278: Congenital left coronary ostial atresia or stenosis - a series of four neonatal fatal cases

Abstract

Congenital left coronary ostial atresia or severe stenosis is an extremely rare coronary abnormality. The clinical picture is either cardiac failure in the small infant or chest pain in the older child or adult patient. This report describes four neonatal cases of this rare abnormality. We retrospectively accessed all cases of left coronary ostial stenosis or atresia seen in our center during an 11-year-period (2000 - 2011). Two older patients with this diagnosis seen at 18 months and 4 years were excluded from the study. Four neonates with the diagnosis of left coronary ostial atresia or stenosis were identified during the study period. One newborn died within minutes, the other within hours after birth because of cardiac failure refractory to all treatment strategies. In both cases left coronary stenosis (one case with a “pinpoint” orifice and the other with a “slitlike” orifice) was diagnosed at autopsy. The third neonate was in cardiac failure due to a severe aortic stenosis. Left coronary ostial atresia was diagnosed during emergency catheter procedure. The infant subsequently died after the aortic dilatation. The forth infant had a cardiac arrest at the third day of life after normal delivery and neonatal adaptation. She was diagnosed with left coronary ostium atresia by coronary angiography performed because of persistent biventricular dysfunction. She died during the attempt of revascularization surgery at 2 weeks of life. Congenital left coronary ostium atresia or stenosis is very rare. Coronary angiography is the diagnostic method of choice especially in the small child or infant. Revascularization surgery seems indicated in symptomatic children based on case reports and smalls series. The clinical picture described here for the first time in the neonate is dramatic and quickly fatal with scarce surgical options. Systematic examination of the coronaries should be part of any neonatal autopsy.