Coronary cameral fistula is a rare congenital heart disease where there is an abnormal connection between one or more coronary arteries and a heart chamber, vessel, or sinus. It can cause a left-to-right shunt and myocardial ischemia. Diagnosis is typically made using echocardiography, CT, MRI, or coronary angiography. Treatment involves closure of the fistula via percutaneous or surgical methods, with the approach depending on factors like fistula size, location, and whether it is causing symptoms or heart issues. Both techniques usually result in residual fistula in 20-30% of patients.
2. Definition
Coronary artery fistulae are a rare form of
congenital heart disease, which can cause a
communication between one or more coronary
arteries with heart chambers, coronary sinus,
arteries and pulmonary veins.
3. History
KRAUSE in 1865 was the first to describe the
disease in a case of accessory coronary artery,
which drained to the pulmonary vein, and
since then more than 400 cases have been
reported in publications.
4. Embryology
The embryogenesis of coronary arterial
fistulas is uncertain. Fistulas that enter
the right ventricle have been related to
persistence of primitive intramyocardial
sinusoids or to the development of a rectiform
vascular network in the distal branches of the
involved coronary artery.
8. Distribution
Approximately half of these fistulas arise
from the right coronary artery, somewhat less
from the left coronary artery, and only 5%
from both coronary arteries. Even more rarely,
all three coronary arteries are involved,or
multiple fistulas arise from one coronary
artery or from a single coronary artery.
9. An estimated 1% to 2% of coronary arterial
fistulas close spontaneously in infants,
children, and adults.
10. The physiologic consequences of coronary
arterial fistulas
depend on the volume of blood flowing through
them, the chamber or vascular bed into which
they drain, and the myocardial ischemia that
results from a coronary steal caused by low-
resistance vascular channels. About 10% of blood
from the aortic root normally enters the coronary
circulation, but in the presence of a coronary
arterial fistula, the volume is considerably larger.
A fistula that drains into the right atrium, right
ventricle, or coronary sinus constitutes a left-to-
right shunt. If drainage is into the right ventricular
outflow tract, pulmonary trunk
11. Coronary fistulae can involve connection to a
cardiac chamber (coronary–cameral fistula)
or to a central venous structure (coronary
arteriovenous fistula). The most common
sites for coronary fistulous connection are the
right ventricle, right atrium, and pulmonary
artery. Most patients who experience
symptoms related to a coronary artery fistula
present during the 4th through 6th decades of
life.
12. Large coronary artery fistulae are
uncommon. Small (<1-mm-diameter)
connections of coronary arteries, however, are
not uncommon. Such small coronary fistulae
are typically of no clinical or hemodynamic
consequence. In cases involving a large fistula
and marked flow, myocardial ischemia due to a
coronary steal phenomenon may occur. Other,
less common, complications related to
coronary artery fistulae—including
endarteritis, thrombosis within a fistula, fistula
rupture, and pulmonary hypertension— have
been reported.
13. The coronary artery that gives rise to the
fistula is characteristically dilated, elongated,
and tortuous. and the coronaries distal to the
fistula are of normal caliber. A fistulous
coronary artery may contain saccular
aneurysms that reach an astonishing size and
may rupture
15. Treatment
The management of coronary fistulae must
be individualized on:
1) the basis of the presence or absence of
cardiovascular symptoms,
2) the magnitude of the volume load on the
heart,
3) and the presence or absence of myocardial
ischemia or
4) ventricular dysfunction.
16. both percutaneous and surgical correction
have shown similar rates of residual fistula
flow (20%–30%) during follow-up.
Transcatheter approach,,,surgical
May involve continuous suturing , CPB and
venous graft to repair the coronary aneurysm,.
17. High-risk features for subsequent adverse events
were found to be more likely in patients with a
fistula draining into the CS, regardless of whether
they had undergone surgical or percutaneous
intervention. Even under open surgical inspection,
this abnormal coronary connection can be
particularly difficult to close completely,
consequent to the multiplicity of distal coronary
artery-to-CS connections and the location of the
connection on the posterior base of the heart.
18. Percutaneous closure is often feasible when
the fistulous communication departs from the
coronary artery proximally.
19. Surgical repair is often preferred when the fistula is
large and tortuous, with distal connections to the
low-pressure chamber. In the most recent
American College of Cardiology/American Heart
Association Guidelines for the Management of
Adults with Congenital Heart Disease,
percutaneous or surgical closure is a Class I
recommendation for large fistulae regardless of
symptoms and for small- to moderate-size fistulae
with evidence of myocardial ischemia, arrhythmia,
ventricular dysfunction, ventricular enlargement, or
endarteritis.
20. Example of cases
left and right
ventricular
enlargement,
and the dilated
coronary sinus
Coronary Arteriovenous Fistula
Todd L. Kiefer, MD
Anna Lisa Crowley, MD
James Jaggers, MD ,
J. Kevin Harrison, MD , Tex Heart Inst J 2012;39(2):218-22
21. the origins of
the fistulae from
the dilated left
circumflex and
right coronary
arteries, along
with the
uninvolved,
normal-
diameter left
anterior
descending
coronary artery.
24. References
• 1. Sommer RJ, Hijazi ZM, Rhodes JF Jr. Pathophysiology of
congenital heart disease in the adult: Part I: Shunt lesions.
Circulation 2008;117(8):1090-9.
• 2. Liberthson RR, Sagar K, Berkoben JP, Weintraub RM,
Levine FH. Congenital coronary arteriovenous fistula. Report
of 13 patients, review of the literature and delineation of
management. Circulation 1979;59(5):849-54.
