Discussion
Anomalous origin of the right coronary artery is a rare congenital
anomaly that was 1st described in 1948 by White and
Edwards7 .The prevalence of this anomaly in the white
population, as determined from autopsy studies, is
0.026%.8The prevalence of this anomaly in patients
undergoing coronary angiography was0.25% 9-10 .
It is well established that an anomalous origin of the right coronary
artery can lead to angina pectoris, myocardial infarction, or sudden
death, in the absence of atherosclerosis11. The
pathophysiologic basis for this association, however, is unclear.
Mechanical compression of the right coronary artery by the great vessels
is the usual explanation, because the anomalous right coronary artery
generally courses between the aorta and the pulmonary artery to its
normal position. Others have suggested that the oblique angle at the
juncture of the anomalous right coronary artery and the left coronary
sinus produces a slit-like orifice in the aortic wall that can collapse
during exercise11 Recently, Kaku’s
group12 suggested that the proximal portion of the
right coronary artery, situated between the aorta and the pulmonary
artery, might be more prone to spasm than it would be otherwise.
Regardless of the causes of the adverse associations, there is a need to
study prospectively those patients at highest risk. Moreover, the
anomalous right coronary artery was 2nd only to the anomalous left main
coronary artery as the cardiac anomaly ,most frequently associated with
sudden cardiac death.
In an effort to stratify such cases by risk, Taylor and associates later
(in 1997)13performed a blinded pathologic analysis of
21 cases of anomalous origin of the right coronary artery. Despite
reviewing a variety of anatomic variables (including ostial size, length
of intramural course, angle of takeoff, and the presence of symptoms),
this group found that only an age of 30 years or older was associated
with a lower incidence of sudden cardiac death.
Understandably, the choice of treatment for this coronary anomaly is
controversial, with some advocating revascularization in all cases.
Proposed options include translocation of the right coronary artery to
the aorta,14ostioplasty (excision of the common wall
between the right coronary artery and the aorta),15and bypass grafting of the right coronary artery (with optional ligation
of the native artery proximal to the graft anastomosis to prevent
competitive flow)16.However, the long-term benefits of
such therapies have not yet been demonstrated.
In Japan, treatment for this condition is more conservative. Kaku and
associates17studied 56 patients with an anomalous
origin of a coronary artery and treated them medically with β-blockers.
Approximately 9% of these patients experienced episodes of hypotension
and arrhythmias on exertion, and no death was found to be directly
related to the congenital anomaly during 5 years of follow-up.
With the available data, it would be good idea to subject them for
illicitable ischemia . Any evidence of the risk factors such as oblique
course, slit like opening and long abnormal course should err towards
re-translocation. Otherwise, close monitoring with frequent evaluation
for ilicitable ischemia as in our case may be a good alternative.
Efforts can give rise to the large-scale studies needed to define the
prognosis and optimal treatment of individual forms of coronary artery
abnormality.
Conclusion :-Coronary artery anomalies should be regarded as an uneven
diverse group of congenital disorders whose manifestations and
pathophysiological mechanisms are highly variable. This is a case report
of rare coronary artery anomaly with interarterial course, without
evidence of ischemic sign, in otherwise asymptomatic young boy. Watchful
observation and strenuous activity restriction was applied in our case.
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Legends:-
Figure 1: 12 lead ECG in the patient with abnormal right coronary artery
from left sinus, showing no evidence of ischemia.
Figure 2: Parasternal short axis view showing the origin of right
coronary artery from left sinus (marked by arrow). Ao: aorta, PA:
pulmonary artery
Figure 3: CT coronary angiogram showing anomalous origin of right
coronary artery from the anomalous sinus (marked by arrow). The original
expected position of the coronary artery is marked by the star. Ao:
aorta, RCA: right coronary artery.