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CLINICAL RESEARCH: SURGERY FOR CONGENITAL DISEASE

Evaluation of Myocardial Ischemia After Surgical Repair of Anomalous Aortic Origin of a Coronary Artery in a Series of Pediatric Patients

Julie A. Brothers, MD^_*,_*, Michael G. McBride, PhD^_*, Mohamed A. Seliem, MD, FACC^_*, Bradley S. Marino, MD, MPP, MSCE, FACC, Ryan S. Tomlinson, BSE, Miguel H. Pampaloni, MD, PhD, J. William Gaynor, MD, Thomas L. Spray, MD, FACC and Stephen M. Paridon, MD, FACC^_*

^_* Cardiology, The Children's Hospital of Philadelphia
Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
Department of Anesthesiology and Critical Care, University of Pennsylvania, Philadelphia, Pennsylvania
Department of Radiology, University of Pennsylvania, Philadelphia, Pennsylvania

Manuscript received April 4, 2007; revised manuscript received May 3, 2007, accepted June 4, 2007.

^_* Reprint requests and correspondence: Dr. Julie A. Brothers, Division of Cardiology, The Children's Hospital of Philadelphia, 34th Street & Civic Center Boulevard, 2nd Floor Main, Philadelphia, Pennsylvania 19104. (Email: brothersj{at}email.chop.edu).

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, 1 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 1 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.

Abbreviations and Acronyms   AAOCA = anomalous aortic origin of a coronary artery with an interarterial course   ALCA = anomalous origin of left main coronary artery   ARCA = anomalous origin of right coronary artery   EST = exercise stress test   MPS = myocardial perfusion scan   SE = stress echocardiogram

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