Today we had our echocardiogram. It was no beach. It was more like a beach being hit by a hurricane, and us being caught in the eye of the storm. It felt calm – but all around us everything was moving in every which way. I am still trying to figure out where we are.
The Echo showed that baby’s right heart valve is a little bit thickened and that there is a bit of leakage through the valve. Because of this thickening and leakage, there is less blood going to the right side of the heart. Because there is less blood going to the right side of the heart, the right ventricle and pulmonary artery are a bit smaller than the left side of the heart. This is called tricuspid valve displasia.
This on its own is good – if it does not progress. If it stays the same baby won’t need any heart surgery. However, there is a chance – no one knows how big or how small – that the valve will get thicker and thicker. And then it won’t let the blood get into the right ventricle at all. The right ventricle and pulmonary artery will therefore stop growing altogether. This is called hypo plastic right heart syndrome.
Before baby is born, that is not a problem. The oxygenated blood comes from me. However, once baby is born, this will be a major problem because the right side of the heart brings blood to the lungs. Without a functioning right side of the heart, the blood can’t get to the lungs. This is called hypo plastic right heart syndrome. Babies with this type of syndrome needs 3 surgeries – one at birth, one at around 6 months and one between 2 to 4 years of age. The three combined surgeries basically bypass the right side of the heart and the un-oxygenated blood (blue blood) goes to the lungs via gravity drainage. The left side of the heart therefore acts as the one single pump. This procedure is called the Fontan procedure. The cardiologist is very clear that this is not a fix. They have done this procedure for about 20 years, and the long term survival rate is approximately 60-70%. There are some indications that these children have more health issues, and also illnesses affect them more. There also seems to be some correlation with liver issues.
The one major issue for us is that baby also still has the omph. As previously mentioned, some babies with omphs have breathing problems when they are born, and would need to be ventilated. If this is the case, they would not be able to do the Fontan procedure because the procedure requires strong lungs. So at this point, our baby having both the hypo plastic right heart syndrome and weak lungs is our worst case scenario because baby would have no surgical options.
There is also any range in between no problem to worst case scenario. They will figure out if anything is required by the oxygen saturation level in the baby’s blood. If saturation is low they have some options for surgery, and the prognosis for those options are really really good.
So what are our next steps? Other than biting our nails and stressing out, we will also now have more Echos to closely watch the progress of the heart. If we end up with hypo plastic right heart syndrome, then at 32 weeks we will go to Edmonton to meet with the cardiac surgery team for an assessment by them. There is only one cardiac surgical team in Alberta, and they are at the children’s hospital in Edmonton. If by then the heart still looks like it did today, we will be in much better shape and may not need surgery for the heart right at birth. We are also scheduled for an MRI. The MRI is to try to give the doctors a better view of what is going on. They will do the one now, and then one at around 32 weeks so that they can do a comparison and see if everything is developing properly. The MRI will also be a better tool for checking on the lungs.
How do we feel about all this? Like I said earlier I feel like I am caught in the eye of the storm. Everything feels like this surreal calmness, but all around me everything is spinning all over the place. I feel like we are trying to make it through this storm to reach our goal of having our healthy happy child in our arms, but the definition for healthy keeps changing on us, and there is nothing we can do to get us to our target. At the end of the day right now we only have one choice and that is to put on our rain jackets and forge ahead blindly, and hope we make it through the storm.