In the presence of coronary artery disease, angina
is a common finding in elderly patients. However, in
younger patients and, particularly in females, angina
should suggest congenital arteriovenous fistulas.
Although coronary fistulas are mostly congenital,
they may occur after blunt external thoracic trauma,
myocardial infarction, angioplasty or cardiac surgery.[
3]
Other congenital anomalies may be also accompanied
by coronary fistulas with an incidence of 40%.[
3]
They become symptomatic after the third decade of
life. The angina is caused by coronary steal. Fistulas
opening into the right heart chambers create shunts
from left-to-right which may result in congestive heart
failure in proportion to the size of the shunt. Such
symptoms as angina pectoris, fatigue, shortness of
breath, palpitation, and findings suggestive of rupture
of an aneurysm, embolism, endocarditis, congestive
heart failure can be seen. The degree of symptoms is
proportional to the amount of physiological coronary
stealing phenomenon developed by the fistula tract.[
4]
The frequency of the continuous murmur, the most
frequent physical examination finding, ranges from
20 to 80%.[
5] Almost half of the patients have nonspecific
electrocardiographic changes. Cardiomegaly
can be observed on telecardiography due to an
increased cardiac output.[
1] For a definitive diagnosis,
selective coronary angiography is required. Thus,
the origin and termination of the fistula, the path it
follows anatomically, and the affected structures can
be precisely visualized.
Coronary fistulas mainly originate from the right
coronary artery, less frequently from the left coronary
artery, or both. They were found to originate from
the right coronary artery with a frequency of 19.7% in
the Albeyoglu et al.,[6] 50% in the Levin et al.,[7] 65%
in Lowe,[8] and 51% in the Wilde and Watt[9] series.
Drainage occurs to the low-pressure heart chambers.
Frequency in a decreasing order is as the right
ventricle (39%), right atrium (33%), pulmonary artery
(20%), left atrium, coronary sinus, vena cava superior,
and bronchial arteries.[1,7] Drainage to the left heart
chambers is relatively rare (2%).[1] Pathophysiological,
myocardial ischemia or infarction occurs, when the
blood to be directed to the left ventricle is directed
to the low-resistance heart chambers through the
fistula, creating a steal phenomenon. In fistulas which
open to the left ventricle, the blood flow is frequently
diastolic. Ischemia occurs, when the pulse pressure providing the coronary flow is lowered by steal
phenomenon.[6] One of the potential complications
is premature atherosclerosis, resulting in an intimal
damage caused by high-volume blood flow.[3] Our
case had primarly angina symptoms and tachycardia
episodes.
Nonetheless, treatment of asymptomatic
fistulas is still controversial. However, most
surgeons agree that they should be closed in the
presence of a significant shunt or aneurysmal
dilatation.[1] Symptomatic patients need to be
treated. Cardiopulmonary bypass may be required
or not.[9] Fistulas which are intramural, short,
close to the sinus Valsalva and characterized by an
aneurysm are often closed using cardiopulmonary
bypass, while extramural and anatomically
accessible ones are closed using simple ligation
and resection. In addition, closure with the use
of percutaneous transcatheter closure devices has
become increasingly widespread, particularly in
pediatric patients.[10] Some authors do not consider
surgery in asymptomatic patients, while surgery
is appropriate for moderate to large-flow fistulas.
Small fistulas are likely to spontaneously close as
primarily or secondarily. Nevertheless, most authors
consider the surgical removal of fistulas to prevent
complications and sudden death risk, regardless of
whether they are symptomatic or not. The reported
operative mortality and morbidity rates are very low.
It should be kept in mind that fistula complications
may increase surgical morbidity and mortality.
In conclusion, we tried to evaluate giant sinus
node artery fistula in the light of literature data. In
particular, considering the causes of chest pain and
arrhythmia in young patients, the consideration of
coronary arteriovenous fistulas is important for the
correct diagnosis.
Declaration of conflicting interests
The authors declared no conflicts of interest with respect
to the authorship and/or publication of this article.
Funding
The authors received no financial support for the research
and/or authorship of this article.