Syndrome of congenital internal mammary-to-pulmonary arteriovenous fistula associated with mitral valve prolapse
L. A. Robinson and D. C. Sabiston Jr
Congenital fistulas of the internal mammary artery to the pulmonary
circulation are rare, with only 12 patients described in the world
literature. Two additional patients are now described who have an
associated prolapse of the mitral valve, one of whom had mitral
insufficiency. In most instances, the arteriovenous fistula is essentially
asymptomatic and is often discovered when a continuous murmur is heard on
routine chest examination. The definitive diagnosis is made best by
selective arteriography. The embryologic origin of these fistulous
communications may be the maldevelopment of accessory bronchial arteries
arising from the internal mammary artery. In view of the potential risks of
congestive heart failure as well as proximal dilation and degeneration of
the parent artery, secondary hypertension, and endocarditis, operative
closure of the arteriovenous fistula is recommended.
