I know it has been awhile since I have posted anything and our next major surgery was The Fontan. I decided to add this one today because a very special friend is having his Fontan done today as I type. I remember that day like it was yesterday but in fact we are almost exactly 4 years from that very day. Tristan had his Fontan at 18 months old. We found out during the summer before that he was going to have his Cath to place a stint into his Left Pulmonary Artery and to take a look at things for his last open heart surgery. All went well with that. We got the call saying his surgery would be Oct. 6, 2008. I was scared and relieved all at the same time. We knew it was time because he was doing so well and hitting all his mile stones. We checked into the Corporate Apartment the day before surgery with my Mom in tow. The night before his surgery was filled with restlessness and worry. He had already had a hand surgery in between his last open heart surgery so he was OK with being at the hospital. I was just not mentally prepared to see him with all the tubes and wires again. We were the 2nd case or so we thought. We get there about 10am. All is well then we hear we have been pushed back because of an emergency...OK no problem. So we waited and waited. All the while trying to keep an 18 month old contained in the Pre-Op area with nothing to eat or drink since the night before. They were kind enough to give him an ounce of apple juice to hold him over. That only seems to make him worse. Then finally around 1pm we got word that he would be going back within the hour. In comes everyone for one last final check and to give him some fantastic medicine. Versed is the best loopy medicine for Tristan. He was trying to run around and continue to play with the trucks and read but he couldn't walk in a straight line. No sobriety tests for him while driving his little tonka dump truck. They finally came to get him and the emotions ran wild with me. I had to hold back my tears because I wanted to be strong for him. I still can see his face when he went into the operating room with a big cheesy smile and waving. He let the Anesthesiologist carry him back there with no problem. He waved and blew kisses and as she closed the door it was like the flood gate had been opened. I pulled myself together fairly quickly because of the strength of my husband and our family that was there with us.
After about 3 hours we got the call he was out of surgery so we all headed that way to go wait to talk to the Dr. I remember Dr. Jonas coming in to the room without much emotion and my heart sank. I had no idea what to expect but I wasn't expecting good news. Then he said everything went beautifully and he was on his way upstairs. More tears, but this time they were happy tears. We found out that the placement of the Fontan was a little complicated as most because it goes through his heart to connect the Inferior Vena Cava to the Pulmonary Artery where he wanted it to be. There wasn't really any more risk with doing it that way but the risk of infection rose just a little bit. So we all waited about 45 minutes before we were able to file 2 by 2 into see him in the CICU. He looked amazing when we first laid eyes on him. He still had the breathing tube in but all was great. Since Tristan's surgery was so much later in the day it was almost time for shift change by the time we took turns eating and visiting. Scott and I decided we were also going to take shifts staying with Tristan. So if I remember correctly I got the first night. It was fairly quiet because they weren't really going to do much since he wasn't long out of surgery. The following day however is when things started happening. Tubes and lines were already starting to come out. We were a little surprised because we were told that this surgery is usually 7 days in the CICU and about that many on the floor or more. So on day one post-op when stuff started happening we were a little excited. He was eating again on day two post op and I had to laugh when I walked into his room and he had a sausage in his hand because he was starving. Once he started eating he didn't stop for what seemed like 2 whole days. They decided to take his cath out on Saturday which was only the 2nd day after surgery, and this little guy knows what pain is. He peed once and then wouldn't do it again because it hurt. Poor little man was having trouble with his pressures and his oxygen saturation because he wouldn't pee because it hurt. We finally got the doctor to give lasix to help him go but the nurse wanted to cath him again. We tried to fight it as long as we could and then they wouldn't give him the lasix so they had to cath him. Well that was not fun. They basically did it then gave him the lasix....duh anyone would pee as soon as a diuretic was given. So later that day they took it out again and this time I told them they were not putting it back in. They did keep the lasix on board so we didn't have any trouble peeing after that. Day 4 were were moved to floor because little hot shot was doing so well. Tristan amazes me all the time, then and now. We ended up in the hospital for a total of 9 days. Tristan did so well they kicked us out early. To our surprise he was ready to go home.
