So Ruby is doing awesome. It’s obvious in her energy and her spunk ;). She’s fiesty and full of life. And we couldn’t be happier and we acknowledge God’s hand in her recovery and how well she is doing now.One thing that is unfortunately always lurking in the background is that just because she had her complete repair, doesn’t mean that she is free and clear forever. She will always be seen by a cardiologist throughout her entire life (thank heavens for that!). Since her pulmonary arteries are on the smaller side, they said there is a possibility that she may someday need a procedure to balloon those arteries. But apart from those, here is what I have found.
These questions and answers were all found on the Adult Congenital Heart Association website, with the exception of one or two at the end, which were found on reputable medical sites:
If I had my ToF repaired as a child am I at risk for new heart problems?
Most adults with ToF had a “complete ToF repair” in childhood. In this repair, surgeons close the hole in the heart and open up the narrow heart valve to the lungs (the pulmonary valve). While most people who undergo this repair feel well and have few problems in childhood, this does not mean that their heart is fixed permanently. As people born with ToF age, new heart problems can develop. As adults, it’s important that they stay in the care of an adult congenital heart specialist.
What is the most common heart problem in adults with repaired ToF?
The most common problem for adults with ToF is a leaky valve between the heart and lungs (pulmonary regurgitation or PR). As part of the ToF repair, surgeons usually stretch out the valve between the heart and lungs (pulmonary valve). This almost always makes the valve leak. As people age, this leaking can get worse.
What health problems can be caused by a leaky heart valve?
A leaky valve can cause a number of serious problems. A leaky pulmonary valve can cause the right side of your heart to get bigger, which affects its pumping strength. This can lead to a loss of function in your heart and put you into heart failure. A leaky valve also increases your risk of serious rhythm problems, especially in the lower chambers of your heart (ventricular tachycardia or VT). VT is a dangerously fast heartbeat and can cause sudden death if not treated. Other rhythm problems in the upper chambers of the heart are common too. In some people with ToF, the aortic and tricuspid valves may also leak (for more details, see below).
How would I know if I have a problem with a leaky valve?
A leaky valve can be a sneaky valve! There are often few or no symptoms to alert you. The first sign can often be rhythm problems, like an unusually fast heartbeat or feeling your heart “flutter.” If your valve has been leaking a lot and for a long time, you may feel tired. It is important to see your ACHD cardiologist regularly to know how and when to treat a leaky valve.
How do doctors find and treat a leaky valve?
Your ACHD care team has a number of tools to look at your heart. Your doctor may want you to have an echocardiogram (echo), a scan that uses sound waves to look at your heart. You will also have an electrocardiogram (ECG or EKG), a test that records your heart’s electrical activity. You many also need to have an exercise stress test and/or a cardiac MRI. Some patients’ doctors may recommend having a heart catheterization. Together these tests can help your doctor decide if you need a pulmonary valve replacement (PVR) to replace your leaky pulmonary valve with a new valve.
What other health problems might I be at risk for?
If you are an adult with repaired ToF, you may be at risk for an enlarged aorta. This can cause your aortic valve to leak and require an aortic root replacement. This is a surgery to replace the stretched-out part of your aorta and/or aortic valve. Less than 10% of people with ToF may have a leaking tricuspid valve, the valve between your right atrium (receiving chamber) and your right ventricle (pumping chamber). This is known as tricuspid regurgitation or TR and can only be repaired through surgery.
Other possible health problems include heartbeats that are too fast or too slow (you can learn more in the educational sheet, “The Heart and Its Electrical System,” on the ACHA website). You may also have leaking around the area that closed the ventricular septal defect (VSD), and you may be at risk for lung problems or problems with the heart muscle. Talk to your ACHD heart doctor about what you need to look for.
It is important to see your ACHD cardiologist regularly so that any changes in your heart can be detected early. Early detection can help prevent permanent damage.
Can women with ToF have children?
Many women with ToF can have children successfully. If you are a woman with ToF and are thinking about becoming pregnant you should first talk with your ACHD cardiologist. You should also talk with an obstetrician who specializes in higher-risk pregnancies. Your ACHD care team and your obstetrician should work together. Many women can safely go through pregnancy and delivery; however, some women may need to have a valve replacement or adjust medication first. Your doctors can develop a plan to care for and protect your heart during your pregnancy and delivery.
If I have ToF, do I have a higher chance of having a baby with a heart defect or other health problems?
Yes. Most people with ToF will have a baby without any health problems. But people with heart defects do have a slightly higher chance of having a baby with a heart defect or other health problem. One genetic problem called 22q11.2 deletion syndrome has been linked to ToF. If you have ToF and are thinking about having a baby, you should be screened for this syndrome.
Do I need special care for my ToF as an adult?
