The CHD facts that is. I thought I would take this opportunity during CHD Awareness week to give you the facts about Claire’s CHD – Tetralogy of Fallot with Right Aortic Arch. A lot of the information I am getting ready to share with you, you may already have figured out from reading Claire’s blog. But since she was diagnosed when I was about 23 weeks pregnant, we were not blogging then and I am not sure that I have really written a blog strictly explaining the ins and outs of Claire’s CHD. So here we go! (All of the facts, pictures, and videos are taken from the Children’s Hospital of Philadelphia’s website. I copied and pasted a lot to make it easier, but didn’t want to quote and cite the whole entire post.)
What is Tetralogy of Fallot?
This condition has four characteristics:
· Ventricular septal defect (VSD) - There is a hole between the two bottom chambers (the ventricles) of the heart that eject blood to the body and lungs.
· Overriding aorta - The aorta, the large artery that takes blood to the body, is on top of both ventricles, instead of just the left ventricle as in a normal heart.
· Pulmonary stenosis - There is a narrowing of the pulmonary valve, the area below the valve, or the pulmonary arteries, which carry blood from the heart to the lungs.
· Hypertrophy - The right ventricle becomes thicker and more muscular than normal as a result of working harder to pump blood through the narrow pulmonary valve.
Also, below is a picture of a “normal” heart and a heart with ToF.
Claire’s heart is even more different that the one shown above with ToF. Claire also has a Right Aortic Arch. You can see in the picture of the normal heart and the ToF heart how the aorta comes out of the heart and turns towards the left (well, on the picture it’s to the right but if you turn the heart around like it is in your body, then your aorta turns towards your left arm and then goes down into your body. Well, Claire’s aorta comes up out of her heart and then turns towards her right arm and then down into her body like the picture below. So the picture below is a heart with ToF and a right aortic arch – Just like Claire’s. The surgeon had told us that this was a “good” thing because he didn’t have to work around Claire’s aorta to get to her pulmonary artery during surgery.
The symptoms of Tetralogy of Fallot include:
- blue or purple tint to lips, skin and nails (cyanosis)
- heart murmur - the heart sounds abnormal when a doctor listens with a stethoscope
- in older children, abnormal shape of the fingertips ("clubbing")
- spells during which oxygen levels drop - lips and skin will become bluer, and the child will become fussy or irritable and then sleepy or unresponsive
Fortunately, Claire never showed the cyanosis symptom and did not have a tet spell before her surgery. Claire did and still does have a heart murmur. She fortunately does not have the clubbing either.Tetralogy of Fallot may be diagnosed with fetal echocardiogram (ultrasound). This is how Claire’s ToF was originally detected when I was 23 weeks pregnant.
Doctors might make the TOF diagnosis before the newborn leaves the hospital if they hear a murmur or see a blue tint to the skin; a primary care pediatrician might detect the same symptoms during a checkup; or a parent might notice symptoms and bring the baby to a doctor or hospital.
Diagnosis may require some or all of these tests:
- pulse oximetry - a painless way to monitor the oxygen content of the blood
- electrocardiogram (ECG) - a record of the electrical activity of the heart
- echocardiogram (also called "echo" or ultrasound) - sound waves create an image of the heart
- chest X-ray
- cardiac MRI - a three-dimensional image shows the heart's abnormalities
- cardiac catheterization - a thin tube (catheter) is inserted into the heart through a large vein in the leg
The cardiologists were concerned before Claire was born that she could have DiGeorge Syndrome since ToF with the RAA is usually a sign of DiGeorge. But they tested Claire after she was born and she tested negative for it.
Surgery is required to repair tetralogy of Fallot.
In the first few months of life surgeons will perform open-heart surgery to patch the hole (VSD) and widen the pulmonary valve or artery. In some cases, depending on the unique needs of the patient, doctors will perform a temporary repair until a complete repair can be done. The temporary repair involves connecting the pulmonary arteries (which carry blood from heart to lungs) with one of the large arteries that carry blood away from the heart to the body. This increases the amount of blood that reaches the lungs, and so increases the amount of oxygen in the blood.
After surgery, your child will initially recover in the Pediatric Cardiac ICU or Pediatric ICU as he or she improves.
Here is another short You Tube video showing how doctors surgically repair a heart with ToF (click HERE.)
Claire’s VSD patch was not the Gore-tex patch that they talk about, but rather a bovine patch (cow skin patch). Also, Claire had a valve sparing surgery. This means that the surgeon was able to remove some of the extra muscle tissue from in front of the pulmonary valve and he also placed a patch on the top part of the artery to widen it. But he did not have to widen the valve part of her PA (the area with the little flaps that prevent blood from flowing back into the heart). This was a great thing as this valve area is usually the part that leaks and has to be replaced.
HERE is a link to a short video about the leaky valve.
The surgeon did have to trim Claire’s valve “curtains” as they were sort of stuck together (like they show in the video). So by trimming the curtains, it does allow some leakage but not a lot. At her last cardiology appointment, the doctor said she had “moderate leakage.” They will watch this and as she grows, she may have to have another heart surgery for valve replacement.
As Claire gets older, we will have to watch her energy level to see if she is showing any symptoms of needing a valve replacement. If she becomes tired all the time or gives out of energy quicker than normal or than other children, we would need to have her heart checked.
Through the age of 18, a child who has had surgical repair of Tetralogy of Fallot will require life-long care by a cardiologist.
Pediatric cardiologists follow patients until they are young adults, coordinating care with the primary care physician. Patients will need to carefully follow doctors' advice, including staying on any medications prescribed and, in some cases, limiting exercise. I have even read in some cases that children with CHDs like Claire’s will have to take antibiotics before every visit to the dentist – even for regular cleanings. I think this is because if an infection gets in their mouths it can get into their bloodstream very quickly through their mouths and go straight to their hearts and that could be very hard for them to fight off.
Sometimes children with this condition experience heart problems later in life, including a leaky heart valve and irregular heartbeat (arrhythmia). Medicine or repeat surgery may be required.
Once a child is over 18 years old, they will transition over from a pediatric cardiologist to an adult cardiologist and will have to be followed every year for the rest of their lives.
Because of enormous strides in medicine and technology, today most children with heart conditions like Tetralogy of Fallot go on to lead healthy, productive lives as adults.
So I hope that helps explain Claire's special heart!
The Scott Family