Congenital heart defect corrective surgeries
Definition
Congenital heart defect
corrective surgeries fix or treat heart defects that a child is
born with. A baby born with heart defects has congenital heart disease. Surgery is needed
if the defects are dangerous to the child’s health or
well-being.
Alternative Names
Congenital heart surgery; Patent
ductus arteriosus ligation; Hypoplastic left heart repair;
Tetralogy of Fallot repair; Coarctation of the aorta repair; Atrial
septal defect repair; Ventricular septal defect repair; Truncus
arteriosus repair; Total anomalous pulmonary artery correction;
Transposition of great vessels repair; Tricuspid atresia repair;
VSD repair; ASD repair; PDA ligation
Description
The surgeries described below are
done to correct many different heart defects in
children.
For more information about risks,
how to prepare for surgery, and descriptions of open-heart and
closed-heart surgery techniques, see: Pediatric heart
surgery.
Patent
ductus arteriosus (PDA) ligation
- Before birth, there is a natural
blood vessel between the aorta (the main artery to the body) and
the pulmonary artery (the main artery to the lungs) called the
ductus arteriosus. This opening usually closes shortly after birth.
A PDA occurs when this opening fails to close.
- Sometimes a simple surgery can
be done. In this procedure, the surgeon inserts a few small tubes
into an artery in the leg and passes them up to the heart. Then, a
small metal coil or another device is put into the child’s
arteriosus artery. The coil or other device blocks the blood flow,
and this corrects the problem.
- Another method is to make a
small cut, or incision, on the left side of the chest. The surgeon
reaches in and ties off the ductus arteriosus, or divides and cuts
it. Tying off the ductus arteriosus is called ligation.
Coarctation of the aorta repair
- Coarctation of the aorta occurs
when a part of the aorta has a very narrow section, like in an
hourglass timer. To repair this defect, an incision is usually made
on the left side of the chest, between the ribs.
- The most common way to repair
this is to cut the narrow section and make it bigger with a patch
made of Gore-tex, a man-made material.
- Another way to repair this
problem is to remove the narrow section of the aorta and stitch the
remaining ends together. This can usually be done in older
children.
- A third way to repair this
problem is called a subclavian flap. First, an incision is made in
the narrow portion of the aorta. Then, a patch is taken from the
left subclavian artery (the artery to the arm) to enlarge the
narrow section of the aorta.
- A fourth way to repair the
problem is to connect a tube to the normal sections of the aorta,
on either side of the narrow section. Blood flows through the tube
and bypasses the narrow section.
Atrial
septal defect (ASD) repair
- The atrial septum is the wall
between the left and right atria (upper chambers) of the heart.
There is a natural opening before birth that usually closes on its
own when a baby is born. When the flap does not close, the child
has an ASD.
- Sometimes ASDs can be closed
without open-heart surgery. First, the surgeon makes a tiny cut in
the groin. Then the surgeon inserts tubes into a blood vessel that
go into the heart. Next, 2 small umbrella-shaped "clamshell"
devices are placed on the right and left sides of the septum. These
2 devices are attached to each other. This closes the hole in the
heart. Not all medical centers do this procedure.
- Open-heart surgery may also be
done to repair ASD. Using open-heart surgery, the septum can be
closed using stitches, or sutures. Another way is to cover the
septum with a patch made of membrane or a man-made
material.
Ventricular septal defect (VSD)
repair
- The ventricular septum is the
wall between the left and right ventricles (lower chambers) of the
heart. A hole in the ventricular septum is called a
VSD.
- By age 8 for most children with
this problem, small VSDs often close on their own and do not need
surgery. This depends on where the hole is.
- Larger VSDs, small ones in
certain parts of the ventricular septum, or ones that cause heart
failure or endocarditis (inflammation) need open-heart surgery.
They also require placing a man-made patch over the hole to cover
it.
- Some septal defects can be
closed using heart catheterization (passing a thin tube into the
heart).
Tetralogy of Fallot repair
- Tetralogy of Fallot is a
congenital heart defect that usually includes 4 defects in the
heart.
- Open-heart surgery is needed,
and it is often done when the child is between 6 months and 2 years
of age.
- Different types of repairs are
done, depending on the defects. The ventricular septal defect is one repair, and
it is described above. The pulmonary valve is opened and the
thickened muscle (stenosis) is removed. A patch may be placed on
the right ventricle and main pulmonary artery to improve blood flow
to the lungs.
