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.
Selamet
S E
SE
Department of Pediatrics, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA. ss1807@columbia.edu
Hsu
D T
DT
Thaker
H M
HM
Gersony
W M
WM
D003330:Coronary Vessel Anomalies / Q000601:surgery*; D017809:Fatal Outcome; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000601:surgery*; D006801:Humans; D007231:Infant, Newborn; D009203:Myocardial Infarction / Q000209:etiology; D011183:Postoperative Complications; D018633:Pulmonary Atresia / Q000601:surgery*