eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Coronary Artery Fistula: Treatment & Medication

Author: Andrew N Pelech, MD, Professor, Department of Pediatrics, Medical College of Wisconsin; Director of Cardiac Catheterization Laboratory, Cardiology Research Focus and Cardiology Database, Director of Herma Heart Center Clinical Research, Children's Hospital of Wisconsin; Chairman of Wisconsin Pediatric Cardiac Registry
Contributor Information and Disclosures

Updated: May 28, 2009

Treatment

Medical Care

In childhood, most patients with coronary artery fistulae (CAF) are asymptomatic; however, some patients may present with symptoms of dyspnea on exertion, increased fatigability, and, possibly, signs of high-output congestive heart failure. Rarely, patients may present with angina,7 palpitations, or signs of exercise-related coronary insufficiency. Direct medical treatment for symptomatic relief can be used until investigations and operative repair can be performed. Spontaneous closure is rare but may occur in small fistulae. Small fistulous connections in the asymptomatic patient may be monitored. Most lesions enlarge progressively and warrant operative repair, either by transcatheter or surgical techniques. Provide endocarditis prophylaxis in all patients.

Diagnostic cardiac catheterization should be performed initially with or without additional therapeutic intervention. Initial diagnostic catheterization should both define hemodynamic significance of the lesion and provide detailed angiographic assessment of the anatomy of the abnormality, in particular, the origin, course, regional narrowings, and the nature of the insertion.8 Procedural options can be optimized by careful identification of the number of fistulous connections, nature of feeding vessel or vessels, sites of drainage, and quantification of myocardium at risk for injury or loss. The goal of treatment is the obliteration of fistula, while preserving normal coronary blood flow. Therapeutic transcatheter embolization is described as follows:

  • Indications: In view of the natural progression in larger fistulae to dilate over time, with progressively increasing risk of thrombosis, endocarditis, or rupture, the general advice is to close all but the small fistulous connections. In borderline situations, provide close echocardiographic or angiographic follow-up imaging to identify enlargement of feeding vessel in asymptomatic patients. Patients with large fistulae, multiple openings, or significantly aneurysmal dilatation may not be optimal candidates for transcatheter closure.
  • Technique
    • Transcatheter embolization techniques using coils (see Media file 4), bags, or other devices can be performed on an outpatient basis at the time of diagnostic studies or later, and may obviate the need for cardiac surgical intervention.

      Selective left coronary angiogram immediately aft...

      Selective left coronary angiogram immediately after transcatheter coil occlusion of the circumflex coronary fistula (4 7-mm X 70-mm target coils). A tiny residual leak and the proximal circumflex coronary dilatation are shown. Image courtesy of Texas Heart Institute. (Reproduced from McMahon CJ, Nihill MR, Kovalchin JP, et al. Coronary artery fistula. Management and intermediate-term outcome after transcatheter coil occlusion. Tex Heart Inst J. 2001;28(1):21-5.)

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      Selective left coronary angiogram immediately aft...

      Selective left coronary angiogram immediately after transcatheter coil occlusion of the circumflex coronary fistula (4 7-mm X 70-mm target coils). A tiny residual leak and the proximal circumflex coronary dilatation are shown. Image courtesy of Texas Heart Institute. (Reproduced from McMahon CJ, Nihill MR, Kovalchin JP, et al. Coronary artery fistula. Management and intermediate-term outcome after transcatheter coil occlusion. Tex Heart Inst J. 2001;28(1):21-5.)

    • Generally, the course of the fistulous tract is delineated angiographically, selectively catheterized, and wired along its entire length. A delivery catheter or sheath is then positioned antegradely or retrogradely along the stabilizing wire for delivery of a suitable occlusive coil or device. The occlusive device is positioned so as to minimize myocardial muscle loss or injury. Often multiple devices or coils may be required for effective occlusion.
    • The transcatheter approach is, frequently, a fairly complicated intervention and requires an experienced operator and interventional specialist with expertise in both coronary arteriography and embolization techniques. Embolization often requires complicated catheter manipulation, as well as selection of various catheters and wires.
  • Results: To date, the literature has primarily provided only case reports and reports of small series. Results have been comparable to surgery without associated morbidities of cardiopulmonary bypass and/or sternotomy.

