Complete Atresia of Coronary Ostia in Pulmonary Atresia and Intact Ventricular Septum
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.
KeywordsAtresia of Coronary Ostia Pulmonary Atresia Ischemia Myocardial Infarction
We thank Drs. Donald Leichter from the Division of Pediatric Cardiology and Melissa Gill from the Department of Pathology for their contributions.
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