Cardiac Surgery Department, Beijing Anzhen Hospital, Capital Medical University, Beijing Institute of Heart, Lung, and Blood Vessel Disease, Beijing 100029, China.
Cardiovasc Ther. 2011 Jun;29(3):153-75. doi: 10.1111/j.1755-5922.2009.00095.x. Epub 2010 Jun 18.
Acute pulmonary arterial hypertension (PAH), which may complicate the course of many complex disorders, is always underdiagnosed and its treatment frequently begins only after serious complications have developed. Acute PAH is distinctive because they differ in their clinical presentation, diagnostic findings, and response to treatment from chronic PAH. The acute PAH may take either the form of acute onset of chronic PAH or acute PAH or surgery-related PAH. Significant pathophysiologic differences existed between acute and chronic PAH. Therapy of acute PAH should generally be aimed at acutely relieving right ventricular (RV) pressure overload and preventing RV dysfunction. There are three classes of drugs targeting the correction of abnormalities in endothelial dysfunction, which have been approved recently for the treatment of PAH: (1) prostanoids; (2) endothelin receptor antagonists; and (3) phosphodiesterase-5 inhibitors. The efficacy and safety of these compounds have been confirmed in uncontrolled studies in patients with PAH. Intravenous epoprostenol is suggested to serve as the first-line treatment for the most severe patients. In the other situations, the first-line therapy may include bosentan, sildenafil, or a prostacyclin analogue. Recent advances in the management of PAH have markedly improved prognosis.
急性肺动脉高压（PAH）可能使许多复杂疾病的病程复杂化，但往往诊断不足，其治疗通常在出现严重并发症后才开始。急性 PAH 有其独特的特点，因为它们在临床表现、诊断发现和对治疗的反应上与慢性 PAH 不同。急性 PAH 可能表现为慢性 PAH 的急性发作，也可能是急性 PAH 或与手术相关的 PAH。急性和慢性 PAH 之间存在显著的病理生理差异。急性 PAH 的治疗一般应旨在急性缓解右心室（RV）压力超负荷和预防 RV 功能障碍。目前已有三种针对内皮功能障碍异常的药物获批用于治疗 PAH：（1）前列腺素；（2）内皮素受体拮抗剂；和（3）磷酸二酯酶-5 抑制剂。这些化合物在未经控制的 PAH 患者研究中已证实其疗效和安全性。静脉内依前列醇被建议作为最严重患者的一线治疗药物。在其他情况下，一线治疗可能包括波生坦、西地那非或前列环素类似物。PAH 管理方面的最新进展显著改善了预后。
