Myocardial perfusion, scarring, and function in anomalous left coronary artery from the pulmonary artery syndrome: A long-term analysis using magnetic resonance imaging

Background. Anomalous left coronary artery from thepulmonary artery (ALCAPA) is a rare congenital heartdefect. We aimed to examine the role of cardiac magneticresonance imaging (MRI) in the long-term surveillanceof repaired ALCAPA with regard to myocardial scarring,wall motion abnormalities, perfusion deficits, andmyocardial function.

Methods. Twenty-one patients after direct reimplantationof ALCAPA (median age at operation, 2.8 years)were examined after a median 10.6 years by MRI atrest and under dobutamine stress conditions, echocardiography,and ergometry. Results were compared withpreoperative, immediately postoperative (5 days), andintermediate-term (5.8 years) echocardiography.

Results. No early or late deaths occurred. Improvementsin indexed left ventricular end-diastolic dimension,ejection fraction, and mitral valve function wereobserved in all patients. However, MRI at rest showedwall motion abnormalities in 67% and perfusion deficitsin 28%. Myocardial scars were seen in 67%. Dobutaminestress MRI detected wall motion abnormalities in 19%and perfusion deficits in 14%, which were not seen onMRI at rest. Exercise testing did not reflect cardiacdysfunction.

Conclusions. Although long-term follow-up showedglobal left ventricular function had improved afterALCAPA repair, MRI showed left ventricular wall motionabnormalities, perfusion deficits, and myocardialscarring were seen in many patients. Dobutamine stressMRI identified deficits that were not evident on MRI atrest, and can therefore be considered a valuable surveillancetool. These results suggest the need for lifelongsurveillance of repaired ALCAPA.