Friedman Kevin
1 Boston Children's Hospital, Boston, MA, USA.
2 Harvard University, Boston, MA, USA.
Semin Cardiothorac Vasc Anesth. 2018 Sep;22(3):265-269. doi: 10.1177/1089253218770198. Epub 2018 Apr 12.
Interrupted aortic arch (IAA) is a rare form of critical neonatal heart disease in which there is lack of continuity between the ascending aorta and the descending thoracic aorta. In the absence of prenatal diagnosis, patients with IAA present in shock when the patent ductus arteriosus closes. Diagnosis can generally be made by echocardiography, and initiation of prostaglandin E1 infusion allows for adequate lower body perfusion prior to surgical repair. Full neonatal repair can be achieved with good outcomes in most cases. However, there is often underdevelopment of the left ventricular outflow tract and risk for later surgical re-intervention. Many patients with IAA, particularly those with type B, have DiGeorge syndrome, which has important development implications.
主动脉弓中断（IAA）是一种罕见的严重新生儿心脏病，其升主动脉和胸降主动脉之间缺乏连续性。在未进行产前诊断的情况下，当动脉导管闭合时，患有IAA的患者会出现休克。一般通过超声心动图进行诊断，在手术修复前开始输注前列腺素E1可使下半身获得足够的灌注。大多数情况下，可实现完全的新生儿修复，且效果良好。然而，左心室流出道通常发育不全，后期有手术再次干预的风险。许多患有IAA的患者，尤其是B型患者，患有DiGeorge综合征，这具有重要的发育意义。
