Options and Plan of Action
- Option 1 – Single Ventricle Pathway
- This option would entail two surgeries – the Glenn and Fontan. These two procedures allow blood to be routed directly to the lungs. The Glenn will be done this year. When she is 3-4 years old, she will undergo a Fontan procedure to complete the process. Her heart would work with one large ventricle instead of two separate ones.
What are the Glenn and Fontan Procedures?
Glenn – The main vein that routes oxygen-poor blood from the upper half of the body (superior vena cava) is connected to the pulmonary artery—providing a reliable source of blood flow to the lungs.
Fontan – The vein that routes oxygen–poor blood from the lower half of the body (inferior vena cava) is connected to the pulmonary artery—directing the oxygen-poor venous blood to the lungs to pick up oxygen, after which the single ventricle drives it out to the body again
- Option 2 – Two Ventricle Pathway
- The two ventricle pathway actually has two options within it. This first option is to close the hole in the ventricular septum so that the right ventricle works as the pumping chamber to the body.
- Issue 1: This is an issue over time since the right ventricle is not made to work in that fashion. In the long run, her heart would fail and a transplant would be necessary.
- Additionally, she would need a conduit place of her pulmonary artery since it is closed off. Issue 2: the first issue is that this would need to be replaced multiple times as Reiss grows. Secondly, the conduit would be squeezed due to the location of the heart and her chest plate. This could cause a limitation of blood flow.
- Issue 3: Also, her left ventricle has small volume. The operation would take up more of that space, potentially compromising the amount of blood to her lungs.
- Option 3 – Two Ventricle Pathway
- The second option with the two ventricle pathway is to make the left ventricle work as the pumping chamber as it normally would. Issue 1: Her left ventricle is too small.
- Issue 2: There would be a need to make a tunnel from the hole in the septum connecting the ventricle to the aorta. This would be a long tunnel.
- Issue 3: A conduit would need to be placed connecting the right ventricle to the pulmonary arteries. This conduit would also have to be rather long.
- Issue 4: The long tunnel and conduit would entail many surgeries.
- Issue 5: They would have to redirect blood from the inferior vena cava to the left side so that her blood does not mix (oxygenated vs non-oxygenated), which would be another procedure called an arterial switch or mustard procedure.
- To summarize – this option creates complicated pathways to make sure the left ventricle is the pumping chamber to the body.
PLAN OF ACTION: With the the above information in mind, Dr. E has decided that Reiss will continue on the single ventricle pathway. This process will begin with the Glenn procedure.
Benefits of proceeding with the Glenn
- It doesn’t burn any bridges. Even though she will go through a Glenn procedure this year, that doesn’t mean she will definitely need a Fontan later on. In 3-4 years, they will reassess her situation. Her anatomy could change by that time and one of the two ventricle pathways could be plausible.
- It is simplistic – this is evident after reading through option 2 and 3 above
- Her anatomy is set up for such – Reiss’ heart is set up well for a single ventricle pathway.
Date of Surgery
- August 6 – 10am – Reiss will undergo 6-8 hours of prep and pre-operation evaluations. We will also meet with Dr. E this day to go over the procedure and get a specific surgery time.
- August 7 – surgery day
Anatomy of Reiss’ Heart
I asked Dr. E if her diagnosis was still Tetralogy of Fallot. He said no. Here is what Reiss’ diagnosis is….
- DORV – double outlet right ventricle: Both the aorta and pulmonary artery come out of the right ventricle.
- SLL atrioventricular discordance: This means Reiss’ right ventricle actually resides on the left side of her heart, her left ventricle on the right side of heart. This is a rare defect.
- L-malposed great vessels: The position of the aorta and pulmonary artery is flipped
- Pulmonary atresia: The pulmonary artery is completely closed off
- VSD – ventricular septal defect: This is the hole in the septum which separates the two ventricles.
Ending on a Positive Note 🙂
That is a lot of information to process! And it has such a negative tone. The fact of the matter is Reiss is lucky. She is lucky to live in a time where surgery is an option. She is lucky to have a loving support system to help her along this process. She is lucky that there is a repair available. Reiss is a strong, active and smart little girl. We believe she will bounce back quickly, not missing a beat in development along the way. Even if she does – we’ll deal with that as it comes. She is a happy, vibrant baby girl. Her development is on pace – if not a little ahead of schedule.
Here are some things Reiss can do or likes to do:
- Crawling – she is not mobile. Though, her crawling is on one knee and one foot, which makes it look like a partial bear crawl.
- Shakes her “no” – and uses it in the correct situation
- Shakes her head “yes” – but this is usually when bee-boppin’ to music
- Pulling up
- Chewing on everything- even mom’s shoulder (ouch!)
- 7 TEETH!!!!
- Eating different solid foods
- NOT sleeping through the night (mommy no likey)
- Dances to music
- Loves to multi-task – one toy in mouth, one in each hand, while crawling and growling
- Swimming – her favorite hobby
- She can say – papa, mom, mama, dada, baba -though, none of those are really used in reference to a person or thing
- She does say Hi!, smiles and waves – to cows, tvs, people, cars, dogs, anything
- Yelling is fun
- She can blow raspberries – even in her sleep
- She mimic everything already
- Good memory – she remembers exactly where that cord was that you just said “NO!” to chewing on….even if it is across the room and behind the couch
- Loves her dogs
Thank you for your thoughts and prayers. They are always welcome and appreciated.
Sara, Roy, Reiss MaeLene