Surgical Care

Cardiac surgical intervention is described as follows:

  • Indications: Indications for surgical intervention are the same as in embolization (see above). Some fistulae are unsuitable for the transcatheter approach and preferably are addressed surgically. These coronary artery fistulae may include fistulae with multiple connections, circuitous routes, and acute angulations that make catheter positioning difficult or impossible.
  • Techniques: Surgical repair usually is approached via a median sternotomy and cardiopulmonary bypass. Identify the feeding vessel and delineate its course and site of insertion. Identify the site of presumed fistulous drainage prior to institution of the cardiopulmonary bypass. Transesophageal echocardiographic imaging has been very useful in assisting in the location of fistulous tract insertion. A typical procedure includes opening the chamber into which the fistula drains, identifying the fistula, and closing the site of drainage with a patch or suture. If the fistula enters the ventricle or if the feeding vessel is large, the coronary artery is opened, and the opening to the fistula is closed with a running suture. The arteriotomy is closed. Large aneurysms may require excision. Rarely, when the fistula is an end artery, it may be ligated with or without bypass.

Activity

  • Most patients should anticipate no restrictions on activity; however, patients who wish to compete in athletic endeavors should undergo stress testing and may be at marginally increased risk for dysrhythmias and sudden death.

Medication

  • The primary therapeutic approach to coronary artery fistula is interventional catheterization or surgery. Although medical therapy is seldom indicated, patients may require symptomatic treatment of congestive heart failure and/or coronary insufficiency until definitive treatment can be performed.
  • Antibiotics for endocarditis prophylaxis are required before performing procedures that may cause bacteremia. For more information, see Antibiotic Prophylactic Regimens for Endocarditis. Guidelines for antibiotic prophylaxis in cardiac surgery have been established.9

More on Coronary Artery Fistula

Overview: Coronary Artery Fistula
Differential Diagnoses & Workup: Coronary Artery Fistula
Treatment & Medication: Coronary Artery Fistula
Follow-up: Coronary Artery Fistula
Multimedia: Coronary Artery Fistula
References
Further Reading
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References

  1. Padfield GJ. A case of coronary cameral fistula. Eur J Echocardiogr. May 4 2009;[Medline].

  2. Cemri M, Sahinarslan A, Akinci S, Arslan U. Dual coronary artery-pulmonary artery fistulas. Can J Cardiol. Mar 2009;25(3):e95. [Medline].

  3. Schamroth C. Coronary artery fistula. J Am Coll Cardiol. Feb 10 2009;53(6):523. [Medline].

  4. Liberthson RR, Sagar K, Berkoben JP, et al. Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of management. Circulation. May 1979;59(5):849-54. [Medline].

  5. Weymann A, Lembcke A, Konertz WF. Right coronary artery to superior vena cava fistula: imaging with cardiac catheterization, 320-detector row computed tomography, magnetic resonance imaging, and transesophageal echocardiography. Eur Heart J. May 20 2009;[Medline].

  6. Chen ML, Lo HS, Su HY, Chao IM. Coronary artery fistula: assessment with multidetector computed tomography and stress myocardial single photon emission computed tomography. Clin Nucl Med. Feb 2009;34(2):96-8. [Medline].

  7. Saglam H, Koçogullari CU, Kaya E, Emmiler M. Congenital coronary artery fistula as a cause of angina pectoris. Turk Kardiyol Dern Ars. Dec 2008;36(8):552-4. [Medline].

  8. Ma ES, Yang ZG, Guo YK, Zhang XC, Sun JY, Wang RR. [Clinical value of 64-slice CT angiography in detecting coronary artery anomalies]. Sichuan Da Xue Xue Bao Yi Xue Ban. Nov 2008;39(6):996-9. [Medline].

  9. [Guideline] Society of Thoracic Surgeons Workforce on Evidence Based Surgery. Antibiotic prophylaxis in cardiac surgery. Part 1, duration of prophylaxis. 2005;[Full Text].

  10. Armsby LR, Keane JF, Sherwood MC, et al. Management of coronary artery fistulae. Patient selection and results of transcatheter closure. J Am Coll Cardiol. Mar 20 2002;39(6):1026-32. [Medline].

  11. Carrel T, Tkebuchava T, Jenni R, et al. Congenital coronary fistulas in children and adults: diagnosis, surgical technique and results. Cardiology. Jul-Aug 1996;87(4):325-30. [Medline].

  12. Culham JAG. Abnormalities of the coronary arteries. In: Freedom RM, Mawson JB, Yoo SJ, eds. Congenital Heart Disease: Textbook of Angiocardiography. Armonk, NY: Futura Publishing; 1997:849-67.

  13. De Wolf D, Vercruysse T, Suys B, et al. Major coronary anomalies in childhood. Eur J Pediatr. Dec 2002;161(12):637-42. [Medline].

  14. Demirkilic U, Gunay C, Bolcal C, et al. Are discrete coronary artery fistulae different from coronary arteriovenous malformations?. J Card Surg. Mar-Apr 2005;20(2):124-8. [Medline].

