Congenital heart defect corrective surgeries
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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
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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.
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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.
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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.
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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