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A31 year old woman presented with significant effort induced dyspnoea and was noted to have a heart murmur at birth which was not investigated. During childhood and adolescence she experienced slight effort related dyspnoea and experienced a single syncopal event in her 20s. Physical examination disclosed a mild parasternal right ventricular (RV) lift and grade 3, high pitched, continuous murmur best heard at the mid upper left sternal border radiating to the right sternal border. Chest x ray demonstrated cardiomegaly involving the right atrium (RA) and main pulmonary artery as well as most of the lungs. On transthoracic echocardiography, the proximal left coronary artery (LCA) appeared large and tortuous and possibly communicated with the RA. Transoesophageal echocardiography (TOE) was performed to confirm and better define the anatomy and characterise the precise point of chamber communication of a presumed coronary arteriovenous fistula. TOE showed the proximal LCA to be 1.1 cm in diameter. The dilated coronary artery coursed posterior to the aortic root and terminated in multilobulated aneurysmal dilatations, maximum diameter 2.8 cm. The exit orifice of the fistula was approximately 1 mm in diameter with a peak Doppler velocity of 4.7 m/s. The proximal pulmonary arteries were mildly dilated, consistent with volume overload. Exercise ECG was negative for ischaemia, however the patient did experience significant (grade 3/4) exertional breathlessness and chest heaviness. Right heart pressures were normal. Coronary angiography confirmed the echocardiographic findings. The coronary artery fistula was surgically ligated, under continuous TOE monitoring and without going on cardiac bypass.