After our appointment on Friday with the cardiologist, we talked with our surgeon, Dr. McKenzie (Dr. M), and his Nurse Practitioner (NP), Kelly. Reiss’ issue of heart failure is centered around her tricuspid valve’s regurgitation (TR) and her left-sided, right ventricle’s dysfunction.
Before we get started with what that discussion entailed, I believe it is important to give you a picture of Reiss’ heart – see picture below. On the left is a normal heart. On the right, it shows how the tricuspid valve and right ventricle both sit on the left side of the body. In Reiss’ heart, however, the ventricular septum has two patches in it from the closing of her VSDs, and her PA (pulmonary artery, which pumps to the lungs) was ligated and replaced with a conduit with its own valve. Also, Reiss’ heart is positioned in her body more towards the center of her chest instead of on the left side, as well as turned slightly.
Dr. M mentioned that he was puzzled about her mild to severe TR just within two months of open heart surgery. When we were admitted, he asked that we get a cath to look at the pressures in the heart. He thought that her TR and ventricular dysfunction may have to do with the pressure in the left ventricle (LV). If the pressure in the LV was low, then the ventricular septum would bow towards the LV, pulling apart the tricuspid valve (TV). The fix for this low pressure would be to have an additional open heart surgery to decrease the size of the conduit going from the LV to the PA. However, this was not the case. In fact, after cath results were in, they saw that the pressures in her LV were almost perfect.
Another guess on why Reiss has TR and right ventricular dysfunction is that her pacemaker is inducing cardiomyopathy. The echo Reiss had in the cath lab does not confirm this, though. Additionally, multiple electrophysiologists (EP) discussed her case and reviewed her data, finding no hint at ventricular dissyncrony.
At this point, they just plain don’t know why. They do know that TR is a sign that the ventricle isn’t happy, but they don’t know why the ventricle isn’t functioning properly. Everything else in her heart looks good – pressures, conduit, VSDs are closed, LV is happy, branch pulmonary arteries are good. They don’t see a quick or easy solution.
She is currently on lasix and enalapril to reduce fluid build-up and increase heart function and they seem to be working. However, her TR is listed as “moderate to severe” and this is not durable over time. They sent us home on Friday and told us to see our local cardiologist this week, as well as schedule a follow-up in Texas a month from now to check on the TR.
What do we do if the TR is better? Nothing. We continue with the medicines we are on, go to follow-up appointments locally and in Texas, and keep watching for signs of heart failure.
What do we do if the TR is worse? Surgery. We will schedule a surgery date two months from now in case we need it after our follow-up in a month in Texas.
- Tricuspid valve repair | The ring around the tricuspid valve is called an annulus. Reiss’ annulus is currently dilated. This repair – an annulaplasty – would be to decrease the size of the annulus, allowing the leaflets of the tricuspid valve to touch and close properly. The team would use a cloth ring that would grow with her probably into her teenage years.
- This surgery would be open heart
- Her tricuspid valve is not easy to get to because of her anatomy
- They aren’t sure if the tricuspid or the ventricle is the issue. If they fix the tricuspid, but the RV still isn’t functioning properly, then they have just had an open heart surgery to repair something that didn’t fix the problem. They are pleased (at this point) that her RV dysfunction is tagged as “mild”, which may mean TR repair could work.
- Bi-ventricular pacing | A controversial option, according to Dr. M, for her pacemaker is to place a lead on both ventricles so that an electrical current is sent to both ventricles at the same time, allowing them to push against each other to provide support in the pumping function of the heart.
- The team in general does not usually have an additional surgery on a child just to place these types of leads – there is controversy over if this is beneficial, as well as if the trauma from an open heart surgery is warranted solely to place these leads. Because of this, they would most likely only do this option if they were already planning on the tricuspid valve repair mentioned above.
- Reiss would also get a new pacemaker that had a port for this extra lead.
So, we will wait. Wait until Monday to schedule appointments. Wait to see what her echo and EKG looks like this week. Wait to go back to Texas in a month for a follow-up. Wait to see if she has more symptoms of heart failure.
Thank you for following our journey!