Chikkabyrappa Sathish M, Loomba Rohit S, Tretter Justin T
1 Seattle Childrens Hospital, University of Washington, Seattle, WA, USA.
2 Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Semin Cardiothorac Vasc Anesth. 2018 Sep;22(3):245-255. doi: 10.1177/1089253218756757. Epub 2018 Feb 7.
Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare complex cyanotic congenital heart disease with heterogeneous morphological variation. Prenatal diagnosis allows for developing a safe plan for delivery and postnatal management. While transthoracic echocardiography allows for detailed delineation of the cardiac anatomy, additional imaging modalities such as computed tomography, magnetic resonance imaging, and catheterization may be necessary to further outline features of the cardiac anatomy, specifically coronary artery anatomy. The size of the tricuspid valve and right ventricular cavity as well as the presence of right ventricle-dependent coronary circulation help to dichotomize between biventricular repair versus univentricular palliation or heart transplantation, as well as predicting the expected survival. The delineation and understanding of these features help to dictate both medical and surgical management.
室间隔完整的肺动脉闭锁（PA-IVS）是一种罕见的复杂性青紫型先天性心脏病，具有形态学异质性变异。产前诊断有助于制定安全的分娩计划和产后管理方案。虽然经胸超声心动图能够详细描绘心脏解剖结构，但可能需要其他成像方式，如计算机断层扫描、磁共振成像和心导管检查，以进一步勾勒心脏解剖特征，特别是冠状动脉解剖结构。三尖瓣和右心室腔的大小以及右心室依赖型冠状动脉循环的存在有助于区分双心室修复与单心室姑息治疗或心脏移植，以及预测预期生存率。对这些特征的描绘和理解有助于指导药物治疗和手术管理。
