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My Child Has:
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Tricuspid Atresia
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Tricuspid atresia
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Tricuspid atresia (TA) is a congenital (present at birth) heart defect that occurs when the tricuspid valve, normally located between the right atrium and the right ventricle, does not develop properly during pregnancy.
Normally:
- oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs where it receives oxygen.
- Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle, then is pumped through the aorta out to the body.
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Normal heart
(click to enlarge) |
In tricuspid atresia, the following occurs:
- Improper development of the tricuspid valve prevents oxygen-poor (blue) blood from passing from the right atrium to the right ventricle and on to the lungs as it should.
- The right ventricle is underdeveloped.
- Openings are present in the atrial and ventricular walls (called atrial septal defect and ventricular septal defect), allowing oxygen-poor (blue) blood and oxygen-rich (red) blood to mix.
- A patent ductus arteriosus allows blood to pass through from the aorta to the pulmonary artery and receive oxygen from the lungs.
Tricuspid atresia occurs in two out of every 10,000 live births. It makes up 1 to 2 percent of all cases of congenital heart disease. TA occurs equally in boys and girls.
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The heart is forming during the first eight weeks of fetal development. In TA, during the middle of this development period, the tricuspid valve does not develop properly. Ventricular development is influenced by blood flowing through it, and since no blood is able to pass through the tricuspid valve, the right ventricle remains small.
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Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality or environmental exposure, causing heart problems to occur more often in certain families. Most of the time, this heart defect occurs sporadically (by chance), with no clear reason for its development
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This heart defect causes children to be cyanotic (blue) since a mixture of oxygen-poor (blue) and oxygen-rich (red) blood leaves the heart and goes to the body. Just how much oxygen or how little oxygen will be in the bloodstream depends on a number of factors. Some children will only be mildly cyanotic, while others will not have enough oxygen in the blood to meet the body's needs.
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Symptoms are noted shortly after birth. The following are the most common symptoms of tricuspid atresia. Each child may experience symptoms differently. Symptoms may include:
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- blue color of the skin, lips and nailbeds (cyanosis)
- rapid breathing
- labored breathing
- rapid heart rate
- cool, clammy skin
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The symptoms of TA may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.
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A pediatric cardiologist and/or a neonatologist may be involved in your child's care. A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. A neonatologist specializes in illnesses affecting newborns, both premature and full-term.
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Cyanosis is the major indication that there is a problem with your newborn. Your child's physician may have also heard a heart murmur during a physical examination. A heart murmur is simply a noise caused by the turbulence of blood flowing through the openings that allow the blood to mix.
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Other diagnostic tests are needed to help with the diagnosis, and may include the following:
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- Chest X-ray — A diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs onto film.
- Electrocardiogram (ECG or EKG) — A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias) and detects heart muscle stress.
- Echocardiogram (echo) — A procedure that evaluates the structure and function of the heart by using sound waves, recorded on an electronic sensor, that produce a moving picture of the heart and heart valves.
- Cardiac Catheterization — A procedure that gives very detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the inside of the heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as in the pulmonary artery and aorta. Contrast dye is injected to more clearly visualize the structures inside the heart.
- Cardiac Magnetic Resonance Imaging (MRI) — A non-invasive test that uses three-dimensional imaging technology produced by magnets to accurately determine blood flow and functioning of the heart as it is working.
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Specific treatment for TA will be determined by your child's physician based on:
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- your child's age, overall health and medical history
- extent of the disease
- your child's tolerance for specific medications, procedures or therapies
- how your child's doctor expects the disease to progress
- your opinion or preference
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Your child most likely will be admitted to the intensive care unit (ICU) or special care nursery once symptoms are noted. Initially, your child may be placed on oxygen or a ventilator to assist his/her breathing. Intravenous (IV) medications may be given to help the heart and lungs function more efficiently.
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Other important aspects of initial treatment include the following:
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- Cardiac Catheterization — A procedure that can be used as a diagnostic procedure, as well as an initial treatment procedure for some heart defects. A cardiac catheterization procedure will usually be performed to evaluate the defect(s) and the amount of blood that is mixing.
