Coronary Arteriovenous Fistulae: A Review

Abstract

Coronary arteriovenous fistulae (CAVF) are a major coronary anomaly, being present in 0.002% of the general population and was first described by Krause in 1865.1,2 The majority are congenital in origin, representing 0.4% of all cardiac malformations.3 These coronary artery anomalies involve one or more coronary arteries abnormally communicating with one of the cardiac chambers (coronary cameral fistulae) or the great vessels adjacent to the heart (coronary artery or arteriovenous fistulae). 4According to the classification by Ogden in 1970, CAVF belong to coronary arteries anomalies that also include anomalous pulmonary artery origins of the coronaries, anomalous aortic origins of the coronaries, congenital atresia of the left main coronary artery, coronary artery bridging, coronary aneurysms, and coronary stenosis. Coronary anomalies may be abnormalities of origin, distribution, or termination. CAVF are considered mainly to be termination abnormalities. 5,6Nomenclature is based upon a descriptive analysis of the vessel of origin and the chamber of termination. According to Sakakibara et al, there are two types of angiographic classification: Type A-proximal type, where the proximal coronary segment is dilated to the origin of the fistula and the distal end is normal; and Type B-distal type, where the coronary artery is dilated over its entire length terminating as a fistula mainly into the right side of the heart (end-artery type) and the proximal coronary segment can or might have regular branches.6 Each one of these groups can be presented with different clinical signs and symptoms.6,7 EpidemiologyThe incidence of coronary anomalies is 0.2 to 1.2% of the population. 6CAVF are present in 0.002% of the general population and comprise 48.7% of all congenital coronary anomalies.8,9 They are the most common of hemodynamically significant coronary lesions. 8-11Among all pediatric coronary vasculature anomalies, approximately half of them are CAVF. 12,13Keywords ► arteriovenous fistula ► cardiac surgery ► coronary artery ► fistula ► symptomatic ► cardiovascular disease ► cardiac catheterization AbstractCoronary arteriovenous fistulae are a coronary anomaly, presenting in 0.002% of the general population. Their etiology can be congenital or acquired. We present a review of recent literature related to their epidemiology, etiology, pathophysiology, clinical presentation, diagnostic approach, and therapeutic management.

Congenital multiple coronary arteriovenous fistulas

“…Coronary arteriovenous fistulas have a low incidence in the general population (0.3%‐0.8%), originating from the left and right coronary arteries in 5% of cases and draining into the PA in 17% of cases 1. After the diagnosis, an exercise stress echocardiography was performed that was negative for ischemia and showed good functional capacity.…”

Coronary Artery Fistulas: Case Series and Literature Review

“…CAFs are defined as abnormalities of termination. There is abnormal direct communication between one or more coronary arteries with another vessel (also called coronary-vascular fistulas) or a cardiac chamber (called coronary-cameral fistulas) bypassing the myocardial capillary network [2,3,4,5,6,7,8]. This is in contrast to abnormalities of origin (i.e., anomalous coronary arteries or arteries arising from the pulmonary artery).…”

“…During coronary angiography, CAFs are usually discovered as a coincidental finding, with a prevalence of 0.002% in the general population [3,11,14,21,22,23,24,25]. Among patients undergoing coronary angiography, the prevalence of CAFs is approximately 0.05-0.25% [3,14,15,23,26]. In patients undergoing coronary computed tomography angiography, the prevalence of CAFs ranges from 0.4-0.9% [14,15].…”

“…The circumflex coronary artery is rarely involved (17-18%) [10,11,14,23,27,28,29]. A single origin is the most common form of CAF, ranging from 74 to 90% of cases [3,11,17]. The left and right coronary systems are simultaneously involved in approximately 4-18% of reported cases [4,7,10,14,17,22,29,30].…”

“…They comprise 13-50% of congenital coronary artery anomalies [3,5,9,10,11,12]. Communications between the coronary arteries and the cardiac chambers are thought to develop during coronary artery development.…”