Abnormal direct connection between coronary artery and any cardiac chamber
Coronary arteriovenous fistula (CAVF)
Connection between coronary artery and any portion of systemic or pulmonary circulation
Definitions
Abnormal connection between coronary artery branch and cardiac or vascular chambers without normal transition through capillary bed of myocardium
May connect to pulmonary artery, bronchial artery, systemic veins, coronary veins, coronary sinus, atria, or ventricles
Accounts for 0.3% of congenital heart diseases
Usually congenital malformation (90% of cases); uncommonly develops as iatrogenic fistula after biopsy/surgery, irradiation therapy, or as traumatic fistula
CAVFs are differentiated from anomalous coronary artery origin from pulmonary artery [Bland-White-Garland syndrome, anomalous left coronary artery origin from pulmonary artery (ALCAPA), anomalous right coronary artery origin from pulmonary artery (ARCAPA)]
Main difference is in ALCAPA/ARCAPA, abnormal vessel arise from pulmonary artery, and no separate ostium from aorta is identified
IMAGING
General Features
Imaging Recommendations
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Yun G et al: Coronary artery fistulas: pathophysiology, imaging findings, and management. Radiographics. 38(3):688-703, 2018
Shriki JE et al: Identifying, characterizing, and classifying congenital anomalies of the coronary arteries. Radiographics. 32(2):453-68, 2012
Díaz-Zamudio M et al: Coronary artery aneurysms and ectasia: role of coronary CT angiography. Radiographics. 29(7):1939-54, 2009
Zenooz NA et al: Coronary artery fistulas: CT findings. Radiographics. 29(3):781-9, 2009
Cowles RA et al: Bland-White-Garland syndrome of anomalous left coronary artery arising from the pulmonary artery (ALCAPA): a historical review. Pediatr Radiol. 37(9):890-895, 2007
Gulati GS et al: Utility of multislice computed tomography in the diagnosis of a right coronary artery fistula to the right atrium. J Postgrad Med. 53(3):191-2, 2007
Iwasawa Y et al: Cardiac tamponade due to rupture of coronary artery fistulas with a giant aneurysm containing a free floating ball thrombus: a case report. J Cardiol. 50(1):71-6, 2007
Kharouf R et al: Transcatheter closure of coronary artery fistula complicated by myocardial infarction. J Invasive Cardiol. 19(5):E146-9, 2007
Latson LA: Coronary artery fistulas: how to manage them. Catheter Cardiovasc Interv. 70(1):110-6, 2007
Oncel D et al: An aneurysmal left circumflex artery-to-right atrium fistula in a patient with ischemic symptoms: accurate diagnosis with dual-source CT angiography. Cardiovasc Intervent Radiol. 2007
Gowda RM et al: Coronary artery fistulas: clinical and therapeutic considerations. Int J Cardiol. 107(1):7-10, 2006
Luo L et al: Coronary artery fistulae. Am J Med Sci. 332(2):79-84, 2006
Takahashi Y et al: Successful surgical treatment of a mycotic right coronary artery aneurysm complicated by a fistula to the right atrium. Jpn J Thorac Cardiovasc Surg. 53(12):661-4, 2005
Friedman WF et al: Diseases of the heart, pericardium and pulmonary vascular bed. In Braunwald E et al: Heart Disease. 6th ed. Philadelphia: W. B. Saunders. 1505-91, 2001
Vavuranakis M et al: Coronary artery fistulas in adults: incidence, angiographic characteristics, natural history. Cathet Cardiovasc Diagn. 35(2):116-20, 1995
Sarkis A et al: [Left coronaro-ventricular fistula after septal myectomy.] Arch Mal Coeur Vaiss. 85(4):457-60, 1992
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Synonyms
Coronary artery fistula
Coronary cameral fistula
Abnormal direct connection between coronary artery and any cardiac chamber
Coronary arteriovenous fistula (CAVF)
Connection between coronary artery and any portion of systemic or pulmonary circulation
Definitions
Abnormal connection between coronary artery branch and cardiac or vascular chambers without normal transition through capillary bed of myocardium
May connect to pulmonary artery, bronchial artery, systemic veins, coronary veins, coronary sinus, atria, or ventricles
Accounts for 0.3% of congenital heart diseases
Usually congenital malformation (90% of cases); uncommonly develops as iatrogenic fistula after biopsy/surgery, irradiation therapy, or as traumatic fistula
CAVFs are differentiated from anomalous coronary artery origin from pulmonary artery [Bland-White-Garland syndrome, anomalous left coronary artery origin from pulmonary artery (ALCAPA), anomalous right coronary artery origin from pulmonary artery (ARCAPA)]
Main difference is in ALCAPA/ARCAPA, abnormal vessel arise from pulmonary artery, and no separate ostium from aorta is identified
IMAGING
General Features
Imaging Recommendations
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Yun G et al: Coronary artery fistulas: pathophysiology, imaging findings, and management. Radiographics. 38(3):688-703, 2018
Shriki JE et al: Identifying, characterizing, and classifying congenital anomalies of the coronary arteries. Radiographics. 32(2):453-68, 2012
Díaz-Zamudio M et al: Coronary artery aneurysms and ectasia: role of coronary CT angiography. Radiographics. 29(7):1939-54, 2009
Zenooz NA et al: Coronary artery fistulas: CT findings. Radiographics. 29(3):781-9, 2009
Cowles RA et al: Bland-White-Garland syndrome of anomalous left coronary artery arising from the pulmonary artery (ALCAPA): a historical review. Pediatr Radiol. 37(9):890-895, 2007
Gulati GS et al: Utility of multislice computed tomography in the diagnosis of a right coronary artery fistula to the right atrium. J Postgrad Med. 53(3):191-2, 2007
Iwasawa Y et al: Cardiac tamponade due to rupture of coronary artery fistulas with a giant aneurysm containing a free floating ball thrombus: a case report. J Cardiol. 50(1):71-6, 2007
Kharouf R et al: Transcatheter closure of coronary artery fistula complicated by myocardial infarction. J Invasive Cardiol. 19(5):E146-9, 2007
Latson LA: Coronary artery fistulas: how to manage them. Catheter Cardiovasc Interv. 70(1):110-6, 2007
Oncel D et al: An aneurysmal left circumflex artery-to-right atrium fistula in a patient with ischemic symptoms: accurate diagnosis with dual-source CT angiography. Cardiovasc Intervent Radiol. 2007
Gowda RM et al: Coronary artery fistulas: clinical and therapeutic considerations. Int J Cardiol. 107(1):7-10, 2006
Luo L et al: Coronary artery fistulae. Am J Med Sci. 332(2):79-84, 2006
Takahashi Y et al: Successful surgical treatment of a mycotic right coronary artery aneurysm complicated by a fistula to the right atrium. Jpn J Thorac Cardiovasc Surg. 53(12):661-4, 2005
Friedman WF et al: Diseases of the heart, pericardium and pulmonary vascular bed. In Braunwald E et al: Heart Disease. 6th ed. Philadelphia: W. B. Saunders. 1505-91, 2001
Vavuranakis M et al: Coronary artery fistulas in adults: incidence, angiographic characteristics, natural history. Cathet Cardiovasc Diagn. 35(2):116-20, 1995
Sarkis A et al: [Left coronaro-ventricular fistula after septal myectomy.] Arch Mal Coeur Vaiss. 85(4):457-60, 1992
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