Yes. The American Heart Association and the American College of Cardiology classify ToF as moderately complex heart disease. This means that you should get your care at a special ACHD center and see your ACHD cardiologist regularly, at least every two years. Regular check-ups can help detect any problems early on and prevent long-term damage to the heart.
*Although this operation is often called a ‘repair operation’ or ‘corrective surgery’, it never makes the heart completely normal.
*Even many years after apparently successful surgery, your child may develop a new problem – such as an abnormal heart rhythm – which may need treatment. An abnormal heart rhythm can usually be treated, but it can be serious, and in very rare cases can even be fatal. So, it’s important that your child has regular outpatient reviews with a specialist, even if he or she appears well.
*Everyone who has Tetralogy of Fallot is at risk of getting infective endocarditis, both before and after surgery or treatment. Infective endocarditis is a rare condition where the inner lining of the heart,
most commonly one of the heart valves, becomes infected. Infective endocarditis is a serious condition which can be life-threatening if it’s not treated quickly. Nowadays, if it is diagnosed early, most people with it will recover well with antibiotic treatment, although some damage may occur to the heart valves as a result of the infection. Endocarditis is caused by a bacterium, or (rarely) another type of infective organism that is in the bloodstream, which settles onto the abnormal structure or defect in the heart. Although it is not possible to prevent all bacteria from getting into the bloodstream, there are some things your child can do, as he or she grows up, to reduce the risk of getting endocarditis:
• Maintain good oral hygiene and have regular check-ups with a dentist
• Avoid body piercing and tattooing
• Never inject recreational drugs
*If you have a daughter with congenital heart disease, you need to be aware that pregnancy could carry risks to both the mother and the baby. So, when your daughter gets older, it’s particularly important that she avoids having an unplanned pregnancy. You will need to discuss this with your daughter in whichever way you, as a parent, think is appropriate for her. If your daughter wants to have a baby, it’s best that she speaks to her cardiologist about it first, so that the pregnancy can be planned for when your daughter’s heart condition is most stable. People who have congenital heart disease themselves have an increased risk of having a child with a heart problem. This applies to both males and females. You can discuss this with your cardiologist. Early scans in pregnancy can be arranged to look for heart disease in the baby.
*If you have one child with congenital heart disease, there is about a 1 in 50 chance of having another child with congenital heart disease. However, this risk may be higher (or lower) depending on the type of congenital heart disease your child has.
Sometimes my heart sinks when I read all of that. But most of the time I feel so blessed. Ruby’s pulmonary valve was saved and so she didn’t have to have it replaced with something she will outgrow that will need replacing at a later time. I was looking at her the other night, snuggling with her as I put her to bed. Sometimes I wonder why we were so lucky to get off so easily when so many babies struggle and struggle—even within Tetralogy of Fallot. I thought back to her first echocardiogram, where they were pretty sure she would need her pulmonary valve replaced. Then I thought about how each echocardiogram after that showed the valve looking better and better, until the one right before surgery showing that they probably wouldn’t have to replace the valve. Once again (as I knew so well at surgery time) I felt so strongly as I put her to bed that God truly made a miracle happen with Ruby. She is our miracle. He performed a miracle in Ruby. You rarely hear of people with Tetralogy of Fallot having only one surgery and we are hopeful that if she does have to have more, that they will not be open heart surgery, but rather smaller procedures performed to help her out. Brings tears to my eyes, realizing how blessed we all were to witness that miracle in her.
Another statement that makes my heart flood with gratitude is on every website—without the surgery, most babies born with Tetralogy of Fallot would die before they reached adulthood. How blessed we have been—even with the advances of 20 years things are so much better for TOF babies!!! It really is so amazing. I have a friend who’s brother born with TOF (he’s probably in his early 40’s) is waiting for his second heart transplant. The advances they have made is amazing. How many babies had to die to perfect this surgery? I think that all the time. To know what medicines, to know to leave an ASD for the pressures to be released, to place things into the heart to give medicine, to drain the fluid, etc. etc. etc. I could go on and on. People are amazingly smart and absolutely inspired. And I feel so sad for those who have had to die to get this surgery where it is now. One study I read had a pool of over 200 people who had the surgery done in the 60’s. Only half of them were even alive to research and study because of heart complications after surgery or down the road a bit! And that was not very long ago. It’s crazy.
All this to say I’m so thankful. I still have a lot of concerns about her future and I don’t know that they will go away. But living a life in fear isn’t fun. I’ve been doing it off and on since she was born. And I have a hard time not imagining horrible things happening to any of my children now, not just Ruby. It’s sad to live like that. And I’m going to try my best to do better and stop living in fear of something awful happening!
Originally posted on my family blog September 29, 2014.