There is so much I want to share this evening. On the other hand, I am beyond exhausted and organized thoughts are few and far between right now. So, I will try to land somewhere in between – which will be mainly facts and I’ll leave the ‘color’ for another day.
Alayna is considered to have a Major Congenital Heart Defect. The main issue is a condition titled Transposition of the Great Vessels where her two main arteries are attached to the wrong chambers of the heart (they are transposed). She also has a VSD (hole in her septum – the wall that separates the chambers of the heart).
In a nutshell, the condition means that her blood is not circulating properly. Blood should go into the heart, to the lungs to get oxygen, and then back out to the body. Since her vessels are reversed, the good oxygen rich blood only circulates between her heart and lungs and the bad oxygen poor blood continues to circulate throughout her body.
The VSD in this case is actually good because it allows for some of her blood to mingle as it flows through that hole between chambers. It is not being pumped right, but some natural mixture does occur. There is also a hole at the top of the heart (every baby has this) that helps with this interaction.
Alayna’s heart is also in the wrong position (more midline than left), but that should not affect her treatment. The pediatric cardiologist also wasn’t 100% sure if she had a double outlet. In a regular transposition of the arteries, they two main blood vessels are each attached to a different chamber, just the wrong chambers. A double outlet would mean that they are still transposed, but both attached to the same chamber. Future ECHO tests should clarify which one she has, but again the treatment is the same.
The Good News:
All of Alayna’s major organs (aside from the heart) appear to be perfect. All of her heart ‘pieces’ appear to be present, just not connected properly. The small veins appear to be correct, the valves appear to be correct and not ‘leaky’. The contraction strength of her heart is good. There doesn’t appear to be any immediate risk to her at the moment, only when she is born and the umbilical chord is clamped.
Treatment immediately at birth:
That hole I mentioned at the top of her heart (the one every baby has) typically closes naturally at birth. She will need immediate medication to keep that hole open and possibly a procedure to enlarge that hole. This allows more of a mixture of the oxygen rich and poor blood to mingle in the heart.
It is possible that she can be delivered near home because of the advanced NICU at Spectrum Health (20 minutes from home). She cannot be delivered anywhere other than a hospital with an advanced NICU. She will mostly likely have the ‘blue baby syndrome’ due to her poorly oxygenated blood. We are looking at the situation where she will be immediately stabilized after birth rather than given to mommy.
Treatment her first week:
This condition does require heart surgery within the first few days after she is born. It is not something that will fix itself or something she will grow out of…she will need surgery. In most cases this is a one time surgery to rearrange the arteries. The surgeon would also close the hole in her septum at this time. This surgery would be done in Ann Arbor, about 2.5 hours away from where we live. We would expect her to be there about a month after the surgery.
If possible, she will be delivered and treated at Ann Arbor. The difficulty would be in how quickly labor progresses. Unless we are in Ann Arbor when I go into labor, we’re looking at a 2.5 hour drive. Since she must be born either here or there, nowhere in between, it really depends on my labor.
We will be scheduled for another fetal ECHO down in Ann Arbor sometime in the next month. The doctor referred to this as a ‘second opinion’, but it really is just another check/confirmation of what we already know and to check to see if anything has changed.
An amniocentesis test is strongly recommended (basically required) to check to see if Alayna has any chromosome abnormalities that might further complicate her condition. There are risks involved, but we understand that the surgeons need to be working with complete information as well. We are not sure when we will do this test (the timing was left open to us).
Points of Prayer:
The amnio test – for Alayna’s safety during/after the test and for the results
That the doctors do not discover any other abnormalities in her organs in subsequent ultrasounds (of which I’m told I’ll have plenty).
A premature birth would be disastrous for Alayna. Since her heart is not functioning properly, poorly developed lungs would make a bad situation far worse…almost impossible to survive.
There are far more, but those are the immediate concerns that we have.
Our main take aways:
We are greatly encouraged by the positive outlook that the doctors seem to have at this point. However, we do not want anyone to misunderstand – this is a big deal and everything has to go right for Alayna to have a fighting chance. Without any further complications, her prognosis is really good. It is, however, open heart surgery on a newborn. Any other complications and her chances will drop drastically.