Division of Pulmonary and Critical Care Medicine, Stanford School of Medicine, Stanford, California, USA.
Division of Cardiology, Stanford School of Medicine, Stanford, California, USA.
Pulm Circ. 2014 Mar;4(1):10-24. doi: 10.1086/674885.
Pulmonary hypertensive crisis is an important cause of morbidity and mortality in patients with pulmonary arterial hypertension secondary to congenital heart disease (PAH-CHD) who require cardiac surgery. At present, prevention and management of perioperative pulmonary hypertensive crisis is aimed at optimizing cardiopulmonary interactions by targeting prostacyclin, endothelin, and nitric oxide signaling pathways within the pulmonary circulation with various pharmacological agents. This review is aimed at familiarizing the practitioner with the current pharmacological treatment for dealing with perioperative pulmonary hypertensive crisis in PAH-CHD patients. Given the life-threatening complications associated with pulmonary hypertensive crisis, proper perioperative planning can help anticipate cardiopulmonary complications and optimize surgical outcomes in this patient population.
肺动脉高压危象是先天性心脏病（先心病）相关肺动脉高压（PAH-CHD）患者行心脏手术的重要致死致残原因。目前，针对肺血管中环前列腺素、内皮素和一氧化氮信号通路，应用各种药理学药物来优化心肺相互作用，以此预防和管理围手术期肺动脉高压危象。本综述旨在使临床医生熟悉目前针对 PAH-CHD 患者围手术期肺动脉高压危象的药物治疗。鉴于肺动脉高压危象相关的致命性并发症，适当的围手术期计划有助于预测此类患者人群的心肺并发症，并优化手术结局。