The Fontan procedure is today the last staged operation for all children born with congenital heart disease who cannot be offered a 2-ventricle repair. Originally designed by Fontan for treatment of tricuspid atresia, the procedure has undergone 2 major successive technical modifications.1–3 In its first version, the atriopulmonary connection (AP), the right atrial chamber was isolated by the closure of the atrial septal defect and the hypoplastic tricuspid valve. The right atrial appendage was then anastomosed to the right pulmonary artery.3 It was later understood that better streaming of the blood flow in the systemic venous pathway to the lungs improved the patients hemodynamics and might avoid complications related to progressive atrial dilatation. The operation was therefore modified to the lateral tunnel technique (LT), whereby the right atrium was baffled with an intraatrial patch and the superior vena cava was sutured directly to the right pulmonary artery.1 Performing the anastomosis between the superior vena cava and the right pulmonary artery (bidirectional Glenn) at an earlier age as an intermediate step decreased total mortality and morbidity to achieve a final Fontan circulation.4 The most recent modification of the technique consisted in the replacement of the intra-atrial routing of the venous blood by the insertion of an extra-cardiac conduit (EC) between the inferior vena cava and the right pulmonary artery (Figure 1).2
Figure 1. Fontan surgical techniques: Classical atriopulmonary connection (A), Lateral tunnel (B), and extracardiac conduit (C).
An increasing number of “Fontan patients” are now entering adulthood, and these patients are facing an uncertain future. It is clear from the 20 years follow-up of the operations performed in the initial era that this operation remains palliative. Patients are prone to developing arrhythmias, heart failure, and progressive rise of their pulmonary vascular resistances.5–9 Conversion of the atriopulmonary connection or the lateral tunnel technique to the extracardiac technique has been successful in treating failing Fontan patients, but the indication of this new procedure are still under investigation.10,11 Because it is still unclear whether the technical modifications brought to the initial Fontan operation translated into long-term clinical benefits, we decided to review our clinical experience with these three techniques.
Once we got home things were great for a whole day! Then the following day I started weening him off his Tylenol and Motrin and things started going down hill really fast. He was very lethargic and tired and started running a fever. At first we were in the in the 99's, then slowly it crept up to finally topping out at 103.9. We were already packed and I called the Cardiologist on call and by the time we got to the hospital the ER was waiting for us and we went back then up to a room. We spent another week in this hospital. I had never been so happy to finally hear that it was a UTI from being Cathed a second time. While there we found out that Tristan's Spleen was in multiple pieces which is known as Polyspleenia. So now he gets put on another medication that will follow him for quite some time...Amoxicillin everyday. Hear we are almost 4 years later and still on Amoxicillin once a day. Because of the Polyspleenia he is immune deficient. Seriously at this point I was like whatever, keep throwing stuff at us, we can handle it, and handle it he has.
Tristan is now in Kindergarten, has all 10 separated and almost fully functional fingers and is doing fantastic. Last summer he has his Fenestration closed that was left open during the Fontan. His oxygen saturation's are between 92-99. He is about to go into the gifted program at his school and tested out of Kindergarten for reading and math. I couldn't be more proud of all he has been through and all the obstacles he has over come. He is one smart little boy with a heart of gold! He truly touches the hearts of all he meets. Do not get me wrong his is still a little boy who likes to torment his little brother who fights back pretty well. He played T-ball last spring, swam over the winter and we spent almost all summer at the water park. I will try to update this about once a week now but no promises. Drayk is in school twice a week so I should at least have one day a week to let all know what is going one.
Please send lots of good thoughts and prayers to our heart buddy Caelen who is having his Fontan right now!!! Good Luck Caelen...Katy and Martin we are thinging about you guys!
Oct 6, 2008
Sept16, 2012
