What's wrong with Pierson's heart? Pierson was born with a congenital heart defect called Coarctation of the Aorta.
What is Coarctation of the Aorta? Coarctation of the Aorta is a congenital heart defect where the aorta has a narrowing usually immediatly at the heart as the aorta begins to arch. The narrowing can range from mild to severe. Pierson's was severe. The picture below is actually a picture of a section of aorta that has the coarctation. Not just any picture, mind you, this is actually Pierson's coarctation. The tiny hole in the middle is all the room oxygenated blood had to get through to get to the rest of his body. How he made it 3 years without intervention or death is a miracle.
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| Bottom Left is the actual Coarctation of the Aorta. The other pieces are portions of Pierson's Aorta that never fully developed and were weakened from the pressure the coarctation of put on the aorta. Yes, those would have burst very soon if he had not been diagnosed and received heart surgery when he did. |
What are some of the symptoms of Coarctation of the Aorta? There are several.
1. Elevated blood pressure in the arms and lower than average or lower than average blood pressure in the legs. Multi-extremity blood pressure checks would catch this every time.
2. Cold or blueish-tinted feet. The coarctation is blocking proper blood flow and hardly any oxygenated blood is reaching the bottom of their little bodies.
3. Decreased pulses in feet and groin. Again, the blood flow to the lower extremties isn't sufficient so there are either no pulses or they are very faint. After surgery you can literally see your child's pulse in their feet becaus their little bodies are so happy to be fixed.
4. Falling asleep during feedings. I wish I had know this one! Eating is exhausting for these kiddos so they fall asleep eating more times than not.
5. Not being able to keep up physically with their peers. They may get fatigued more easily than their peers or look like they are gasping for air.
6. Complaining of headaches upon exertion
7. Sweating! Oh the sweat!
8. A heart murmur -- NEVER let those go unchecked.
9. Pale skin -- this hasn't changed a whole lot with Pierson's coarctation repair -- he's just very caucasian.
10. Shortness of breath - especially when they're playing.
Note: It's common thinking that all babies and children born with heart defects are blue. That's not true!
Is Coarctation of the Aorta life threatening? Yes. Coarctation of the Aorta is considered a critical congenital heart defect. It usually requires surgical intervention within the first year of life. God was super gracious to us and protected Pierson untreated for three years. How he survived is a miracle.
How is Coarctation of the Aorta treated? For children who have severe to moderate coarctation of the aorta heart surgery is usually required. The type of surgery most commonly performed (and what Pierson had) is called end to end anastomosis. That means that the aorta is cut above and below the coarctation at the most healthy points and then sewn back together. In some cases patches or grafts may need to be used to put the aorta back together. In children with moderate to mild coarctation of the aorta many times a balloon angioplasty can be used to widen the coarctation. A small ballon is run up through the aorta to the coarctation and inflated leaving the aorta widened. Many children require blood pressure medicine after the coarctation is repaired. Most can usually have their blood pressure medicine weined and eventually be able to go through life without it.
Surgery is a repair, not a fix or a cure. There will always be at least a little scar tissue present and in rare cases the coarctation can reform. A CHD is never "fixed" but the outcomes for children who are born with CHDs and have the repair is very good! I know one 31 year old who was born with Coarctation of the Aorta who played college soccer, got his masters and is married with 3 beautiful children. There is hope and a future.
How common is Coarctation of the Aorta? 1 in 150 children are born with Coarctation of the Aorta. 1 in 100 children are born with a congenital heart defect. It often accompanies other heart defects but sometimes like in Pierson's case can be stand alone. It can often cause further congenital heart disease like left hypertrophic ventricle or cardiomyopathy. Pierson had both.
What causes Coarctation of the Aorta? Gosh I wish I knew. Around 8 weeks gestation something mis-fires causing the aorta to not completely form. I took awesome care of myself, took prenatal vitamins for months before coming pregnant, got plenty or rest, didn't smoke or drink... but still my child has congenital heart disease. Pierson's caridoligst assures us there is nothing I could have done or not done to prevent this. There are a few working theories that left sided heart defects like coarctation of the aorta have some sort of genetic factor. That's one of the reasons we need to raise awareness. If there is something that can be done to prevent this we need to find out and do it! No child should have to go through this!
What kind of futures do children born with Coarctation of the Aorta have? Give this kid some shades, his future is bright! The life expectancy for these kids used to be 40 but according to P's cardiologist, they are doing much better than that now and he expects him to grow old. They may need more surgery in the future, they may need some angioplasties but there isn't much that this will hold them back from. Yes there are some things that they may face like learning to live with blood pressure spikes when they get too hot but that is such small stuff in the big picture. Every child is different so I can't speak for everyone, but Pierson has zero restrictions. His cardiologist told us the best thing we can do for him is keep him active so that's what we do!
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