Although a CAVF, in the strictest sense of the term, implies a communication between the coronary arteries and coronary venous system, the term has been adopted and accepted to refer to any communication between the coronary arteries and other cardiac or vascular structures 1. Furthermore, large CAVFs have sometimes been known as ‘cor medusae’ in the literature 2.
CAVFs are rare, being present in approximately 0.002% of the population 3,4.
Patients may be asymptomatic 1,3-5, but may also have a range of clinical features:
- angina, especially on exertion 1,3-5
- dyspnea, especially on exertion 1,3-5
- cardiac murmur heard continuously in systole and diastole, decreases in intensity during inspiration 1,3-5
The most common origins for the CAVF are the RCA (50%) and LAD (42%) while the most common drainage sites are the relatively low-pressure right ventricle (41%), right atrium (26%), or pulmonary artery (17%) 3,4. However, any coronary artery may be an origin and the fistula may less commonly drain into the coronary sinus, superior vena cava, or left-sided cardiac chambers 3,4.
Due to the most common drainage sites being in the right side of the cardiac circulation, CAVFs often cause a left-to-right shunt and result in the ‘coronary artery steal’ phenomenon 3-5. This coronary artery steal results in less blood being delivered to the myocardium at the origin of the fistula causing continual myocardial ischemia and the development of the aforementioned clinical presentation 3-5.
CAVFs are most commonly congenital, but can also be rarely acquired 3,4. Risk factors and etiologies for acquired CAVFs include 4:
- pathology-related: acute myocardial infarction, hypertrophic cardiomyopathy, dilated cardiomyopathy, coronary artery aneurysm rupture into adjacent structures 4
- iatrogenic: percutaneous coronary intervention, coronary artery bypass grafting, cardiac transplant, pacemaker placement 4
- chest trauma 4
Sub types / Classification
They can be classified according to their origin, drainage site, or complexity; the number of fistulous tracts; or the presence of accompanying anomalies.
One method of classifying coronary arterial fistulae is as 8
- coronary cameral fistula: which coronary arteries drain into a cardiac chamber
- coronary arteriovenous fistulae
Imaging can demonstrate not only the CAVF, but also the numerous complications (as listed below).
Coronary artery angiography is an invasive imaging modality for evaluating CAVFs and has long been considered to be the gold-standard 3,4,6,7. Angiography is able to provide details regarding the origin, drainage, size, and course of CAVFs 3,4,6,7.
Electrocardiographically-gated CT is able to not only detect the same characteristics of CAVFs as demonstrated by coronary artery angiography, but also has the additional benefit of being easily accessible and non-invasive 3,7. Thus, CT is increasingly considered to be the ideal imaging modality for evaluating CAVFs instead of angiography 3,7.
Treatment and prognosis
Although there have been reports of spontaneous closure of the fistula due to thrombus, generally symptomatic patients require closure via either transcatheter embolization or surgical ligation 4. Patients with known CAVFs should also receive prophylactic anti-platelet therapy and antibiotic prophylaxis against bacterial infective endocarditis 4.
Patients who have undergone a closure procedure tend to have an excellent prognosis 4.
- 1. Steinberg I, Baldwin JS, Dotter CT. Coronary Arteriovenous Fistula. Circulation. 17 (3): 372. doi:10.1161/01.CIR.17.3.372 - Pubmed
- 2. Wong GR, Nelson AJ, Rajwani A. Cor Medusae: Giant Coronary Arteriovenous Fistula. Rev Esp Cardiol (Engl Ed). 2016 Oct;69(10):976-977. DOI:10.1016/j.rec.2015.12.025 - Pubmed
- 3. Zenooz NA, Habibi R, Mammen L, Finn JP, Gilkeson RC. Coronary artery fistulas: CT findings. Radiographics : a review publication of the Radiological Society of North America, Inc. 29 (3): 781-9. doi:10.1148/rg.293085120 - Pubmed
- 4. Challoumas D, Pericleous A, Dimitrakaki IA, Danelatos C, Dimitrakakis G. Coronary arteriovenous fistulae: a review. The International journal of angiology : official publication of the International College of Angiology, Inc. 23 (1): 1-10. doi:10.1055/s-0033-1349162 - Pubmed
- 5. Engle MA, Goldsmith EI, Holswade GR, Goldberg HP, Glenn F. Congenital coronary arteriovenous fistula. Diagnostic evaluation and surgical correction. The New England journal of medicine. 264: 856-8. doi:10.1056/NEJM196104272641705 - Pubmed
- 6. Fujise K, Sherman W. Images in clinical medicine. Coronary arteriovenous fistula on coronary angiography. The New England journal of medicine. 331 (19): 1265. doi:10.1056/NEJM199411103311904 - Pubmed
- 7. Kacmaz F, Isiksalan Ozbulbul N, Alyan O, Maden O, Demir AD, Atak R, Senen K, Erbay AR, Balbay Y, Olcer T, Ilkay E. Imaging of coronary artery fistulas by multidetector computed tomography: is multidetector computed tomography sensitive?. Clinical cardiology. 31 (1): 41-7. doi:10.1002/clc.20286 - Pubmed
- 8. Gabin Yun, Tae Hyun Nam, Eun Ju Chun. Coronary Artery Fistulas: Pathophysiology, Imaging Findings, and Management. (2018) RadioGraphics. 38 (3): 688-703. doi:10.1148/rg.2018170158 - Pubmed
Related Radiopaedia articles
Congenital coronary artery anomalies
congenital coronary artery anomalies
- absent coronary artery
- coronary hypoplasia
- ectopic origin
- anomalous left coronary artery off the pulmonary artery (ALCAPA)
anomalous aortic origin of coronary artery
- origination of a coronary artery from the opposite sinus (ACAOS)
- left circumflex or LAD artery arising from the right coronary sinus
- right coronary artery arising from mid portion of LAD
- right coronary artery originating from the left sinus (ARCA)
- left main artery arising from the right anterior sinus (ALMCA)
- single coronary artery
- LCA or RCA (or a branch of either artery) arising from the non-coronary sinus
- LCx and LAD arising separately from the left coronary sinus
- anomalous location of coronary ostia
- from sinotubular junction
- from ascending aorta
- split artery origins
- origination of a coronary artery from the opposite sinus (ACAOS)
- anomalous course of coronary arteries
- anomalies of intrinsic coronary arterial anatomy
- congenital ostial stenosis or atresia (LCA, LAD, RCA, Cx)
- coronary ostial dimple
- coronary ectasia or aneurysm
- intramural coronary artery (myocardial bridging)
- subendocardial coronary course
- coronary crossing
- anomalous origin of posterior descending artery (PDA) from the anterior descending branch or a septal penetrating branch
- dual right coronary artery
- dual left anterior descending artery
anomalies in coronary arterial termination
- inadequate arteriolar/capillary ramifications
- presence of fistulation from terminal coronary arteries to various structures (coronary arteriovenous fistula)
- extracardiac termination