Tapvr

Total Anomalous Pulmonary Venous Return (TAPVR) Explained

TAPVR is a rare birth defect of the heart. It's a type of congenital heart disease, meaning it's present from birth. Sometimes it's called total anomalous pulmonary venous connection (TAPVC). In a healthy heart, blood carrying oxygen from the lungs travels through special vessels called pulmonary veins to the left upper heart chamber (left atrium). This oxygen-rich blood then moves to the rest of the body.

However, in TAPVR, something goes wrong with these connections. Instead of flowing to the left atrium, the pulmonary veins connect to the right upper heart chamber (right atrium). This is a problem because oxygen-rich blood mixes with oxygen-poor blood that's already in the right atrium. This mixture means the blood flowing to the rest of the body has less oxygen than it should, which can cause problems.

The exact location where the pulmonary veins connect incorrectly determines the specific type of TAPVR. Importantly, most children with TAPVR don't have a family history of similar heart problems.

In short, TAPVR is a condition where the blood vessels carrying oxygen-rich blood from the lungs connect to the wrong part of the heart, leading to a mixing of oxygenated and deoxygenated blood. This mix results in the body receiving less oxygen-rich blood, which needs to be corrected by medical professionals.

Babies with TAPVR (Total Anomalous Pulmonary Venous Return) sometimes have trouble breathing. Their skin might look gray or blue, a condition called cyanosis, because their blood isn't carrying enough oxygen.

Doctors might notice these symptoms soon after the baby is born, but in some cases, the problems don't appear until later.

To check for TAPVR, a doctor will do a physical exam and listen to the baby's heart with a stethoscope. A whooshing sound, called a heart murmur, could be a sign.

A special test called an echocardiogram is crucial for diagnosing TAPVR. This test uses sound waves to create a moving picture of the heart. The picture shows the pulmonary veins (the blood vessels that carry blood from the lungs to the heart), any holes in the heart, and the size of the heart's chambers. It also shows how blood flows through the heart and the heart valves. This helps doctors see exactly how the blood is circulating and identify the problem.

Other tests, such as an electrocardiogram (ECG) to check heart rhythm, a chest X-ray to see the overall structure of the chest, or a CT scan for detailed images, might also be needed for a complete picture of the child's condition.

Surgery is usually necessary for TAPVR when the baby is young. The timing of the surgery depends on whether there's a blockage or other issues that need immediate attention. During the surgery, doctors connect the pulmonary veins to the left upper chamber of the heart. They also fix any holes between the upper heart chambers.

People who have had TAPVR surgery need regular checkups throughout their lives. These checkups will monitor for infections, blockages, or problems with the heart's rhythm. It's essential for these people to be seen by a doctor specializing in congenital heart conditions, called a congenital cardiologist. This specialized care ensures ongoing monitoring and management of the condition.

Congenital Heart Defects in Children: A Guide for Families

Congenital heart defects (CHDs) are problems with the heart's structure that are present at birth. Some CHDs, like small holes in the heart or mild valve issues, might only need regular check-ups with imaging tests like echocardiograms every few years. More serious CHDs might need surgery, either through a traditional open-heart procedure or a less invasive method using tools in a cardiac catheterization lab. In extremely severe cases, a heart transplant might be necessary.

Recognizing Symptoms

The signs of a CHD can vary greatly depending on the child's age. Infants often show symptoms during feeding, like shortness of breath, difficulty breathing, or sweating. Younger children might experience nausea, vomiting, or discomfort during and after eating or physical activity. Older children and teenagers might have chest pain, fainting (syncope), or a racing heartbeat (palpitations), especially during exercise. These are all important warning signs that a child needs to be evaluated by a doctor, particularly if they occur with physical activity.

Questions to Ask Your Doctor

After a CHD diagnosis, it's normal to have many questions. It's crucial to ask your doctor about the future plan for your child. Discuss:

• Future procedures: What surgeries or other treatments might be needed in the next few years?
• Testing and follow-up: What types of tests and doctor visits will be needed?
• Impact on daily activities: How will this affect your child's ability to participate in sports, school activities, and other daily routines?
• Long-term outlook: How can you work with the medical team to help your child live as normal a life as possible despite the CHD?

Surgery Timing

If surgery is required, discuss with your doctor the optimal timing for the procedure. Different CHDs are best treated at specific times to maximize short-term and long-term outcomes.

Sports and Physical Activity

Many parents and children ask about participating in sports. Most children with CHDs can participate in sports, but certain types of intense activity might be restricted. Some children may have CHDs that put them at higher risk of complications from strenuous exercise. Your doctor can assess your child's specific condition and advise on appropriate activities and limitations.

