It’s natural for you to be concerned right now about your child’s health—a diagnosis of tricuspid atresia can be overwhelming. But you can rest assured that at Boston Children's Hospital, your child is in expert hands.
Consistently ranked among the top pediatric hospitals in the United States, Boston Children’s is home to the world's most extensive pediatric hospital research enterprise; and we partner with elite health care and biotech organizations around the globe. But as specialists in innovative, family-centered care, our physicians never forget that your child is precious, and not just a patient.
Why is tricuspid atresia a concern?
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 or how little oxygen will be in the bloodstream depends on a number of variables. Some children will be only mildly cyanotic, while others won't have enough oxygen in the blood to meet the body’s needs.
How common or rare is TA?
Tricuspid atresia occurs in two out of every 10,000 live births. While relatively rare, it accounts for 1 to 2 percent of all cases of congenital heart disease, and is the third most common form of cyanotic congenital heart disease. TA occurs equally in boys and girls.
What are the forms of tricuspid atresia?
There are three major variations in tricuspid atresia, usually characterized by the size of the hole (VSD) between the right and left ventricles:
- If the VSD is large, the baby (newborn or in the first few weeks of life) might have congestive heart failure (CHF), in which the heart pumps too much blood to the lungs and has difficulty pumping enough blood to the rest of the body. If the left side of the heart fails, the lungs can get flooded with too much blood and fluid; the child breathes rapidly, sweats and has difficulty gaining weight. An initial surgical procedure called pulmonary artery banding will usually be performed in the first few weeks of life to reduce the amount of blood to the lungs.
- If the VSD is small or absent, the right ventricle will be severely underdeveloped, and, in addition, the pulmonary valve may be obstructed (pulmonary valve stenosis). As a result, not enough blood will be able to pump through to the lungs and pick up oxygen. Without sufficient oxygen, the baby’s skin will turn blue (cyanotic). This condition is usually recognized in the hospital after birth or soon after going home.
- In this case, to stabilize the baby before surgery, medication will keep the PDA open, allowing blood to pass from the aorta to the pulmonary artery so that some oxygen-rich blood can circulate. Initial surgery will usually be performed early in the baby’s life.
- If the VSD is moderate sized, the amount of blood going to the lungs is more balanced, causing less distress in the baby and reasonable levels of oxygen in the blood. Initial surgery will usually be performed when the baby is about six months old, or even older.
Will my child be OK?
Open heart surgery at Boston Children’s has among the highest success rates in the United States among large pediatric cardiac centers. With our advanced surgical techniques and timely, family-centered care, the likelihood is very good that your child will undergo successful surgery.
Note: Infants who’ve had initial surgery for a single ventricle defect are typically enrolled in Boston Children’s Home Monitoring Program between the baby’s Stage I and Stage II operations in the Fontan sequence.
What about later in life?
Your cardiologist will help you create a long-term care program as your baby grows into childhood, the teen years and even adulthood. Most people who've had congenital heart disease repair will have an ongoing relationship with their cardiologist; we'll treat complications, and will advise on daily-life issues such as exercise and activity levels, nutrition and precautions related to pregnancy.
Surgical techniques for TA and its associated defects are continually being refined, and long-term outcomes are continually improving. Nevertheless, patients will need lifelong monitoring and medication, since they will always be at some risk for arrhythmias, infections, heart failure or stroke.
Where can my child find care and support when he grows up?
- The Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH) provides long-term inpatient and outpatient care and advanced therapeutic options for patients with congenital heart disease and pulmonary hypertension as they reach and progress through adulthood.
BACH is an international center for excellence, with physicians and services from Children’s, Brigham and Women’s Hospital and Beth Israel Deaconess Medical Center. The center promotes and supports clinical and scientific research for the advancement of care of these patients, and is a leader in the education of providers caring for this unique population.
- The Adult Congenital Heart Association (ACHA) is a national not-for-profit organization dedicated to improving the quality of life—and extending the life—of adults with congenital heart defects. The organization serves and supports many of the adults with congenital heart defects (thought at this time to be as many as one million), their families and the medical community, as well as conducting research and providing advocacy, outreach and education.
It’s important for parents to understand that you’ve done nothing to cause your baby’s tricuspid atresia and its accompanying defects. Some congenital heart defects may have a genetic link, causing heart problems to occur more often in certain families. Most often, though, this heart defect occurs sporadically (by chance), with no clear reason for its development.
- rapid breathing
- rapid heartbeat
- blue color of the skin, lips and nailbeds (cyanosis)
- disinterest in feeding or tiring while feeding
- poor weight gain
- heart murmur (detected by doctor)
When to seek medical advice
Call your health care provider immediately if your baby or child is having difficulty breathing or is breathing rapidly, has a bluish color, tires easily or is uninterested in eating.
Who’s at risk
Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development. So, it’s difficult to predict who’s at risk. Familial cases have been reported, but no genetic link has been confirmed. TA occurs equally in boys and girls. In general, if you have a child with a congenital cardiac defect, the chance of later children having a defect is about 2 to 3 percent.
The first children who underwent the staged surgical repairs for TA are now in their 20s and largely doing well. However, complications can occur, including arrhythmias, blood clots, infections within the heart (endocarditis), easy tiring and loss of protein from the digestive tract. As he grows, it’s important for your child to be monitored closely by his cardiologist.
Surgical techniques for TA and its associated defects are continually being refined, and long-term outcomes are continually improving. But patients will still need lifelong monitoring and medication, since they'll always be at some risk for arrhythmias, blood clots, infections, heart failure or stroke.
