PMC6642646_01
Electrocardiogram showed fibrillation, complete left bundle branch block, and occasional premature ventricular beats.
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The electrocardiogram showed fibrillation with a ventricular rate of 96 beats per minute (Figure 1), complete left bundle branch block, occasional premature ventricular beats.
Transthoracic echocardiography. (A) Abundant collateral circulation. (B) Severe mitral regurgitation. (C) Dilated right coronary artery and severe tricuspid regurgitation. (D) Moderate pulmonary hypertension with pulmonary artery pressure of 56 mmHg.. Abbreviations: MR, mitral regurgitation; TR, tricuspid regurgitation; RCA, right coronary artery.
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Transthoracic echocardiography (TTE) showed left main coronary artery (LMCA) originating from the pulmonary artery (PA), systolic and diastolic blood flow signals at the origin of LMCA, slightly decreased left ventricular function (ejection fraction 52%), enlargement of the ventricle and atrium, moderate pulmonary hypertension with pulmonary artery pressure of 56 mmHg, expansion of the right coronary artery (RCA), and severe mitral and tricuspid regurgitation (Figure 2).
Coronary computed tomography angiography (CTA)clearly identifies the anomalous origin of the left main coronary artery (LMCA) from pulmonary artery (PA) (asterisks, A and B). The construction image of the double source coronary CTA shows the LMCA originating from the PA with no identified connection to the aortic root, a severely dilated  right coronary artery (RCA), and the abundant intercoronary collaterals.. (C) Anterior position; (D) posterior position.. Abbreviations: LA, left atrium; RA, right atrium; RCA, right coronary artery.
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Then, PA computed tomography angiography (CTA) and double source CT coronary angiography revealed a congenital anomalous LMCA originating from the PA with no identified connection to the aortic root and a severely dilated RCA originating from the normal sinus of Valsalva along with abundant intercoronary collaterals circulation (Figure 3). The diagnosis of BWGS was confirmed.
Intraoperative TEE. (A) Modified mid-esophageal aortic valve short view, color flow Doppler examination showed that the left main coronary artery (LMCA) origin was showed arising from pulmonary artery (PA), with the retrograde flow draining from dilated LMCA into the PA. (B) Modified mid-esophageal aortic valve long view showed severely dilated  right coronary artery (RCA).. Abbreviations: RV, right ventricle; IAS, interatrial septum;  LA, left atrium; RA, right atrium; TEE, transesophageal echocardiagraphy.
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Intraoperative transesophageal echocardiagraphy (TEE) showed severely dilated RCA and the LMCA originating from PA, 16 mm distal to the pulmonic valve, with the retrograde flow draining from dilated LMCA into the PA, causing coronary "steal phenomenon" (Figure 4).