E2526. Coronary Artery Fistula in the Pediatric Age Group
  1. Ankita Chauhan; University of Tennessee Health Science Center/Le Bonheur Children's Hospital
  2. Vijetha Maller; University of Tennessee Health Science Center/Le Bonheur Children's Hospital
A coronary artery fistula (CAF) is an abnormal connection between the coronary artery and a major vessel (aorta or pulmonary) or a heart chamber, bypassing the myocardial capillary bed. Pediatric coronary artery fistulas are rare. Unlike adults, most pediatric coronary artery fistulas are congenital, with a higher incidence of associated cardiac defects. Through this exhibit, we will review CAF in children.

Educational Goals / Teaching Points
Our exhibit will discuss the congenital and acquired disease conditions associated with coronary artery fistulas. We will illustrate the imaging spectrum of different types of CAF in the pediatric age group and briefly discuss the pathophysiology and treatment options. Our discussion will include coronary-cameral fistulas and coronary arteriovenous fistulas.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
Coronary artery fistulas (CAF) may cause coronary steal, myocardial ischemia, or heart failure due to volume overload. A CAF to the right atrium or systemic veins is physiologically similar to an atrial septal defect. The fistula to the pulmonary arteries behaves like a patent ductus arteriosus. A CAF to the left heart may result in volume overload with similar pathophysiology as mitral or aortic regurgitation. Coronary CT angiography is the primary diagnostic technique to detect CAF and assess the structures involved and associated hemodynamics. It is also helpful in guiding interventional therapy. Based on the drainage site of the fistula, CAF is classified as coronary cameral fistula (involving any cardiac chamber, right atrium, right ventricle, left atrium, left ventricle), and coronary arteriovenous fistula involving pulmonary artery (coronary sinus, superior and inferior vena cava, bronchial vessels, other extracardiac veins). Sakakibara classification based on the site of origin (Sakakibara Type A - originates from the proximal native vessel with the proximal coronary segment dilated at the origin of the fistula - normal caliber of the distal end. Sakakibara Type B - originates from the distal native vessel with coronary dilated over the entire length and terminating as a fistula (end-artery type).

Clinical history, physical exam, and good communication with pediatric physicians help plan the appropriate imaging workup of pediatric patients with cardiovascular anomalies. Knowing the various imaging appearances of CAF in children further helps diagnose and plan the most appropriate management.