Normal coronary artery anatomy is characterized by two ostia placed centrally in the right and left sinus of Valsalva. The three main coronary arteries branch superiorly to the atria and inferiorly to the ventricles; they end in broom-like arborizations, which penetrate the myocardium [
1]. Clinical suspicion that a patient's problem may be the result of coronary anomalies remains an important challenge in diagnosis, especially in children. Hemodynamically significant congenital anomalies of coronary arteries are shown in Table
1[
2]. One of the primary forms is coronary artery fistula (CAF), that are defined as a direct communication of a coronary artery with a cardiac chamber, great vessel or other vascular structure, bypassing the myocardial capillary bed. Embryologically, these fistulae seem to represent persistent junctions of primordial epicardial vessels with intramyocardial sinusoidal circulation. CAF accounts for 0.27–0.40% of all congenital defects [
3,
4] and was first described by Krause in 1865 [
5]. Some studies showed that the source is most often the right coronary artery (RCA) with the left coronary artery (LCA) being much less involved [
6], while other studies found that the origin is 50% the RCA and 50% LCA [
7‐
9]. Furthermore, there are studies showing that the LCA is more often the origin than RCA [
10]. The exit of the CAF is in decreasing order of frequency the right ventricle, right atrium and the pulmonary artery [
7,
8,
11]. Consistent in all studies is that the LV is a very uncommon exit of a CAF [
7‐
11]. Considering the rarity of this, we present a case of a CAF with the diagnosis and echocardiogradphic evaluation within the first days of life.
Table 1
Hemodynamically significant congenital anomalies of the coronary arteries
Isolated/primary – without CHD: | Secondary – with CHD: |
• Congenital coronary artery fistula | • PA + IVS |
• Anomalous origin of accessory coronary arteries from the pulmonary artery | • AA + MS |
• Ectopic origin of the coronary arteries from aortic sinus | |
• Absence of a coronary artery | |