像、右心房起搏和肺动脉去神经支配。这里为更广泛的肺病学社区总结了这些和其他 PAH 最新进展。
