Tetralogy of Fallot with Pulmonary Atresia and Major Aorto-Pulmonary Collateral Arteries
TOF/PA/MAPCAs -- click the link to see a .pdf put out by the Heart Clinic
When I was pregnant, it was difficult to know for sure as to whether or not Margaret actually had any Pulmonary Arteries, but it was clear there definitely was no Pulmonary Valve. The MAPCAs were an added complication in that they were difficult to see and identify for certain as well. Fortunately, although she has some MAPCAs, hers are not as severe or large in number as they can be. It's my understanding that these MAPCAs develop as a sort of way for the heart to make up for not having pulmonary arteries (or sufficient arteries).
TOF is basically a defect where there are 4 main issues and the result is not enough oxygen ends up in the blood being pumped throughout the body. Pulmonary Atresia and MAPCAs are an "added bonus".
*This is the picture the cardiologist who diagnosed Margaret's condition with drew when she first sat down with us. It was scary and overwhelming but it's helpful to look back at and try to better understand our little warrior's heart.
*This is a picture the surgeon drew for us when we met with him a week after our initial diagnosis. He proved right then and there how much he knows and his confidence was obvious!
Here is another good site to read up on TOF put together by Boston Children's Hospital.Many times babies who are born with TOF are labeled a "pink" or "blue" tet but honestly we don't know what Margaret would fall under. She was born nice and pink but she did require a shunt surgery within her first week of life and her team never actually used pink or blue to describe her. It's really not important but you do hear people talking about pink and blue TOF often in the CHD world.
The big surgery Margaret will be having is often called the "full repair" because the ultimate goal is to repair the four defects. This isn't always the case for each child and some require multiple surgeries to finish the "repair". I'm not sure how I feel about the word repair being used to describe what is going to happen to Margaret's heart tomorrow because I think it gives a false hope of being "done" and her heart being "fixed". This isn't the case. Margaret will always have TOF and her heart will always be different and require follow-up care by the cardiology team. Margaret will also have future surgeries to replace her Pulmonary Valve throughout her lifetime as she grows. Eventually the goal is for those to happen via heart catheterization procedures though instead of open heart surgery.
The major things we hope to have accomplished tomorrow during surgery in bullet form:
- undo the Melbourne Shunt that was done when she was 6 days old (basically put her pulmonary arteries back where they should be and connect them to a conduit)
- close the VSD
- build and place a conduit/pulmonary valve
- re-route one of the bigger MAPCAs to utilize it more efficiently/effectively
We've been warned several times that there's a possibility of the VSD not being closed fully because Margaret's heart has been working with it open and closing it completely might be too much pressure for it. The surgery will take place in the hybrid operating room so after completing everything they will do a heart catheterization to check pressures and see how her heart holds up before taking her off bypass.
Margaret's pacemaker will remain in her for now as well. She has a spunky history and it's best to keep it in and see how she recovers from this major surgery before any decision can be made about her possibly not needing it forever. We aren't in a hurry to have it removed anyways. Not having to worry about hear heartbeats not being in a normal rhythm is something we don't want to have to worry about if we don't have to!
And here's a picture of Mighty Margaret from church last week. She's recently decided that she likes to make faces at the people who sit behind us and she LOVES to squeal during the quietest parts of service. :) **check out those cheeks!!