Heredity and Pregnancy

If a parent has a CHD, there's a small chance their child might also have one. The type of CHD could be the same or different. If a parent with a CHD becomes pregnant, close monitoring during pregnancy, including extra fetal echocardiograms, is important.

Important Note

Maintaining open communication with your child's cardiologist is essential. These patients are often followed for decades, and you should ask questions about anything that seems unclear or doesn't make sense to you. Don't hesitate to contact the team with any questions or concerns.

Diagnosing Congenital Heart Defects

Congenital heart defects can sometimes be detected during pregnancy through a standard 2D ultrasound. After birth, a healthcare professional might suspect a CHD based on:

• Growth Delays: Slowed growth can indicate a heart problem.
• Color Changes: Changes in the color of lips, tongue, or fingernails (cyanosis) can suggest low oxygen levels.
• Heart Murmurs: A sound heard with a stethoscope during a heartbeat. Not all heart murmurs are dangerous, but some indicate a CHD.

Diagnostic Tests

Several tests can help diagnose a CHD:

• Pulse Oximetry: Measures oxygen levels in the blood.
• Electrocardiogram (ECG/EKG): Records the electrical activity of the heart.
• Echocardiogram: Uses sound waves to create images of the heart and blood flow.
• Chest X-Ray: Shows the condition of the heart and lungs.
• Cardiac Catheterization: A thin tube is inserted into a blood vessel to examine the heart's structure and function. Some treatments can be performed during this procedure.
• Heart MRI: Uses magnetic fields and radio waves to create detailed images of the heart.

These tests help doctors determine the type and severity of the CHD to develop the best treatment plan.

Treating Children with Congenital Heart Defects

Congenital heart defects are problems with the heart that a child is born with. The treatment a child needs depends on the specific problem and how serious it is.

Some heart defects are minor and don't cause any long-term health issues. In these cases, no treatment might be needed.

Other defects, like a small hole in the heart, might close up naturally as the child grows older.

However, serious heart defects usually require treatment soon after diagnosis. This treatment can include:

• Medicines: These are used to manage symptoms or complications. For example, some medicines help the body get rid of extra fluid to lessen the strain on the heart. Others help regulate irregular heartbeats.

• Heart procedures: These are specialized treatments that don't involve opening the chest. One common procedure is cardiac catheterization. This uses thin, flexible tubes (catheters) to reach the heart. Doctors can use these tubes to repair some defects, such as fixing holes or narrow areas, without needing open-heart surgery. Sometimes these procedures are done in stages over time.

• Heart surgery: This is needed for more complex problems and may involve opening the chest to repair the heart. There are different types of heart surgery, including minimally invasive techniques.

• Heart transplant: If other treatments aren't successful, a heart transplant might be necessary.

• Fetal cardiac intervention: This is a very specialized treatment done before the baby is born. It's used to fix serious heart defects or prevent complications during pregnancy, but it's only used in very specific situations.

Many children with congenital heart defects need multiple procedures and surgeries throughout their lives. This is why regular checkups with a cardiologist (a doctor specializing in heart conditions) are crucial. These checkups often include tests like blood tests and imaging scans to monitor the child's heart health and look for any new problems.

Long-term care is essential for these children. This involves regular checkups with a cardiologist, along with necessary tests and procedures as needed.

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Hope and Healing for Little Hearts

[Dr. Dearani speaking] I focus a lot on minimally invasive heart surgery in children and teens. My training in adult heart surgery has allowed me to bring these advanced techniques to children's hospitals, which can be very beneficial. The availability of this more advanced technology isn't always present in all children's hospitals.

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Children with congenital heart defects might need some lifestyle adjustments to stay healthy.

Activity Levels: Some kids with congenital heart conditions might need to limit their sports or physical activities. This isn't the case for everyone. A doctor specializing in the heart (a cardiologist) can advise on which activities are safe and appropriate for your child. They'll consider the specific type of heart defect and your child's overall health.

Antibiotics for Prevention: Certain congenital heart defects can make a child more likely to get an infection in the heart lining or valves (infective endocarditis). To prevent this, some children might need antibiotics before dental work or other procedures. This is especially true for children with artificial heart valves. Always talk to your child's cardiologist about whether preventive antibiotics are necessary.

Finding Support: Connecting with others who understand what you're going through can be very helpful. Your child's healthcare team can tell you about support groups in your area. Sharing experiences with others who have similar challenges can offer comfort, encouragement, and practical advice.

Managing Stress and Anxiety: Living with a congenital heart condition can sometimes cause stress or anxiety in children. A therapist or counselor can help both you and your child develop healthy ways to cope with these feelings. Your child's doctor can provide information about therapists or counselors in your area.