With Hannah’s surgery getting closer, and many people asking for details, I thought I would take some time to make a post explaining exactly what her surgery entails.
Hannah will go in on August 10th for about 4-5 hours for pre-op testing. She will have an echo, ekg, nasal swab for viruses, and I’m sure a multitude of other tests. Once she is cleared, they will tell us what time we will need to be at the hospital on the 11th.
Once we get to the hospital on the 11th, and everything is underway, Hannah will be put under general anesthesia and intubated. Dr. Morales will make an incision in her chest through her skin and breastbone. She will be placed on the heart-lung bypass machine, which will take over the heart’s pumping action, and move blood throughout her body. Hannah’s heart will not be beating during this time. This allows the surgeon to perform the surgery on a still heart.
Once she is on the heart-lung bypass machine, and it is working properly, the repair will begin. There are different techniques used, and the surgeon can’t be sure exactly what will be done until he is actually looking at the heart, but I will tell you what my understanding is based upon what Dr. Morales has told us.
The large hole in her heart will be patched. She has a VSD (ventricular septal defect) and an ASD (atrial septal defect). Since she has both, it is an AVSD (atrioventricular septal defect). Basically, she has a very large hole between her two atria and her two ventricles. Below is a picture of what a healthy heart looks like compared to a heart with AVSD
The hole allows the blue, deoxygenated blood to mix with the red, oxygenated blood. This is why Hannah’s oxygen saturations (the amount of oxygen in her blood) hang out around 85, instead of between 97 and 100 like a person with a healthy heart. The surgeon will use a piece of pericardium from Hannah to patch the AVSD. This will prevent the blue blood and red blood from mixing. The pieces of tissue he will be working with are as thin as tissue paper, and will be sutured into place.
Once the AVSD is repaired, the two stents that were placed in her RVOT (right ventricular outflow tract) with be removed. These stents are what have allowed her to receive enough blood flow through her pulmonary artery to keep her oxygen saturations high enough for her to be safe. This is one of the components of Tetralogy of Fallot. Her RVOT is stenosed (narrowed) causing her to need stents to hold it open in order for blood to more efficiently pump through her pulmonary artery. Once those stents are removed, a patch will be placed where her pulmonary valve is.
Doctors has discovered that it is best to place a patch in the RVOT instead of a new valve right away. The valve ends up needing replaced in a few years, whereas the patch can usually stay in place until the patient is in their 20’s, when a valve will then be placed.
The surgeon will then make sure the patch isn’t leaking too badly, although there will always be a bit of backflow. As long as the heart doesn’t get too large from backflow, the patch can stay.
The AV valves will also need patched. Typically this patch will last for the rest of the patient’s life, although in about 10% of patients, it leaks too badly and another surgery is required within a couple years.
The surgeon can use either a two patch technique, or a one patch technique. Generally, patients with Trisomy 21 require two patches. Once all the patches are in place, Hannah’s heart will resume beating and the heart-lung bypass machine will be switched off.
The survival rates for this surgery are excellent, and Dr. Morales has never had a patient pass away while performing this repair. One of the worst complications would be if he were to hit the AV node, which is a bundle of nerves that coordinates the top of the heart. The AV node has been known to be located in a different place in the heart in patients with ToF. Because of this, the risk of hitting it while suturing the patches in place is higher in patients with ToF. Dr. Morales has never hit the AV node in prior repairs.
The surgery itself will last 4-6 hours. This does not include time beforehand to administer anesthesia. Once she is stable, she will be taken to the cardiac ICU. She will probably still be intubated, but there is a chance they will be able to extubate in recovery. Patients with DS (Down syndrome) however, tend to take a bit longer to effectively breathe on their own again, so we are planning on her still being intubated. Her total hospital stay will be between 5-7 days, but she could be there longer.
The main goal of the surgery is to repair her heart so that the blue and red blood no longer mix, and she doesn’t need oxygen to maintain sats close to 100. It is also to relieve the stenosis of the RVOT, so that blood can efficiently move through her body without stents or shunts. The hope is that she will need no other surgeries until a pulmonary valve needs placed around 20 years old. They are hoping by that time, medicine will have advanced enough to be able to routinely place valves via a catheter, instead of open heart surgery.
Needless to say, it is extremely difficult to think of my little 6 month old baby with her chest open, heart not beating, with tubes and wires coming out of her. I have heard from numerous people that I am stronger than them and that they have no idea how I have gotten through everything so far, and how I can speak so calmly about her upcoming OHS. The truth is, I’m not strong. I am so very weak. I cry and worry and there have been times where I was angry and bitter that this is happening to my daughter. My only hope and my only strength comes from knowing we serve a sovereign God. He has given me strength I never could have imagined having. Looking back at Hannah’s time in the hospital, I have no answer for how I got through it except for God. He has worked in me through Hannah the past 5 months, and I don’t even recognize the person I’ve become. To me, ToF, AVSD, Down Syndrome, O2 sats, and doctors appointments are just life. It has become my new normal. The Lord has given me the ability to find peace in turmoil and uncertainty.
I still worry, I still cry, I still get anxious, but I hold onto words like these during those times:
“Be gracious to me, O Lord, for to you do I cry all the day. Gladden the soul of your servant, for to you, O Lord, do I lift up my soul. For you, O Lord, are good and forgiving, abounding in steadfast love to all who call upon you. Give ear, O LORD, to my prayer; listen to my plea for grace. In the day of my trouble I call upon you, for you answer me.” – Psalm 86:3-7
We have the promise of an answer. Maybe not in the way we hope, or when we hope, but an answer always comes.