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Anomalous Origin of the Left
Coronary Artery from the Pulmonary Artery (ALCAPA)
What is anomalous left coronary
artery?
Anomalous left coronary artery occurs when the left coronary artery arises
from the pulmonary artery instead of its usual site of origin, the aorta. It
is a rare problem comprising <1% of congenital heart defects.

How will this problem affect my child's health?
The coronary arteries are the blood vessels that supply the heart muscle
with red or oxygen-rich blood. There are usually two large coronary arteries
that arise from the aorta, one from the right side (1) and one from the left
side (2). Sometimes, for unknown reasons, when the heart and blood vessels
are forming, the left coronary artery (3) arises from the pulmonary artery
(4). Right after birth, the pressure is the pulmonary artery is high so that
enough blood flow through this vessel to supply the heart muscle with
oxygen. Over the first two months of life, the pressure in the pulmonary
artery drops and so does the blood flow through this vessel.
The decreased amount of oxygen-rich blood leads to damage to the heart
muscle cells. As a result, the heart is not able to pump as much blood as
the body needs. This causes symptoms of congestive heart failure such as
poor feeding, slow growth, clammy sweating, and poor growth. Often these
symptoms are seen between 2 and 6 months of age, but they can occur during
early infancy or rarely, during later childhood.

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Normal Right Coronary Artery
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Normal Left Coronary Artery
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Anomalous Left Coronary Artery
-
Pulmonary Artery
How is this problem diagnosed?
Symptoms: As described above, the onset of symptoms of congestive
heart failure most often occurs within the first 6 months of life. Possible
symptoms include irritability, lethargy, rapid breathing, clammy sweating,
poor feeding and slow growth.
Physical findings: The physical findings of congestive heart
failure in children include slow weight gain, rapid breathing and heart
rates, and enlarged liver. In older children, there may also be swelling
around the eyes and/or feet. Often a heart murmur is present.
Medical tests: The suspected diagnosis is usually made by an
echocardiogram. Sometimes, a heart catheterization may is needed to confirm
the diagnosis. The electrocardiogram is usually abnormal. On chest x-ray,
the heart is usually quite enlarged. An oxygen saturation test is used to
measure the blood oxygen levels.
How is the problem treated?
ALCAPA is a serious problem and requires surgery as soon as possible after
the diagnosis is made. The aim of surgery is to connect the Anomalous left
coronary artery with the aorta. The precise surgery depends on the exact
location of the Anomalous left coronary artery. Sometimes, it can be moved,
along with a button of tissue, from the pulmonary artery and sewn into the
aorta. If the Anomalous left coronary arteryis located too far away from the
aorta to move, a "tunnel" is made from the aorta to the Anomalous left
coronary artery
What are the long-term health issues for these children?
The long-term health effects reflect the degree of damage to the left
ventricle. If the problem was found early, before much damage occurred, and
the surgery was successful, there are few long-term health effects. If the
left ventricle was damaged, there may be ongoing symptoms of congestive
heart failure. As a result, the child may have low stamina, and may need to
be restricted from sports. The symptoms are often treated with medicines
such as digoxin, diuretics, blood pressure lowering medicines, and/or blood
thinners. If the damage if very severe, a heart transplant may be needed.
SBE prophylaxis: Children with ALCA are at increased risk for
subacute bacterial endocarditis (SBE). This is an infection of the heart
caused by bacteria in the blood stream. Children with heart defects are more
prone to this problem because of the altered flow of blood through the heart
and/or abnormalities of the valves. SBE can occur after dental work or
medical procedures on the GI or respiratory tract because these procedures
almost always result in some bacteria entering the blood. The problem can
usually be prevented by taking an antibiotic before these procedures.
Exercise guidelines: An individual exercise program is best planned
with the doctor so that all factors can be included. In children with ALCA,
sports guidelines are based on the degree of heart damage. If there is some
damage, children are usually restricted from vigorous or competitive sports
but can participate in recreational sports. It is important for them to be
able to self-limit their activity, that is, to rest whenever they feel the
need to do so. The children can usually participate in gym class but should
be allowed to self-limit their level of exertion and they should not be
graded (which could increase the pressure to exceed their natural limits).


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part of this website may be reproduced without the consent of Variety Children's
Heart Centre.
This
website designed, created and maintained by Tony Garcia RN.
Comments
and / or Suggestions tgarcia@hsc.mb.ca
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