Myocardial Perfusion, Scarring, and Function in Anomalous Left Coronary Artery From the Pulmonary Artery Syndrome: A Long-Term Analysis Using Magnetic Resonance Imaging

Abstract

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect. We aimed to examine the role of cardiac magnetic resonance imaging (MRI) in the long-term surveillance ofrepaired ALCAPA with regard to myocardial scarring, wall motion abnormalities, perfusion deficits, and myocardial function. Twenty-one patients after direct reimplantation of ALCAPA (median age at operation, 2.8 years) wereexamined after a median 10.6 years by MRI at restandunder dobutamine stress conditions, echocardiography, and ergometry. Results were compared with preoperative, immediately postoperative (5 days), and intermediate-term (5.8 years) echocardiography. No early or late deaths occurred. Improvements in indexed left ventricular end-diastolic dimension, ejection fraction, and mitral valve function were observed in all patients. However, MRI at rest showed wall motion abnormalities in 67% and perfusion deficits in 28%. Myocardial scars were seen in 67%. Dobutamine stress MRI detected wall motion abnormalities in 19% and perfusion deficits in 14%, which were not seen on MRI at rest. Exercise testing did not reflect cardiac dysfunction. Although long-term follow-up showed global left ventricular function had improved after ALCAPA repair, MRI showed left ventricular wall motion abnormalities, perfusion deficits, and myocardial scarring were seen in many patients. Dobutamine stress MRI identified deficits that were not evident on MRI at rest, and can therefore be considered a valuable surveillance tool. These results suggest the need for lifelong surveillance of repaired ALCAPA.