May 9, 2009

Charlotte's Heart

Some of you have asked about Charlotte's surgery.
She had open heart surgery when she was three months old to correct Tetralogy of Fallot. A slideshow of that is at the bottom of this blog. _________________

TOF happens to 5 out of every 10,000 babies. Heart defects of any sort are fairly common in individuals with Down syndrome, like Charlotte.

It is four (tetra means four) related defects:

1) Pulmonary artery stenosis: the artery taking blood to the lungs to go fetch more oxygen is narrow and thus...
2) Hypertrophy of the right ventricle: the right ventricle wall gets thicker because it has to work harder to get the blood through the narrowed pulmonary artery.
3) VSD- ventricular septal defect: a hole in the septum (the wall) between the two ventricles and thus...
4) Overriding aorta: because the ventral septum is faulty, the aorta kind of fumbles over it. All this allows oxygen-poor blood to leak through and get mixed with oxygenated blood. Leaning the mixture, so to speak.

Charlotte was considered a "pink Tet" baby as opposed to a "blue Tet" because her defects were such that she still got enough oxygenated blood to keep her "pink." Even still, during one clinic visit, the cardiologist decided it was time to get her fixed up. So, about a week later we found ourselves at UAB awaiting the famous Dr. James Kirklin, son of the famous Dr. John Kirklin of Kirklin Clinic fame, to perform her surgery. I have to insert here that I have the greatest respect for this man and will forever be grateful for the kindness he showed me. He mended Charlotte beautifully and although she bears a scar, it is minimal compared to others I have seen.

It was also discovered during surgery that she had a PDA -- patent ductus arteriosus. The blood vessel between the aorta and the pulmonary artery usually closes shortly after birth... unless it doesn't and then it's called patent (open). About 3000 babies in the US are born each year with a PDA. It can close later on its own, or may require surgical repair. Charlotte's leaks so very little now that we're not going to worry anymore.

As a result of having to fiddle with such a small organ, Charlotte now has AV heart block. Her two sinus nodes that tell the heart when to do its pumping are not in agreement, so the result is a lower overall heart rate. I am told that this sort of iatrogenic heart block generally is not a huge concern (??), though she will eventually require a pacemaker, but that's down the road yet.

For now, she's happy and healthy and growing more beautiful each day.


  1. wow! thank you for sharing this with all of us. your slideshow is made me cry. what a lovely daughter (and son!) you have. my nephew was born with ToF and had his repair done in atlanta when he was 5 months old. it was a difficult and amazing time. such a scary experience, but we were so profoundly greatful for the doctor's ability to help bailey.

    look forward to reading more here on your blog.

  2. oh jenna, you are such an amazing momma and she is just an amazing little girl! you are an insperation to all momma's!! thank you for sharing this and i know as a momma it couldn't have been easy to watch and go thru and not be able to do more but she is doing well and you will be so strong and such an awe inspiring person!!

  3. Okay ... now that we all sat in the office and cried ... we want more videos!! :)