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Coronary Fistula
Suhny Abbara, MD, FACR, FSCCT; Christopher M. Walker, MD
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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

                                          1. Yun G et al: Coronary artery fistulas: pathophysiology, imaging findings, and management. Radiographics. 38(3):688-703, 2018
                                          2. Shriki JE et al: Identifying, characterizing, and classifying congenital anomalies of the coronary arteries. Radiographics. 32(2):453-68, 2012
                                          3. Díaz-Zamudio M et al: Coronary artery aneurysms and ectasia: role of coronary CT angiography. Radiographics. 29(7):1939-54, 2009
                                          4. Zenooz NA et al: Coronary artery fistulas: CT findings. Radiographics. 29(3):781-9, 2009
                                          5. 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
                                          6. 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
                                          7. 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
                                          8. Kharouf R et al: Transcatheter closure of coronary artery fistula complicated by myocardial infarction. J Invasive Cardiol. 19(5):E146-9, 2007
                                          9. Latson LA: Coronary artery fistulas: how to manage them. Catheter Cardiovasc Interv. 70(1):110-6, 2007
                                          10. 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
                                          11. Gowda RM et al: Coronary artery fistulas: clinical and therapeutic considerations. Int J Cardiol. 107(1):7-10, 2006
                                          12. Luo L et al: Coronary artery fistulae. Am J Med Sci. 332(2):79-84, 2006
                                          13. 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
                                          14. 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
                                          15. Vavuranakis M et al: Coronary artery fistulas in adults: incidence, angiographic characteristics, natural history. Cathet Cardiovasc Diagn. 35(2):116-20, 1995
                                          16. 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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                                          Related Differential Diagnoses
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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

                                                                                  1. Yun G et al: Coronary artery fistulas: pathophysiology, imaging findings, and management. Radiographics. 38(3):688-703, 2018
                                                                                  2. Shriki JE et al: Identifying, characterizing, and classifying congenital anomalies of the coronary arteries. Radiographics. 32(2):453-68, 2012
                                                                                  3. Díaz-Zamudio M et al: Coronary artery aneurysms and ectasia: role of coronary CT angiography. Radiographics. 29(7):1939-54, 2009
                                                                                  4. Zenooz NA et al: Coronary artery fistulas: CT findings. Radiographics. 29(3):781-9, 2009
                                                                                  5. 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
                                                                                  6. 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
                                                                                  7. 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
                                                                                  8. Kharouf R et al: Transcatheter closure of coronary artery fistula complicated by myocardial infarction. J Invasive Cardiol. 19(5):E146-9, 2007
                                                                                  9. Latson LA: Coronary artery fistulas: how to manage them. Catheter Cardiovasc Interv. 70(1):110-6, 2007
                                                                                  10. 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
                                                                                  11. Gowda RM et al: Coronary artery fistulas: clinical and therapeutic considerations. Int J Cardiol. 107(1):7-10, 2006
                                                                                  12. Luo L et al: Coronary artery fistulae. Am J Med Sci. 332(2):79-84, 2006
                                                                                  13. 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
                                                                                  14. 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
                                                                                  15. Vavuranakis M et al: Coronary artery fistulas in adults: incidence, angiographic characteristics, natural history. Cathet Cardiovasc Diagn. 35(2):116-20, 1995
                                                                                  16. Sarkis A et al: [Left coronaro-ventricular fistula after septal myectomy.] Arch Mal Coeur Vaiss. 85(4):457-60, 1992