Tylers Story Part 1: A Complex Form of Heart Disease
At 17 weeks, Emmy was diagnosed with Dextrocardia with Situs Inversus. In the simplest of terms, her heart is on the right side of her chest cavity and her organs are a mirror image of normal anatomy. Our MFM also saw what he thought was a small VSD in her heart, so he sent us to a fetal cardiologist for further evaluation. At 20 weeks, we heard the words that changed our lives forever…
”I think your baby has a much more complex form of heart disease than we originally thought.”
This was our first appointment with our cardiologist who we adore, Dr. Stewart. After an extensive fetal echo, Dr. Stewart made a Heterotaxy diagnosis. More specifically, she diagnosed Emmy with left atrial isomerism and the following heart defects: ASD, multiple VSD’s, TGA, DORV, LVOTO, PS, I-IVC, and severe AV valve regurgitation.
Miraculously, the AV valve regurgitation was completely resolved two months later. To this day, Dr. Stewart still talks about how she has no medical explanation as to how that happened. She said she has never seen such a severe case of regurgitation just go away. Praise God!
Emmy was born full-term and did so well! Her heart was stable, and all other testing was normal. She technically has polysplenia, but she does have one normal-sized spleen that, to the best of our knowledge, functions normally. She also has intestinal malrotation. Our team opted not to do prophylactic LADD’s, as the risks outweighed the benefits.
We had monthly visits with our cardiologist, and bi-weekly visits to our pediatrician until Emmy had her first surgery at eleven months. When she was seven months old, we met with the surgical team here in Austin to discuss what they felt was possible repair-wise for Emmy’s very complex heart. They said they weren’t sure if a Biventricular repair would be possible. Up until that point in time, our cardiologist had been sure that a BiV repair wouldn’t be an issue, so we were confused about why the thought process had now shifted.
After talking with a fellow heart mom, she suggested that we get an opinion from Dr. Emani at Boston Children’s Hospital. Dr. Emani is the director of the Complex Biventricular Repair Program at BCH. They see the most complex cases on a daily basis from all over the world. I reached out to Dr. Emani and he had personally emailed me back saying that he would be happy to look over Emmy’s case.
After getting all records up to Dr. Emani, he had the chance to present Emmy at their weekly cardiac conference. After discussing her case with the team, he called me and let me know that he felt that Emmy’s heart was favorable for a BiV repair! PRAISE JESUS!! He said it would likely be done in two stages, but we were ecstatic!
Fast forward to February of 2020, we were on a plane to Boston. We didn’t know how long we would be there. We were prepared for a one-month stay, but also knew it could be much longer depending on how her recovery went.
Part of the reason Dr. Emani wanted to do a staged approach was that her left ventricle had always measured a little small on the echos. They believed this was due to the fact that her atrial septal wall had failed to form properly and was laying over the mitral valve, thus blocking a good amount of blood flow into the left ventricle.
The plan was to try and remove this piece of tissue on the mitral valve during the cath so that the left ventricle could start functioning properly and grow to a more favorable size for BiV repair. A few days after arriving, Emmy had her echo, MRI and Cath. The doctors were pleasantly surprised with the results.
They originally were concerned that her left ventricle was considerably smaller than it should be. However, the cath revealed that the size and function of the left ventricle was normal and that her right ventricle was actually enlarged on the echocardiograms due to the fact that it had been receiving the majority of the blood flow.
The cardiologist went on to tell us that Dr. Emani would most likely be doing the full BiV repair that week. We were in shock! We didn’t even know this was a possibility. The next morning, we heard the words that we had been waiting 16 months to hear…
“I can do a biventricular repair.”
As we sat in Dr. Emani’s office, our jaws were on the floor as we heard the man speak those words out loud. Up until that point, we didn’t know for sure if it would be possible at all, and we certainly never thought her first surgery would be a full repair.
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