What you can do at home
Your child’s cardiologist will offer recommendations for post-operative follow-up care, including:
- wound care
- a nutritional program to encourage weight gain
- an oral hygiene program to prevent infection
- an appropriate exercise regimen to build body mass and achieve fitness
As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups.
It’s important to understand that as parents, you’ve done nothing to cause TA and its accompanying defects, and there are no precautions you could have taken to prevent it. Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development.
- aorta: one of the heart’s two great arteries. In a normal heart, the aorta arises from the left ventricle and carries oxygen-rich blood out to the body.
- atrial septal defect (ASD): a congenital (present at birth) heart defect that is present in all children with TA, in which there's an opening in the tissue wall (septum) that divides the two upper chambers of the heart (right and left atria)
- arterial switch procedure: a surgical procedure necessary if the aorta and pulmonary artery are reversed in relation to each other (transposition of the great arteries [TGA]). In TGA, the aorta and pulmonary artery are divided and “switched” so that the blue blood goes out the pulmonary artery and the red blood goes out the aorta.
- bi-directional Glenn: surgical procedure that frequently replaces the Blalock-Taussig shunt with another connection to the pulmonary artery to provide a path for blue blood to go out to the lungs. The superior vena cava is surgically connected to the right pulmonary artery to direct blood from the upper part of the body to the lungs to receive oxygen. This is the second procedure of the Fontan Sequence.
- Blalock-Taussig shunt: a surgical procedure to create a pathway for blood to reach the lungs. A shunt (tube) may be inserted between the aorta or one of its branches and the pulmonary artery to increase blood flow. This is frequently the first procedure of the Fontan Sequence.
- cardiac catheterization: an invasive diagnostic procedure performed under sedation to obtain detailed visual information and measurements about the structures inside the baby’s heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta.
- cardiac/cardio-: pertaining to the heart
- cardiac magnetic resonance imaging (MRI): a non-invasive diagnostic tool using 3-D imaging technology produced by magnets to accurately determine the blood flow and functioning of your child's heart
- cardiac surgery: any surgical procedure performed on the heart or one of the blood vessels connected to the heart
- cardiac surgeon: doctor who performs surgery on the heart. A pediatric cardiac surgeon performs surgery on the hearts of infants and children.
- cardiologist: doctor who diagnoses and treats heart problems non-surgically. A pediatric cardiologist treats infants, children and some adults with heart problems.
- The Center for Families at Boston Children’s: dedicated to helping families find the information, services and resources they need to understand their child’s medical condition and take part in their care
- chest x-ray: a diagnostic tool chest to evaluate the size and spatial relationships of the heart within the child’s chest, as well as the presence of TA and its associated defects
- cyanosis (cyanotic): blue color of skin, lips and nailbeds caused by a reduction in the amount of oxygen-rich (red) blood circulating in baby’s bloodstream
- congenital heart defect: heart defect present at birth. The heart usually starts to form in the first eight weeks of fetal development. It is thought that most congenital heart defects develop during this period.
- diagnosis: medical determination of illness or disease based on history, physical examinations and advanced technology diagnostic testing tools
- echocardiogram (echo, cardiac ultrasound): a diagnostic tool that evaluates the structure and function of the heart using sound waves that produce a moving picture of your child’s heart and heart valves. The ultrasound can be used to understand flow in the different chambers and to estimate pressures.
- electrocardiogram (ECG, EKG): a diagnostic tool that evaluates the electrical activity of your child’s heart. An EKG is usually the initial test for evaluating the causes of symptoms and detecting heart abnormalities, including TA.
- Fontan sequence: a series of three operations performed on children who have just one full-sized functioning ventricle (a type of single ventricle defect). The Fontan operations are usually performed at intervals starting within days or months after birth and ending at a few years of age. The Fontan operation is the third procedure in the Fontan Sequence.
- pulmonary artery: one of the heart’s two great arteries, which normally arises from the right ventricle and carries oxygen-poor blood to the lungs, where it receives oxygen
- pulmonary artery banding (PAB): a repair in which a band is secured around the pulmonary artery to limit blood flow to the lungs
- pulmonary valve stenosis (PVS, PS): a congenital (present at birth) heart defect sometimes associated with TA in which the pulmonary valve is unable to open completely, making it harder for blood to flow from the right ventricle to the lungs
- shunt: an artificial connection of blood vessels in order to redirect blood to the lungs in children with inadequate flow for oxygenation
- single ventricle defect (SVD): one of several congenital (present at birth) heart defects in which the heart has only one fully functioning ventricle. TA is considered a single ventricle defect.
- sporadic: occurring by chance, occasionally, not inherited
- symptoms: the presenting reasons why a child needs medical attention. A symptom’s characteristics—such as onset, quality, triggers and severity—help diagnosticians to determine a disease, or to decide which testing is needed to determine the disease.
- transposition of the great arteries (TGA): an uncommon defect associated with TA, in which the aorta and pulmonary artery are reversed in relation to each other. In TGA, the aorta comes off the right ventricle and the pulmonary artery arises from the left ventricle.
- tricuspid atresia: a rare congenital (present at birth) heart defect in which the tricuspid valve is absent or blocked off, resulting in a small or absent right ventricle that cannot adequately pump blood to the lungs.
- ventricular septal defect (VSD): a congenital (present at birth) heart defect associated with TA in which there's an opening in the tissue wall (septum) that divides the two lower chambers of the heart (right and left ventricles)
For in-depth visual information on several of the conditions, diagnostic tools and procedures described above, visit our Multimedia library.