As part of the cardiac catheterization, a procedure called a balloon atrial septostomy may be performed to improve mixing oxygen-rich (red) blood and oxygen-poor (blue) blood.
- A special catheter with a balloon in the tip is used to create or enlarge an opening in the atrial septum (wall between the left and right atria). The catheter is guided through the foramen ovale (a small opening present in the atrial septum that closes shortly after birth) and into the left atrium. The balloon is inflated.
- The catheter is quickly pulled back through the hole, into the right atrium, enlarging the hole, allowing blood to mix between the atria.
- Medication — An intravenous (IV) medication called prostaglandin E1 is given to keep the ductus arteriosus from closing.
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The ductus arteriosus (the normal connection between the aorta and the pulmonary valve) will likely close if the prostaglandin E1 infusion is stopped. Another pathway for blood to reach the lungs to receive oxygen must be surgically created.
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- Surgical Repair -- A series of operations are performed in the first two years of life that will re-route blood so that enough oxygen is added to the bloodstream to meet the child's needs. Each operation is performed under general anesthesia. Types of operations include the following:
- Blalock-Taussig Shunt -- The first operation creates a pathway for blood to reach the lungs. A connection is made between the first artery that branches off the aorta (called the right subclavian artery) and the right pulmonary artery. Some of the blood traveling through the aorta towards the body will "shunt" through this connection and flow into the pulmonary artery to receive oxygen. However, the child will still have some degree of cyanosis since oxygen-poor (blue) blood from the right atrium and oxygen-rich (red) blood from the left side of the heart mix and flow through the aorta to the body.
- Glenn Shunt -- A second operation, often performed at about 4 to 12 months of age, replaces the Blalock-Taussig shunt with another connection to the pulmonary artery. In this operation, the Blalock-Taussig shunt is removed, and the superior vena cava (the large vein that brings oxygen-poor blood from the head and arms back to the heart) is connected to the right pulmonary artery. Blood from the head and arms passively flows into the pulmonary artery and proceeds to the lungs to receive oxygen. Oxygen-poor (blue) blood returning to the heart from the lower body through the inferior vena cava will still mix with oxygen-rich (red) blood in the left heart and travel to the body, so the child will remain mildly cyanotic. This operation helps create some of the connections necessary for the final operation, the Fontan procedure.
- Fontan Procedure -- The Fontan procedure is for treatment of children with single ventricle. The basic principle of the Fontan operation is that it is not necessary to have a ventricle to pump to the lungs as long as the lung arteries have a low resistance, are well developed and are of a good size, and as long as the single ventricle fills at a low pressure. The surgery involves a direct connection of the blue blood returning to the heart into the pulmonary arteries. This can be achieved in a number of different ways, sometimes with and sometimes without the use of synthetic tubes (conduits). Many centers prefer to place a small hole (fenestration), which prevents a buildup of excessive pressure within the veins of the body when the resistance in the lungs is high. This will result in a certain degree of cyanosis (blueness). After the child has adapted to the Fontan circulation, it may be possible to close the fenestration in the catheterization laboratory with a device delivered by catheter.
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Infants will remain cyanotic after the first two operations and until the final operation (Fontan procedure) is performed. Your child likely will grow and develop more slowly than the average baby because of the lower amounts of oxygen available for the body's needs. Following the Fontan procedure, when oxygen levels improve, many children will see major improvements in growth and development, and can eventually catch up to normal children.
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After each operation, your infant will need to be followed by a pediatric cardiologist who will make adjustments to medications, assist you with feeding problems, measure oxygen levels and determine when it is time for the next operation
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Consult with your child's physician regarding the specific outlook for your child.
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Complementing the Cardiovascular Program Children's Hospital Boston is the nation's most intensive clinical and basic research program focused on pediatric heart disease. Children's is a world leader in opening new avenues of "translational research," bringing laboratory advances to the bedside and doctor's office as quickly as possible. All senior medical staff members of the Cardiovascular Program participate in clinical research activities.
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Children's Hospital Boston has pioneered interventional catheterization repair of many types of congenital heart defects, including initial repair of tricuspid atresia.
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Contact Children's Hospital Boston Cardiovascular Program physicians for a second opinion.
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