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An asymptomatic 7 week old infant presented with a soft continuous murmur at the left sternal border and decreased volume femoral pulses. Both the chest radiograph and ECG were unremarkable. Echocardiography revealed an aortic coarctation and dilatation of the right aortic sinus. Colour flow mapping showed heavy flow in the right coronary artery and a fistulous communication with the left atrium. Angiography showed bilateral coronary fistulae from the right and left coronary sinuses with drainage to the left atrial appendage and coarctation (see figs: CA, coarctation of the aorta; FT, fistulous tract; LAA, left atrial appendage; LCF, left coronary fistula; RCA, right coronary artery; RCF, right coronary fistula). The right coronary artery and the ascending aorta were greatly dilated, suggesting an underlying aortopathy. An extended end-to-end repair of the coarctation via a left thoracotomy was undertaken. The smaller left coronary fistula was visualised and ligated. The patient made an uneventful recovery.
At the age of 9 months surgical exploration via a median sternotomy was undertaken to allow safe closure of the right coronary fistula. A large aneurysmal origin of the right coronary orifice was found. The fistulous tract descended posteriorly, between the aortic root and the roof of the left atrium, discharging into the base of the left atrial appendage. A bovine patch was sutured to the free edge of the origin of the fistulous aneurysm to protect the origin of the right coronary. The distal end of the fistulous tract was then closed with a liga clip. Recovery was uneventful and the patient remains well at 18 months follow up. The differential diagnosis of a continuous murmur includes a persistent arterial duct and, more rarely, an arteriopulmonary window or an arteriovenous fistula.