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Cardiac ultrasound (left) was performed on a 1 month old child with congestive heart failure. Both ventricles were dilated, and the right coronary artery was massively enlarged. There was torrential flow in the atrioventricular groove with the fistula entrance inferior and posterior to the septal leaflet of the tricuspid valve.
An ascending aortogram was performed in the lateral projection (middle). The posterior descending coronary artery (PDA) and posterolateral coronary artery (PL) arose at the origin of the fistula (RCA, right coronary artery; RV, right ventricle). Test occlusions using a 5 French Berman balloon catheter identified a very limited segment where coil deployment would not cause inferior wall ischaemia. Given the small margin for error and the low operative mortality for this lesion, the patient was referred for surgery.
At operation a massively dilated right coronary artery was seen (right). The fistula insertion was posterior (not seen). The fistula's short, intramyocardial course precluded epicardial ligation so the fistula entrance into the right ventricle was oversewn through the tricuspid valve on the endocardial surface with pledgetted mattress sutures. The patient did well and was discharged eight days after the operation.