Matthew's Update From Boston

Hi Friends!

I don't think I've had a chance to talk to you all directly for a couple of months, but I've been spending a lot of time with Mimi and she told me she's been updating everyone. As you know, I'm back in Boston with Mom and Dad, getting ready for my second heart surgery, the first step of the Fontan Procedure. Below is an overview of the issue and the operation, for you really smart folks that like the details. I had the Norwood procedure back in December and now I'm going to have the Bidirectional Glenn procedure this time.

Do I have a story for you!

I was scheduled to have surgery on Monday, but after they made me go to sleep so they could keep me still and get a clear picture of my heart earlier this week, I woke up and have wanted to keep the oxygen ever since. For this reason Doctors Baird and Brietbart decided to move my surgery up to tomorrow.

I heard Dad saying something about Friday the 13th and Mom said that must mean really good luck for me! Plus Aunt Jen told me she and Uncle Ryan intentionally got married on Friday the 13th because they thought it was such a lucky day, so I think this is a great sign.

Dr. Baird told Dad today that I'm in really good shape for this next surgery and they are really excited for a good outcome. Dad told me it's because I've been doing my arm and leg aerobics so much and getting my heart ready!

My Grandpa Hall was planning to be here with us, but Mom's grandma passed away yesterday so he has to be back home with the family. I just found out Mimi has decided to hop on a plane and join us up here for all the festivities. She'll be here tonight and she's going to stay at the hospital with me. It will be just like back home when I get to spend the night with Mimi on the nights Mom and Dad are both working at the hospital!

I think I've told you guys most of the important stuff. You can read more about what the doctors are doing to my heart and why in the information below. Thanks for all your love, thoughts and prayers. I know Aunt Jen and Mimi will keep you updated and I'll write again as soon as I can! XOXOXO!!

Matthew

Children with hypoplastic left heart syndrome have a single effective ventricle supplying blood to the lungs and the body (either from birth or after an initial surgery e.g. Norwood procedure). They are delicately balanced between inadequate blood supply to the lungs (causing cyanosis) and oversupply to the lungs (causing heart failure). In addition, the single ventricle is doing nearly twice the expected amount of work (because it has to pump blood for both lungs and body).

The Fontan is usually done as a two staged repair.
The first stage, also called a Bidirectional Glenn procedure or Hemi-Fontan (see also Kawashima procedure), involves redirecting oxygen-poor blood from the top of the body to the lungs. That is, the pulmonary arteries are disconnected from their existing blood supply (e.g. a shunt created during a Norwood procedure, a patent ductus arteriosus, etc). The superior vena cava (SVC), which carries blood returning from the upper body, is disconnected from the heart and instead redirected into the pulmonary arteries. The inferior vena cava (IVC), which carries blood returning from the lower body, continues to connect to the heart.
At this point, patients are no longer in that delicate balance, and the single ventricle is doing much less work. They usually can grow adequately, and are less fragile. However, they still have marked hypoxia (because of the IVC blood that is not fed into the lungs to be oxygenated). Therefore most patients are referred for another surgery.
The second stage, also called Fontan completion, involves redirecting the blood from the IVC to the lungs as well. At this point, the oxygen-poor blood from upper and lower body flows through the lungs without being pumped (driven only by the pressure that builds up in the veins). This corrects the hypoxia, and leaves the single ventricle responsible only for supplying blood to the body.