Selamet S E, Hsu D T, Thaker H M, Gersony W M
Department of Pediatrics, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA.
Pediatr Cardiol. 2004 Jan-Feb;25(1):67-9. doi: 10.1007/s00246-003-0517-0. Epub 2003 Sep 4.
We describe the clinical course, echocardiography, angiography, and histopathology of a female infant with pulmonary atresia and intact ventricular septum (PA/IVS) with complete coronary ostial atresia and right ventricle-dependent coronary circulation who survived for 7 weeks after palliative surgery. The patient expired from myocardial insufficiency while waiting for a donor heart. Postmortem examination demonstrated atretic coronary ostia, ventricular sinusoids, and myocardial infarctions of various ages. This report suggests that neonates with PA/IVS who have this extreme form of coronary abnormality may potentially be managed medically and surgically until cardiac transplantation is available.
我们描述了一名患有肺动脉闭锁合并完整室间隔（PA/IVS）、完全性冠状动脉开口闭锁及右心室依赖性冠状动脉循环的女婴的临床病程、超声心动图、血管造影及组织病理学情况，该患儿在姑息性手术后存活了7周。患者在等待供体心脏时死于心肌功能不全。尸检显示冠状动脉开口闭锁、心室窦状隙及不同时期的心肌梗死。本报告提示，患有这种极端形式冠状动脉异常的PA/IVS新生儿在心脏移植可用之前，可能可通过药物和手术进行治疗。
