Evaluation of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients

Objectives We sought to prospectively evaluate evidence of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery with an interarterial course (AAOCA). Background An AAOCA is a rare anomaly associated with increased myocardial ischemia and sudden death risk in children. Data evaluating ischemia after AAOCA repair are limited. Methods We included children who underwent AAOCA surgery between October 2001 and December 2006. They were prospectively assessed with exercise stress test (EST), stress echocardiogram (SE), and stress myocardial perfusion scan (MPS). Results Of 24 participating children, 15 (63%) were male, 16 (67%) had anomalous right coronary (ARCA), and 7 (29%) were asymptomatic. Median age was 12 (5 to 18) years; follow-up was 15 (2 to 48) months. All had unobstructed neo-coronary ostia by echocardiogram and were asymptomatic. One anomalous origin of left main coronary artery (ALCA) patient and 8 ARCA patients had post-operative evaluations suggestive of ischemia. The ALCA patient had reversible apical septal and mid-anteroseptal hypokinesis on SE. Of the ARCA patients, 2 had inferior ST-segment depression on EST; subsequently, I had normal tests, but the other developed anterolateral Q waves. Two patients had blunted blood pressure response with EST, 1 had fixed apical inferior hypokinesis on SE, 2 had reversible perfusion defects on MPS, and I had a fixed perfusion defect on MPS. Conclusions Subclinical changes suggestive of ischemia might occur despite patent neo-coronary ostia, notably after ARCA repair. The implication of these results on indication for surgery and subsequent sudden death risk is unknown. Serial EST, SE, and MPS are essential in evaluating ongoing ischemia risk after AAOCA repair.