Multimodal cardiac magnetic resonance imaging of ALCAPA syndrome

An 18-month-old boy presented to cardiology clinic for enlarged heart. Echocardiography revealed a dilated left ventricle with an ejection fraction of 33%, and turbulent flow inside the interventricular septum (Panel A). He underwent a comprehensive cardiac magnetic resonance imaging (MR) including coronary MR angiography, T1 mapping, perfusion imaging, and late gadolinium enhancement (LGE). Coronary MR angiography showed anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA) (Panel B). The extracellular volume (ECV) map demonstrates >40% global myocardial ECV. Increased global ECV was consistent with perfusion defect of perfusion imaging and late enhancement of LGE in endocardium of the anterior, lateral wall of left ventricle (Panels D–F). Coronary angiography confirmed diagnosis of ALCAPA and selective coronary angiography detected retrograde flow from right coronary artery to left coronary artery and pulmonary artery (Panel C and Supplementary material online, Videos S1 and S2). Anomalous origin of the left coronary artery arising from the pulmonary artery is a rare congenital coronary anomaly associated with early infant mortality. With high soft tissue resolution and no radiation exposure, multimodal cardiac MR enables to non-invasively evaluate the anomalous origin of coronary artery, ventricular function, and tissue characterization in the meantime. Supplementary material is available at European Heart Journal online. Conflict of interest: The authors have submitted their declaration which can be found in the article Supplementary Material online.