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A 23 year old man without any prior history or symptoms of heart disease was referred to the adult echocardiography laboratory for evaluation of a systolic heart murmur with its maximal intensity at the apex. Echocardiography showed a dilated left ventricle with severely impaired contractile function and moderate mitral regurgitation. When imaging the proximal aorta (Ao) in its short axis, an enlarged proximal portion of the right coronary artery was identified arising from its normal origin (A). The left main coronary artery (LCA) could not been seen from this view but the LCA with proximal left anterior descending (LAD) and left circumflex (LCX) branches were found to originate from the pulmonary trunk (PA) on transthoracic (A, B, C) and multiplane transoesophageal (D) echocardiography (PV, pulmonary artery valve). Coronary angiography confirmed the diagnosis of Bland-White-Garland syndrome and severely impaired left ventricular function with anterior hypokinesia. Following injection of contrast into the right coronary artery (E, F) retrograde filling of the left coronary artery system via collateral vessels originating from the RCA was also noted, partially opacifying the pulmonary artery. The patient was referred for successful surgical correction by direct coronary reimplantation.