Surgical management of a gigantic circumflex coronary artery aneurysm with fistulous connection to the coronary sinus

Abstract

We report the successful management of a gigantic circumflex coronary artery aneurysm with fistulisation into the coronary sinus. Transoesophageal echocardiography allowed continuous visualisation of ventricular wall motion during dissection and closure of the aneurysm whilst operating on cardiopulmonary bypass on the beating heart.

Coronary Artery Fistula From the Left Circumflex to the Coronary Sinus

“…Similarly, a coronary artery aneurysm is also a rare form of coronary artery disease, and is characterized by an abnormal dilatation to a diameter of more than 1.5 fold that of the adjacent normal coronary artery. 5) Although the majority of coronary artery aneurysms are due to coronary atherosclerosis, congenital fistulous connection of the coronary artery into a cardiac chamber or major vessel often causes a marked dilation of the donor coronary artery leading to aneurysm formation. Congenital fistulous connection of the coronary artery into a cardiac chamber or major vessel often causes a marked dilation of the donor coronary artery leading to aneurysm formation, as seen in our patient.…”

“…10) To our knowledge, only 8 cases of fistulous connection of an aneurysmal circumflex coronary artery to the coronary sinus have been previously described in the English literature (Table). [3][4][5][6][7][8] The optimal therapeutic strategy for CAF is not clearly established. Although it is evident that surgical intervention is necessary for symptomatic patients, those with complications, and those with a significant shunt (Qp/Qs > 2.0), the indications for surgical intervention in asymptomatic patients with no or only mild shunting are still controversial, because of the great variability in the natural history.…”

“…2) Among various coronary artery anomalies, fistulous connection of the aneurysmal circumflex coronary artery to the coronary sinus has been previously reported as an extremely uncommon form. [3][4][5][6][7][8] In this paper, we describe a case of an aneurysmal circumflex artery connecting to the coronary sinus through a fistula, and discuss surgical management for patients with CAF, particularly for asymptomatic patients with a small left-toright shunt.…”

Histopathologic Characteristics of a Coronary-pulmonary Artery Fistula with a Coronary Artery Aneurysm

“…Atrophy and destruction of the musculo-elastic elements of the vessel wall can promote the formation of aneurysms. 10,11) The anomalous coronary artery showed a well-defined internal elastic lamina and a thick muscular layer, which was immunohistochemically characterized by the positivity for αSMA, calponin, and desmin. In contrast, the aneurysm comprised a thin cellular wall with a lack of internal elastic lamina, abundant mucopolysaccharides and SMA/calponin-positive and desmin-negative spindle cells.…”

Giant Circumflex Coronary Artery Fistula Draining into the Coronary Sinus

“…It is defined as a direct connection between a coronary branch and a cardiac chamber, the coronary sinus, the inferior vena cava, or a pulmonary artery or vein. Only few cases of circumflex (Cx) coronary artery fistula, draining into the coronary sinus have been reported in the literature [2,3], representing only the 7% of all CAF [4]. The position and the dimension of the fistula are the most important factor influencing the hemodynamic significance and symptoms.…”