Cari's Cardiology Update

Normally, Faith is the one with all of the appointments but this week it has been my turn. On Monday I had my annual echocardiogram. On Tuesday I had my pacemaker device check and appointment with the cardiologist who comes to Bismarck from the Mayo Clinic in Rochester, MN. 

The doctors who come are pediatric cardiologists who also specialize in adults with congenital heart defects (CHD). This saves anyone in the area who has a CHD from having to travel to Rochester each year. It is very convenient! (I do travel to Rochester to get my pacemaker changed, though!) 

The Bible verse that has kept me going all these years!

When I saw the doctor, we mainly discussed two things: my Fontan and my pacemaker. But first, a little background. I was born with double outlet right ventricle (DORV). Basically, this means both my aorta and pulmonary artery stem from my right ventricle. Because of this, my left ventricle does not really do anything, which is why this CHD is referred to as a single-functioning ventricle. 

I had my first open heart surgery at 6 months of age. When I was 4 years old, I had the surgery now known as the Blalock-Thomas-Taussig (BTT) shunt. At ten years of age, I had my Fontan. During this open-heart surgery, deoxygenated blood goes directly into the pulmonary arteries without having to pass through the heart. 

The thing with the Fontan, is that while it greatly improves the quality of life, it does not fix congenital heart defects. Oftentimes, those who had a Fontan as a child need to get it revised when they become adults. I managed to go 15 years before needing a Fontan revision. 

Despite having the revision, there is a very good chance the Fontan will begin to fail. When this happens, medications or surgery might be able to help reduce symptoms. Some of these symptoms include: 

• Ascites
• Decreased oxygen levels
• Exercise intolerance
• Cyanosis 
• A condition called protein-losing enteropathy (PLE)

There might also be complete heart failure, which means a transplant would be necessary. I just found out that 50 percent of people who have had a Fontan will need a heart transplant by the age of 40. I guess that means I am in the other half of the group! 

I am very hopeful I will never need a heart transplant. But I understand it is a reality that my Fontan could start to fail at some point. I am not afraid of that happening, I would just want to ensure that Faith is looked after if that ever did occur. 

As for right now, my heart is doing well. My ejection fraction is around 45 to 50 percent (which is considered normal). I am also not retaining any fluid thanks to the diuretic I take. Besides a baby aspirin, I am not on any other heart medications. The cardiologist said he wished all his Fontan patients in their 50s were doing as well as me!

This is what I get hooked up to at the clinic to check my pacemaker. 

When I got my pacemaker checked, it showed that it had 9 months of life left on the battery. I had just gotten it replaced in August of 2024. One of the reasons I need to get it replaced so frequently is that I am paced over 90 percent of the time and it is set at 60 bpm. (If I set it lower, it would last longer, but the lower heart rate makes me feel like I can barely function). 

We have tried multiple things to get my pacemaker to work better and last longer, but nothing helps. In 2021, I got the leads replaced and this last time I switched from a Medtronic to Boston Scientific. Neither of these things have helped increase the life of my pacemaker. 

So, it is just another reality I have to face that unless they come up with something super advanced, I will need to keep getting it replaced every 2 to 3 years. If this is what it takes to keep my heart beating, then I will do it! 

I will be heading to Rochester next year to get my pacemaker replaced (again!) 

During these annual cardiology appointments, I always find myself getting a little emotional. Part of it is the realization that this is my life, my life with a complex congenital heart defect that requires lifelong care. And every year, I can only hope and pray that there are no surprises with any of my tests. 

The other part is that I am incredibly grateful to God for His grace and mercy. I am also thankful that I have received such great care over the years from some of the best doctors and surgeons in the country. 

I feel truly blessed to be doing so well. It is something I will never take for granted because I know that is not the case for many born with my same CHD and who have had the Fontan. God has truly blessed me and has answered many prayers. In my mind, there really is no other explanation!