The Battle Begins

Introduction

A few weeks ago, we had an appointment at the Maternal Fetal Medicine Clinic in St. George for an in depth ultrasound of our baby. During that ultrasound, they discovered that our baby has a congenital heart defect, which we now know is Tricuspid Atresia. As a result of this we will not be able to deliver the baby in St. George as we had planned. We will instead need to deliver at the University of Utah Hospital and then take the baby over to Primary Children's Hospital for observation and eventually corrective heart surgery.

Because we have been blessed with many family members and friends who are very supportive and want to know how things are developing with this situation, we decided to start this blog to help keep everyone updated rather than having to rely on the grapevine.

Finding the Issue

We had our first full ultrasound early in October. At that appointment everything looked great except that they weren't able to see the baby's heart as well as they wanted (he likes to put an arm in the way and constantly turn away from the camera). So thinking that we just had an uncooperative child we scheduled an additional ultrasound for early November.

At that follow up appointment they still had a hard time seeing the structures in the heart that they look for. At that point the doctor referred us to the specialists at the Maternal Fetal Medicine Clinic to double check the heart. That was the first indication we had that something might be wrong, but we didn't read too much into it.

We went to the next appointment the following week and spent about half an hour with the ultrasound tech who spent the majority of time getting a better view of the baby's heart. After she was done and the doctor looked over everything, he came in and told us about the defect and the need to deliver in Salt Lake and have surgery. He thought that the baby had a rare condition called a double-outlet right ventricle (DORV), but since that is beyond what he usually deals with he connected us with the specialists up at Primary's. They also took a sample of amniotic fluid for further testing.

Needless to say, this all came as quite a shock to us, and it has been an emotional few weeks for us since then. Among the most difficult things to deal with was not having any more answers than that for several weeks.

We finally had our appointment up at Primary's at the end of November. They did an echocardiogram and looked in detail at the baby's heart to see exactly what structural issues there are and how the blood is flowing. After that we met with a fantastic pediatric cardiologist who spent quite a while with us explaining exactly what is wrong with the heart and what the corrective procedures are (explained in further detail below).

As we were driving back to St. George that day we also received a phone call with our amniocentesis results, which came back completely normal. After the relief of finally having more concrete answers of what is going on with the baby's heart, that phone call helped make for a surprisingly uplifting day. 

So what is Tricuspid Atresia?

In tricuspid atresia the tricuspid valve of the heart doesn’t properly form. This is the valve between the right atrium and right ventricles of the heart. If you need a refresher course on the normal function of the human heart see the first picture below.

Because that valve never forms, the right ventricle of the heart never really develops. Since the right ventricle is responsible for pumping blood to the lungs to get oxygen this is a pretty serious problem. The body compensates by creating holes in the heart (atrial septal defect and ventricular septal defect) that allow oxygenated blood and deoxygenated blood so that the baby at least gets some oxygen. See the second picture for a diagram of what a heart with tricuspid atresia looks like.

What does all of this mean?

So what does this mean for our baby? First off, the doctors want us to deliver him at the University of Utah Hospital, so that we can then transfer him over to Primary Children’s Hospital for observation (Primary’s doesn’t have delivery rooms). They will watch him for a few days to see how well his heart and lungs are compensating.

If his lungs are receiving either too much or too little blood flow they will do a surgery to temporarily adjust the flow to a more acceptable level. However, the doctors are currently hopeful that we won’t need to do that procedure.

At approximately 3-4 months old we will have to go back to Primary’s for a heart surgery known as a Glen procedure. At 2-4 years old a surgery known as a Fontan is done as well. These essentially bypass the non-functioning right side of the heart.

Even after all those surgeries he will have to follow up with a cardiologist regularly to continually watch for future complications. And while those complications can be quite scary, we’re hoping and praying that he will be able to avoid those and live a mostly normal life.

Next Steps

Until our due date we are continuing to follow up with the OBGYN, the maternal fetal medicine clinic, and Primary’s to continue to monitor the baby’s health and heart development. We will also hopefully be meeting soon with the doctors that will actually perform the delivery and get a tour of both facilities.

Thankfully, everything else with the pregnancy is going quite well. We have been very fortunate that Angie has not had to deal with excessive sickness or any other complications. They also did an amniocentisis test to see if there were any other issues with the baby and those results came back completely normal.

So how are we doing?

Needless to say this has been a very emotional month for us. Though the doctor was very kind when he initially told us that our baby has a congenital heart defect and would require heart surgery, it was a lot to process. Add to that not really having any solid answers for several weeks, and it’s been pretty rough. Honestly, it’s kind of a day by day or even moment to moment thing now. Some days we’re feeling really good and hopeful about everything and other days are complete panic attacks. Thankfully we tend to alternate days and have been able to support each other, as well as having great support from family and friends.

Keep watching for future updates here as we continue to learn more and have a solid plan in place.