Diagnostic Considerations

Other conditions to consider in the differential diagnosis of coronary artery fistula include the following:

Ruptured sinus of Valsalva aneurysm
Ventricular septal defect with aortic insufficiency
Venous hums
Intrathoracic systemic fistulae
Congenital systemic fistula to the pulmonary vein
Pulmonary arteriovenous malformation
Aortopulmonary window
Congenital or acquired pulmonary vein stenosis

Important considerations

Be aware of coronary artery fistula to avoid missing the diagnosis.

Realize that conservative management may be appropriate in some patients, because spontaneous closure occurs in 23% of small fistulae, primarily those arising from the left coronary system.

Advise patients that they should receive appropriate antibiotics for any dental, oropharyngeal, gastrointestinal tract, or urologic surgical procedures if associated with a cyanotic heart disease.

Differential Diagnoses

Workup

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Media Gallery

Selective left coronary artery (LCA) injection demonstrating a markedly enlarged left main (*) with normal size circumflex (CX) and left anterior descending (LAD) branches. The fistula continues across the right ventricle free wall to the atrioventricular groove where it terminates at the crux of the heart in the right atrium (straight arrow). (Reproduced from Congenital Heart Disease, Textbook of Angiocardiography.)
Retrograde aortic root injection, dilated left main (LCA) and circumflex (CX) vessels with the fistulous connection to the right ventricle (arrow). (Reproduced from Congenital Heart Disease, Textbook of Angiocardiography.)
Three-dimensional multidetector row computed tomographic image showing a circumflex artery fistula. The left main stem is greatly dilated (arrow) and a dilated, tortuous circumflex artery becomes aneurysmal (An) before draining into the coronary sinus. Note also the left anterior descending (LAD) branches arising from this dilated vessel (arrowhead). (Image courtesy of 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.)
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.)
Coronary artery fistula from right coronary artery to right ventricle. B. No antegrade flow in the fistula after coil placement.
Large coronary artery fistula (A) before Amplatzer vascular plug and (B) after Amplatzer vascular plug placement.

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Author

Monesha Gupta, MD, MBBS, FAAP, FACC, FASE Associate Professor of Pediatrics, Division of Pediatric Cardiology and Nephrology, Children's Memorial Hermann Hospital, University of Texas Medical School

Monesha Gupta, MD, MBBS, FAAP, FACC, FASE is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, Medical Council of India

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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, American Autonomic Society, American Physiological Society

Disclosure: Received research grant from: Lundbeck Pharmaceuticals<br/>Received grant/research funds from Lundbeck Pharmaceuticals for none.

Chief Editor

Stuart Berger, MD Executive Director of The Heart Center, Interim Division Chief of Pediatric Cardiology, Lurie Childrens Hospital; Professor, Department of Pediatrics, Northwestern University, The Feinberg School of Medicine

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

Juan Carlos Alejos, MD Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

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, International Society for Heart and Lung Transplantation

Disclosure: Received honoraria from Actelion for speaking and teaching.

Coronary Artery Fistula Differential Diagnoses

Diagnostic Considerations

Important considerations

Differential Diagnoses

References

Tables

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