  15. Farooki ZQ, Nowlen T, Hakimi M, Pinsky WW. Congenital coronary artery fistulae: a review of 18 cases with special emphasis on spontaneous closure. Pediatr Cardiol. Oct 1993;14(4):208-13. [Medline].

  16. Freedom RM, Benson LN. The etiology of myocardial ischemia: surgical considerations. In: Pulmonary Atresia with Intact Ventricular Septum. Armonk, NY: Futura Publishing Co; 1989:233.

  17. Gittenberger-de Groot AC, Sauer U, Bindl L, et al. Competition of coronary arteries and ventriculo-coronary arterial communications in pulmonary atresia with intact ventricular septum. Int J Cardiol. Feb 1988;18(2):243-58. [Medline].

  18. Latson LA, Forbes TJ, Cheatham JP. Transcatheter coil embolization of a fistula from the posterior descending coronary artery to the right ventricle in a two-year-old child. Am Heart J. Dec 1992;124(6):1624-6. [Medline].

  19. Mahoney LT, Schieken RM, Lauer RM. Spontaneous closure of a coronary artery fistula in childhood. Pediatr Cardiol. 1982;2(4):311-2. [Medline].

  20. Manghat NE, Morgan-Hughes GJ, Marshall AJ, Roobottom CA. Multidetector row computed tomography: imaging congenital coronary artery anomalies in adults. Heart. Dec 2005;91(12):1515-22. [Medline].

  21. McMahon CJ, Nihill MR, Kovalchin JP, et al. Coronary artery fistula. Management and intermediate-term outcome after transcatheter coil occlusion. Tex Heart Inst J. 2001;28(1):21-5. [Medline]. [Full Text].

  22. Moskowitz WB, Newkumet KM, Albrecht GT, et al. Case of steel versus steal: coil embolization of congenital coronary arteriovenous fistula. Am Heart J. Mar 1991;121(3 Pt 1):909-11. [Medline].

  23. Parga JR, Ikari NM, Bustamante LN, et al. Case report: MRI evaluation of congenital coronary artery fistulae. Br J Radiol. Jun 2004;77(918):508-11. [Medline].

  24. Reidy JF, Tynan MJ, Qureshi S. Embolisation of a complex coronary arteriovenous fistula in a 6 year old child: the need for specialised embolisation techniques. Br Heart J. Apr 1990;63(4):246-8. [Medline].

  25. Said SA, el Gamal MI, van der Werf T. Coronary arteriovenous fistulas: collective review and management of six new cases--changing etiology, presentation, and treatment strategy. Clin Cardiol. Sep 1997;20(9):748-52. [Medline].

  26. Tkebuchava T, Von Segesser LK, Vogt PR, et al. Congenital coronary fistulas in children and adults: diagnosis, surgical technique and results. J Cardiovasc Surg (Torino). Feb 1996;37(1):29-34. [Medline].

  27. Trehan V, Yusuf J, Mukhopadhyay S, et al. Transcatheter closure of coronary artery fistulas. Indian Heart J. Mar-Apr 2004;56(2):132-9. [Medline].

  28. Urrutia-S CO, Falaschi G, Ott DA, Cooley DA. Surgical management of 56 patients with congenital coronary artery fistulas. Ann Thorac Surg. Mar 1983;35(3):300-7. [Medline].

  29. Vavuranakis M, Bush CA, Boudoulas H. Coronary artery fistulas in adults: incidence, angiographic characteristics, natural history. Cathet Cardiovasc Diagn. Jun 1995;35(2):116-20. [Medline].

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Keywords

coronary artery fistula, CAF, coronary cameral fistula, coronary arteriovenous fistula, heart disease, coronary fistula, cardiac anomalies, cardiac fistula, cardiac disease, coronary artery anomaly, coronary arterial-venous fistula, CAVF, coronary-pulmonary artery fistula, congestive heart failure, CHF, pulmonary artery branch stenosis, coarctation of the aorta, pulmonary stenosis, coronary stenosis, aortic atresia, myocardial infarction, arrhythmias, infectious endocarditis, aneurysm, treatment, diagnosis

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Contributor Information and Disclosures

Author

Andrew N Pelech, MD, Professor, Department of Pediatrics, Medical College of Wisconsin; Director of Cardiac Catheterization Laboratory, Cardiology Research Focus and Cardiology Database, Director of Herma Heart Center Clinical Research, Children's Hospital of Wisconsin; Chairman of Wisconsin Pediatric Cardiac Registry
Disclosure: Nothing to disclose.

Medical Editor

Juan Carlos Alejos, MD, Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California at Los Angeles
Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and International Society for Heart and Lung Transplantation
Disclosure: Actelion Honoraria Speaking and teaching

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Julian M Stewart, MD, PhD, Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College
Julian M Stewart, MD, PhD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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