Friday, April 17th: it was an early morning out the door, alone for my first appointment at UVA Children's Hospital. All thanks to Covid-19 what should be an appointment shared together, Garrett is not allowed to attend the appointment and is staying home in Roanoke with Ryder. My ride to Charlottesville was peaceful and full of praise and worship. I knew I needed to start my day with uplifting music or I wasn't sure how I would get through this day alone. Once I arrive at the hospital, I parked the car and entered to the Covid-19 questioning booth and sanitation station before being allowed to check in for my appointment. Fortunately I passed with flying colors and was on my way.
Every I met at UVA far surpassed my expectation. I felt so comfortable being there, which was unexpected yet reassuring. I waiting for about 15 minutes before being called back for my fetal echo and sure enough, our sweet one was not the least bit cooperative with letting the sonographer see what she needed too. After about 45 minutes we were done and I was headed down the hall to meet with Dr. Schneider, one of the pediatric cardiologist that would be looking after our son once he is born.
We sat across from each other at a round table in a small room, both of our faces covered with a surgical mask... not quite how you'd expect a first meeting to be but hey, thanks corona! Dr. Schneider has to be one of the nicest doctors I have ever met and unfortunately, I have met my fair share over the last 9 years. He slowly described everything that he saw and didn't see on the fetal echo of our baby's heart. I asked questions, he answered with words I could understand and although the diagnosis was a little different than I went there anticipating I felt good. He made me feel confident in the plan they had come up with and with the abilities of himself and the two surgeons that would be on the front lines of caring for our boy.
The Diagnosis: Hypoplastic Right Heart Syndrome (HRHS) with Tricuspid Atresia, Pulmonary Stenosis and a Ventricular Septal Defect (VSD)
Hypoplastic Right Heart Syndrome (HRHS): is the blanket term that covers a congenital heart defect in which the right ventricle and/or right atrium is underdeveloped. This defect causes inadequate blood flow to the lungs.
Tricuspid Atresia: a congenital heart defect in which the tricuspid valve is missing or abnormally developed. The defect blocks blood flow from the right atrium to the right ventricle. (in our baby's case, he does not have a tricuspid valve)
Pulmonary Stenosis: when the pulmonary valve (the valve between the right ventricle and the pulmonary artery) is too small, narrow, or stiff.
Ventricular Septal Defect (VSD): is a hole in the wall separating the two lower chambers of the heart, the right and left ventricles.
To better understand what all of the terms mean and what they look like compared to a healthy heart take a look at the image below. The top heart is what our baby's heart looks like. The bottom heart is what a healthy heart looks like.
The Treatment Plan: this is the current treatment plan for our little guy, although nothing is set in stone until he is born and they are able to do an echocardiogram on him and they have the clearest understand of what is going on.
1 Week Old: a Blalock-Taussig (BT) shunt is a small artificial tube that will be placed between two of his arteries to increase blood flow to the lungs. There is still a mixing of oxygen poor and oxygen rich blood through the hole between the top chambers of his heart. This is a temporary fix that should last a few months until he has grown stronger.
4 Months Old: the Glenn procedure is when the large vein from the upper half of the body (the super vena cava: SVC) is disconnected from the heart and connected directly to the pulmonary artery. This reduces the amount of blood flow returning to the small right side of the heart by allowing blood returning from the upper body to flow directly to the lungs. - Once this is done, the BT shunt is no longer needed and is removed.
3-4 Years Old: the Fontan operation is when the large vein from the lower half of the body (inferior vena cava: IVC) is disconnected from the heart and connected directly to the pulmonary artery by adding an artificial tube. - After the Fontan procedure is completed, blood high in oxygen and blood low in oxygen are separated which means that more oxygen can get to his body. This also means that for the rest of his life, our son will only have the left side of his heart pumping blood for his entire body.
Although these three open heart surgeries should greatly improve our son's quality of life, he will need lifelong follow-up visits with cardiologists to monitor his progress. Without a right ventricle that works normally, his heart will never work like a normal heart, which has two pumps.
There are so many unknowns about how our little one will handle his heart once he is no longer safe in my belly and how he will recover from the surgeries. We hang our hats on knowing how blessed we are to have caught this prenatally, as it allows us the time to prepare and give our son the best shot at life as we can. We ask you to please keep our baby and our family in your prayers as we navigate all of this. It has been a lot to take in and we are just at the beginning of this very long journey. We appreciate your support and your prayers more than you can imagine.