- The child may have a shunt
procedure done first. A shunt moves blood from one area to another.
This is done if the open-heart surgery needs to be delayed. A shunt
procedure requires making a cut between two of the
ribs.
Transposition of the great vessels
repair
- In a normal heart, the aorta
comes from the left side of the heart, and the pulmonary artery
comes from the right side. Transposition of the great vessels is
when these arteries come from the opposite sides of the
heart.
- Correcting transposition of the
great vessels requires open-heart surgery. If possible, this
surgery is done shortly after birth.
- The most common repair is an
arterial switch. The aorta and pulmonary artery are divided. The
pulmonary artery is connected to the right ventricle, where it
belongs. Then, the aorta and coronary arteries are connected to the
left ventricle, where they belong.
Truncus arteriosus repair
- Truncus arteriosus is a rare
condition that occurs when the aorta, coronary arteries, and the
pulmonary artery all come out of one common trunk. This is a very
complex defect, and it requires complex open-heart surgery to
repair it.
- Repair is generally done in the
first few days or weeks of the child’s life. The pulmonary arteries
are separated from the aortic trunk, and any defects are patched.
Usually, there is also a ventricular septal defect, and that is
patched. A connection is then placed between the right ventricle
and the pulmonary arteries.
- Many children need 1 or 2 more
surgeries as they grow.
Tricuspid atresia repair
- The tricuspid valve is the valve
between the upper and lower chambers on the right side of the
heart. Tricuspid atresia occurs when this valve is missing. To get
to the lungs, blood must cross an atrial septal defect (ASD),
ventricular septal defect (VSD), or a patent ductus artery (PDA).
(These conditions are described above.) This severely restricts
blood flow to the lungs.
- Other defects may exist with
tricuspid atresia. A medicine called prostaglandin E may be given
for a patent ductus arteriosus to
maintain it as an alternate channel to the lungs until surgery can
be done.
- A series of shunts and surgeries
may be necessary to correct this defect. The goal of this surgery
is to allow blood from the body to flow into the lungs, and blood
from the lungs to be pumped to the rest of the body through the
left ventricle.
Total
anomalous pulmonary venous return (TAPVR) correction
- TAPVR occurs when the pulmonary
veins bring oxygen-rich blood from the lungs back to the right side
of the heart, instead of to the left side of the heart, where it
should be.
- This condition requires surgery
to correct it. When the surgery is done will depend on how sick the
baby is. The surgery may be done in the newborn period if the
infant has severe symptoms. If it is not done right after birth, it
is done in the first 6 months of the baby’s life.
- TAPVR repair requires an
open-heart surgery. The pulmonary veins are attached to the left
side of the heart, where they belong, and any abnormal connections
are closed.
- If a PDA is present, it is tied off and
divided.
Hypoplastic left heart repair
- This is a very severe heart
defect that results from a severely underdeveloped left heart. If
it is not treated, it causes death in most babies who are born with
it. Operations to treat this defect are done at specialized medical
centers. Usually surgery corrects this defect. A series of 3 heart
operations is usually needed.
- The first operation is done in
the first week of the baby’s life. This is a complicated surgery
where one blood vessel is formed from the pulmonary artery and the
aorta. This new vessel carries blood to the lungs and the rest of
the body.
- The second operation is usually
done when the baby is 4 to 6 months old.
- The third operation is done a
year after the second operation.
- A heart transplant may be done to treat this
condition. But, finding a donor heart for an infant is very
difficult. Infant heart transplants can be done only ata few
medical centers.
References
Recommendations for preparing
children and adolescents for invasive cardiac procedures: A
statement from the American Heart Association Pediatric Nursing
Subcommittee of the Council on Cardiovascular Nursing in
collaboration with the Council on Cardiovascular Diseases of the
Young. Circulation. 2003;108:2250-2564.
Webb GD, Smallhorn JF, Therrien
J, Redington AN. Congenital heart disease. In: Zipes DP, Libby P,
Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook
of Cardiovascular Medicine, 8th ed. St. Louis, Mo; WB Saunders;
2007:chap 61.
Review Date: 11/21/2008
Reviewed By: Neil K. Kaneshiro, MD, MHA, Clinical Assistant
Professor of Pediatrics, University of Washington School of
Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director,
A.D.A.M., Inc.
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