Left ventricular assist device improves survival in children with left ventricular dysfunction after repair of anomalous origin of the left coronary artery from the pulmonary artery

Background. Repair of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in infants carries a high operative risk, partic ularly in infants with myocardial infarction and poor left ventricular func tion. The marked recovery of left ventricular function reported late after repair, however, suggests that an aggressive approach to repair should be u ndertaken Methods. Of 31 children undergoing primary repair of ALCAPA at our institut ion from 1987 to 1996, 26 were infants (6 weeks to 9 months old). All but 2 had severe left ventricular dysfunction, and 8 had moderate to severe mitr al regurgitation. Seven children were unable to be weaned from cardiopulmon ary bypass because of poor left ventricular function and elevated left atri al pressure. These 7 children were placed on mechanical left ventricular su pport using a centrifugal pump, with support ranging from 2.2 to 70.6 hours . Results. One child died shortly after the start of left ventricular assist (2.2 hours), and another died of arrhythmia within 24 hours after successfu l decannulation. All 5 survivors had significant improvement in left ventri cular function, with 2 requiring late mitral valve repair. Conclusions. Infants with ALCAPA who have severe left ventricular dysfuncti on represent a higher risk group for repair. However, with use of mechanica l circulatory support in those unable to be weaned from cardiopulmonary byp ass, a high survival rate can be achieved with good long-term recovery. We conclude that an aggressive approach to early repair in all children with A LCAPA is warranted, regardless of the degree of left ventricular dysfunctio n. (C) 1999 by The Society of Thoracic Surgeons.