Congenital coronary artery fistula: Presentation in the neonatal period and transcatheter closure

Background: Congenital coronary artery fistula is a rare coronary anomaly. Most commonly, such fistulae drain into the right side of the heart or the pulmonary artery. Children with coronary artery fistulae are generally asymptomatic, although they may have left ventricular enlargement in the setting of a moderate sized left to right shunt. Symptoms of congestive heart failure or ischemia are very rare in neonatal period, and suggest the presence of a very large shunt and/or coronary steal. Methods: Single center retrospective review of transcatheter intervention on coronary artery fistulae presenting with symptoms in the neonatal period from January 2000 to March 2018. Patients with associated congenital heart diseases (except atrial septal defect) were excluded. Patient records, catheterization data, angiograms and noninvasive imaging were reviewed. Results: Two patients underwent transcatheter intervention for symptomatic coronary artery fistula in the first few weeks of life. The first patient had multiple right and left coronary artery to right ventricle fistulae and presented with severe biventricular systolic dysfunction. Transcatheter closure of the fistulae was performed using multiple Gianturco coils. The second patient had a large left main coronary artery to left ventricle fistula (with left anterior descending and circumflex coronary artery atresia) presenting with symptoms of ischemia. This large fistula was closed using one Amplatzer Vascular Plug type-II and two Micro-Vascular Plugs. Both patients had improvement in symptoms post intervention and are doing well at the last follow up at 12 years and 7 months respectively. Conclusions: We hereby describe the rare presentation of symptomatic coronary artery fistulae in the neonatal period and their successful transcatheter management. This is also the first description of left anterior descending and circumflex coronary artery atresia in the setting of a large left main coronary artery to left ventricle fistula.