Approximately 25% of coronary arteriovenous fistulas present aneurysmal dilatation; however, spontaneous rupture of the aneurysm is rare. Most coronary arteries branching the feeding arteries demonstrate aneurysmal formation, possibly because of shunt flow.
A 48-year-old woman was referred to our institution for surgical management of ruptured aneurysm of coronary arteriovenous fistula. The aneurysm was located on the left-anterior aspect of the pulmonary artery trunk, communicating with both left and right coronary arteries through two small feeding arteries draining into the pulmonary artery trunk. Both left and right coronary arteries showed normal diameter. The feeding arteries were ligated externally, and fistulous openings were closed within the aneurysm. Postoperative course was uneventful.
Aneurysm of coronary arteriovenous fistula can occur in patients without aneurysmal coronary artery. Although the association of ruptured aneurysm with coronary fistulas is relatively rare, it should be considered a potential cause of acute cardiac tamponade.

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