Erne.qxp 2/7/09 4:02 pm Page 12
Imaging
Congenital Anomalies of the Coronary Arteries
Peiman Jamshidi and Paul Erne
Division of Cardiology, Luzerner Kantonsspital Luzern, Lucerne
Abstract
Multiple variations of congenital anomalies of the coronary arteries exist that may occur in isolation or in association with other congenital
anomalies. They can cause myocardial ischaemia. A rare but potentially lethal condition is the anomalous origin of the left coronary artery
from the pulmonary artery. The most common haemodynamically significant coronary abnormalities are coronary artery fistulae. A left-to-
right shunt exists in more than 90% of cases. The origin of the left coronary artery from the proximal right coronary artery (RCA) or the right
aortic sinus with subsequent passage between the aorta and the right ventricular outflow tract has been associated with sudden death
during or shortly after exercise in young persons. High anterior origin of the RCA is commonly encountered but is of no haemodynamic
significance. It is difficult to engage the ostium of the RCA selectively using conventional catheter manipulation. In this article we will
discuss various types of congenital coronary anomaly, providing examples.
Keywords
Congenital, coronary anomalies
Disclosure: The authors have no conflicts of interest to declare.
Received: 1 May 2009 Accepted: 21 May 2009
Correspondence: Paul Erne, Head of Cardiology, Luzerner Kontonsspital Luzern, CH-6000 Luzern 16, Switzerland. E:
paul.erne@
ksl.ch
Multiple variations of congenital anomalies of the coronary arteries rarely seen in adults. In children, the diagnosis of coronary anomalies
exist that may occur in isolation or in association with other may often be made by echocardiography with colour flow Doppler.
3–5
congenital anomalies. They can cause myocardial ischaemia. A rare About 25% survive to adolescence or adulthood but develop mitral
but potentially lethal condition is the anomalous origin of the left regurgitation, angina or congestive heart failure.
6
Aortography typically
coronary artery from the pulmonary artery. As pulmonary pressures shows a large RCA with absence of a left coronary ostium in the left
fall in newborns with this condition, myocardial perfusion becomes aortic sinus. Selective left coronary angiography is very difficult but
dependent on collaterals from the right coronary circulation.
1
could be performed with left amplatz via pulmonary artery (author‘s
experience). During the late phase of the aortogram, patulous LAD and
Coronary Artery Fistulae LCX branches fill by means of collateral circulation from RCA branches.
Fistulae arise from the right coronary artery (RCA) or its branches in A retrograde flow from the LAD and LCX opacifies the left main
about 50% of cases; the remaining fistulae arise from the left anterior coronary artery (LMCA) and its origin from the main pulmonary artery.
descending artery (LAD) or the left circumflex artery (LCX) or their The clinical course of the patient tends to be more favourable if
branches, or they have multiple origins. The fistula drains into the right extensive collateral circulation exists. In rare instances, the RCA may
ventricle in 41%, the right atrium in 26%, the pulmonary artery in 17%, arise from the pulmonary artery. The treatment is surgical, with
the left ventricle in 3% and the superior vena cava in 1%.
2
The most reimplantation of the anomalous coronary or aortocoronary bypass.
common haemodynamically significant coronary abnormalities are
coronary artery fistulae.
2
A left-to-right shunt exists in more than 90% Anomalous Origin of the Coronary Artery
of cases. About 50% of patients with a coronary artery fistula remain from the Contralateral Sinus
asymptomatic; the rest develop congestive heart failure, infective This anomaly – origin of the LCA from the proximal RCA or the right
endocarditis, myocardial ischaemia or rupture of an aneurysm.
2
aortic sinus with subsequent passage between the aorta and the right
ventricular outflow tract – has been associated with sudden death
Anomalous Origin of the Left Coronary Artery during or shortly after exercise in young persons (see Figure 1).
7–11
The
from the Pulmonary Artery increased risk of sudden death may be due to acute take-off angles of
These patients may present with ischaemic symptoms or symptoms of the aberrant coronary arteries or possible compression between the
heart failure from an ischaemic cardiomyopathy in infancy. They may pulmonary trunk and aorta. After its aberrant origin, the LCA takes an
present in the neonatal period or later in infancy or childhood. Most abrupt leftward turn and tunnels between the aorta and the right
patients in whom the origin of the left coronary artery (LCA) is the main ventricular outflow tract. Sudden death is thought to result from
pulmonary artery manifest congestive heart failure and myocardial transient occlusion of the anomalous LCA, caused by an increase in
ischaemia in the first four months of life.
2
This coronary anomaly is blood flow through the aorta and pulmonary artery that occurs during
12 © TOUCH BRIEFINGS 2009
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