Gleich Stephen, Latham Gregory J, Joffe Denise, Ross Faith J
1 Seattle Children's Hospital, Seattle, WA, USA.
Semin Cardiothorac Vasc Anesth. 2018 Sep;22(3):256-264. doi: 10.1177/1089253217737180. Epub 2017 Oct 19.
Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare right-heart obstructive lesion with a wide anatomic and physiologic spectrum of disease, ranging from simple membranous pulmonary valve atresia with a fully developed right ventricle (RV) to a severely hypoplastic RV and ventriculocoronary (RV-coronary) fistulas. Affected neonates are dependent on prostaglandin for adequate pulmonary blood flow. Depending on the severity of disease, treatment options range from transcatheter pulmonary valve perforation and ultimate biventricular repair to staged single-ventricle palliation. Cardiac transplantation is recommended in the most severe cases. This review will discuss the perioperative and anesthetic management of patients with PA/IVS and highlight the challenges in management.
室间隔完整的肺动脉闭锁（PA/IVS）是一种罕见的右心梗阻性病变，具有广泛的解剖和生理疾病谱，范围从具有完全发育的右心室（RV）的单纯膜性肺动脉瓣闭锁到严重发育不良的右心室和心室冠状动脉（RV-冠状动脉）瘘。受影响的新生儿依赖前列腺素以获得足够的肺血流量。根据疾病的严重程度，治疗选择范围从经导管肺动脉瓣穿孔和最终的双心室修复到分期单心室姑息治疗。在最严重的病例中建议进行心脏移植。本综述将讨论PA/IVS患者的围手术期和麻醉管理，并强调管理中的挑战。
