A coronary artery-to-pulmonary artery fistula is a type of coronary arteriovenous fistula where there is a fistula (aberrant communicating vessel) between a coronary artery and either a pulmonary artery or the pulmonary trunk.
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Epidemiology
They may account for around 15-30% of cases of all coronary arterial fistulas 3.
Associations
- coronary artery aneurysms: ~20%
- pulmonary atresia: in pediatric cases
- ventricular septal defect: in pediatric cases
Pathology
They may be encountered in a variety of clinical settings. The most commonly accepted embryologic explanation is the Hackensellner involution-persistence hypothesis which suggests that in the normal scenario only two of six branches from the aortic sinus remain to form the coronary arteries, while the rest of the branches involute. A coronary-to-pulmonary artery fistula is formed when this involution fails 3.
Location
Coronary arteries involved 2:
- left-main or left anterior descending artery (most common)
- right coronary artery (second most common)
The most common pulmonary arterial vessel is the main pulmonary artery, implicated in nearly all cases 2.
Subtypes
Two types of coronary artery-to-pulmonary artery fistulas are known to occur:
- single prominent fistulous connection (usually between the LAD or RCA and the main pulmonary trunk)
- multiple small-caliber fistulous connections from the LAD or RCA, which drains into the main pulmonary trunk / pulmonary arterial system.
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