3. prevalence
in less than 1% of the population
0.1% to 0.2% of coronary angiographic
studies
0.2 to 0.4% of congenital anomalies of
the heart
Right coronary system- 55%
Left coronary system- 35%
Bilateral- 5%
No gender or race predilection
4. Etiology
congenital disorder- most common
acquired secondary to trauma or from
invasive cardiac procedures such as
pacemaker implantation,
endomyocardial biopsy, coronary artery
bypass grafting, coronary angiography,
septal myomectomy etc
5. Clinical features
Incidental finding
Asymptomatic in majority
symptoms of heart failure
symptoms of angina due to coronary
steal phenomenon
6. O/E- Collapsing pulse
Wide pulse pressure
Diffuse apex beat
Palpable third heart sound (S3)
cardiac murmur -loud, superficial, and
continuous at the lower or midsternal
border.
site of maximal intensity of the murmur
is related to the site of drainage
7. Fistula-related complications
not common
aneurysmal formation,
infective endocarditis,
Pulmonary hypertension and congestive heart
failure if a large left-to-right shunt exists
myocardial ischemia distal to the fistula due to
a “myocardial steal
pericardial effusion, and
supraventricular and ventricular arrhythmias
8. Diagnosis
ECG- usually normal, however,
evidence of ischemia or chamber
enlargement can be seen with large
shunts.
Chest Radiography- often normal and
seldom show selective chamber
enlargement
echocardiography -The shunt entry
site is characterized by a continuous
turbulent systolic and diastolic flow
pattern
CT Angiography – non invasive test
9. Cardiac catheterization and coronary
angiography:
- test of choice
- The anatomical characteristics (site of
origin, course, site of insertion), and the
exact size of the fistulae.
- This test will also provide information on
the hemodynamic significance of the
fistula
10. Management
depends on several factors including
1. origin of the fistula, i.e., proximal vs.
distal,
2. size of the fistula,
3. anatomy of the fistula,
4. patient’s age,
5. patient’s symptoms
6. existence of other indications for an
invasive procedure
11. In general, small coronary cameral fistulae
should be observed only with close
echocardiographic or angiographic follow
up to determine the enlargement of feeding
vessels over time.
Small fistulae have typically benign course,
asymptomatic, and even may close
spontaneously.
Large fistulae require closure.
There are two approaches for the closure
of coronary cameral fistulae: transcatheter
embolization and surgical closure
12. surgical approach indicated in-
1. large fistulae,
2. fistulae with multiple openings,
3. aneurysmal dilatation, or
4. acute angulations that are not
amenable to catheterization
13. Moderate to large fistulae without symptoms are
managed based on the location of the fistula.
For proximal fistulae, closure (transcatheter or
surgical) is the recommendation.
On the other hand, for distal fistulae, there are two
possible approaches for management.
1. observation with the use of antiplatelet therapy
indefinitely.
2. closure of the fistula, followed by the use of
antiplatelet therapy for one year until endothelization
of the operative surface occurs
14. Prolonged antiplatelet therapy may be
useful in patients with persistent
aneurysmal dilatation.
Patients treated surgically should
undergo regular stress testing and
repeat angiography, particularly if they
have sustained cardiac muscle loss
following the surgery
15. arteriosinusoidal subtypes are less
amenable to surgery, and management
with β-blocker pharmacotherapy is
described
prophylactic antibiotics against endocarditis
are not recommended for isolated coronary
cameral fistulae before procedures
associated with causing bacteremia.
However, antibiotic prophylaxis would be
indicated if there is coexisting cyanotic
congenital heart disease
16. Prognosis
Life expectancy for patients with a
coronary cameral fistula is normal.
both transcatheter and surgical
approaches for management are
associated with a good prognosis.
The need for additional surgery to treat
recurrent disease only presents in
around 4% of patients
