University of Michigan Hospital and Health Systems, Ann Arbor, Michigan.
Feinberg Cardiovascular Research Institute, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
J Am Coll Cardiol. 2015 May 12;65(18):1976-97. doi: 10.1016/j.jacc.2015.03.540.
Pulmonary hypertension (PH) is common and may result from a number of disorders, including left heart disease, lung disease, and chronic thromboembolic disease. Pulmonary arterial hypertension (PAH) is an uncommon disease characterized by progressive remodeling of the distal pulmonary arteries, resulting in elevated pulmonary vascular resistance and, eventually, in right ventricular failure. Over the past decades, knowledge of the basic pathobiology of PAH and its natural history, prognostic indicators, and therapeutic options has exploded. A thorough evaluation of a patient is critical to correctly characterize the PH. Cardiac studies, including echocardiography and right heart catheterization, are key elements in the assessment. Given the multitude of treatment options currently available for PAH, assessment of risk and response to therapy is critical in long-term management. This review also underscores unique situations, including perioperative management, intensive care unit management, and pregnancy, and highlights the importance of collaborative care of the PAH patient through a multidisciplinary approach.
肺动脉高压（PH）较为常见，可能由多种疾病引起，包括左心疾病、肺部疾病和慢性血栓栓塞性疾病。肺动脉高压（PAH）是一种罕见疾病，其特征为远端肺动脉进行性重构，导致肺血管阻力升高，最终导致右心衰竭。在过去几十年中，PAH 的基础病理生物学及其自然病史、预后指标和治疗选择的知识呈爆炸式增长。对患者进行全面评估对于正确描述 PH 至关重要。心脏研究，包括超声心动图和右心导管检查，是评估的关键要素。鉴于目前有多种治疗 PAH 的选择，评估风险和对治疗的反应对于长期管理至关重要。本文还强调了一些特殊情况，包括围手术期管理、重症监护病房管理和妊娠，并强调了通过多学科方法协作管理 PAH 患者的重要性。
