Baby’s Story

Site created on April 6, 2018

During a routine ultrasound at 23 weeks, we discovered that our precious baby has a severe congenital heart defect known as Aortic Stenosis: a narrowing or obstruction in the aorta. It is a fatal condition if left without intervention as it always develops into a more severe condition called Hypoplastic Left Heart Syndrome. As a result, we were rushed to Boston Children’s Hospital to undergo an in-utero operation to help stabilize our baby’s heart valve until birth. We are hoping that this procedure will help prevent a series of 3 open heart surgeries that would otherwise be needed if her heart had been left untreated during pregnancy. 

No matter what, our baby will be born with a severe heart defect that will require surgeries either within the first few days of life or later on. We just simply do not have the answers right now as to when these surgeries will occur, as the variables are all still in play.  During week 26 of our pregnancy,  our amniocentesis results came back positive for Turner Syndrome. She is a mosaic case: 80% of her genes are missing 1 "X" chromosome. More tests after birth will help determine if she has any other symptoms of TS. 

At 33 weeks, we discovered that we will most likely be delivering in DC and looking at a hybrid heart procedure immediately following birth. Links are posted below! 

For your questions, please visit the following sites for more information on the conditions mentioned above: 

Fetal Aortic Valvuloplasy (first page only): https://obgyn.onlinelibrary.wiley.com/doi/pdf/10.1002/uog.9040 (https://obgyn.onlinelibrary.wiley.com/doi/pdf/10.1002/uog.9040)

Fetal Aortic Stats: https://obgyn.onlinelibrary.wiley.com/doi/pdf/10.1002/uog.13632 (https://obgyn.onlinelibrary.wiley.com/doi/pdf/10.1002/uog.13632)

Boston Children's Hospital Study on FAV procedure: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4299861/ (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4299861/)

Hyperplastic Left Heart Syndrome: https://www.cdc.gov/ncbddd/heartdefects/hlhs.html

Turner Syndrome: https://www.turnersyndrome.org/

Pulmonary Artery Banding: https://www.jhltonline.org/article/S1053-2498(16)30116-4/abstract

Hybrid Therapy for HLHS: https://www.jtcvs.org/article/S0022-5223(15)02129-7/abstract

Newest Update

Journal entry by Josiah McClure

Wow, it's been a cray few days! There's so much to update about so I'll be as concise as possible.

Carly was admitted to MedStar Washington Hospital Center Tuesday (7/10) at 9pm. We stayed overnight and the induction process was started first thing Wednesday morning (7/11). She was put on pitocin which kick-started her contractions and then they broke her water around 1pm. That's when things really picked up. She got an epidural around 1pm and delivered vaginally around 1:45. Carly delivered in the OR as a precaution. After Joanna was born Carly got in a quick kiss and then they took Joanna to the OR in the next room over to evaluate and stabilize her. 

Joanna was actually very stable after birth. The initial echo actually surprised our fetal cardiologist. Based off of an echo Carly had the previous Monday, our doc said that the left ventricle (LV) was a "small ball." She also saw "little to no flow" through the aortic arch. She did not expect any function from the LV after borth. However, after delivery the LV nearly doubled in size and surprisingly there was blood flow through the Mitral valve, into the LV, through the aortic valve, and up and across the aortic arch. This was very surprising.

After the evaluation they transferred me (Josiah) and Joanna over to Children's National Hospital.

They did another echo 2 hours after delivery and Joanna's numbers continued to improve. The circulation and function wasn't "normal" but based off of the early trend, our doc wanted to wait 24-48 hours to observe and see if things continued to improve. They put Joanna on Prostaglandin which is a medication that keeps the "ductus" (a small hole) open between the left and right sides of the heart. This allows blood flow to leave the right ventricle (the working ventricle) and pass through the ductus out to the body. In a way, the RV has been assisting Joanna's LV with blood flow output.

Overnight Wednesday into Thursday, Joanna's numbers looked really good and she was very stable. However after an echo on Thursday afternoon, her numbers showed no improvement from the day before. Also, because of her poor LV function she was having too much blood flow from the RV to the lungs and not enough blood flow out to the body (especially the feet). That is when the medical team decided the best thing to do would be to move forward with a hybrid surgery Friday morning (today).

Hybrid surgery: our surgeon will open the chest and place small bands around the pulmonary (lung) artery. This restricts blood flow to the lungs and forces blood through the LV and out to the body. Carly and I were really on board about this hybrid option. Since Joanna is not technically (clearly) HLHS (hypoplastic left heart syndrome) she is in a weird gray area where her LV isn't functioning properly BUT it is there and it is trying. This hybrid surgery allows Joanna to have very stable circulation after surgery but the RV can still assist the LV with output. This will hopefully give us up to 8 months from now to allow Joanna's heart to grow and heal and possibly re-train the LV to function. In 8 months time, if her LV appears ready to handle the load by itself, they can remove the bands and essentially undo the hybrid surgery. At that time, if the LV isn't ready then we still have more options than we do now because Joanna will be bigger and we will have a clearer picture of her heart's capability. All in all, we are in a great situation. 

Joanna is in surgery now which should take 45min-1hr. Anesthesiology came and took her to the OR around 7:45. They will sedate and intubate her (with a breathing tube down her nose) and then proceed with surgery. I'm assuming she will be back in our room sometime before noon. She will be sedated for 24-48hrs for monitoring.

At this point, our hope and prayer is that Joanna has a quick recovery and the hybrid surgery gives her stable circulation. If that happens, my understanding is that we could have her home in 10+/- days. After that, we should have 8months +/- to lay low and be a family! At that time we will know more about next steps.

Thank you all for your incredible outpouring of love and prayers. We have been overwhelmed by our community. God has been so, so good to us and you have all demonstrated His love to us so well. He is faithful!
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