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.

The Diagnosis:
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.

Next steps:
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.

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7 Responses to Diagnosis

  1. Danielle says:

    I came across your blog via Neysa’s blog…
    I, too, am a heart momma (to a little guy in heaven) and I just want to let you know that you are not on this path alone. I remember the exact feeling of being in your shoes as I prayed desperately for a miracle for my son. The “heart community” is filled with incredible people who will surround you in support, encouragement and love… Please know that we are here. I’ll continue to pray for a miracle, for healing, for health for your sweet, sweet Alayna.

  2. Jessica says:

    This sounds almost to a T like what Greyson has. Check out his blog if you want to know what we went through. We were also expecting a repair after birth but weren’t able to get age that repair as his valves attached through his VSD and they couldn’t see this until he was born. We were also about 2.5 hours away from where Greyson had to be delivered and has all of his treatments. He was going to have the arterial switch surgery a week after birth but instead is having a fontan repair and has undergone a balloon septostomy at 10 days old and his Glenn surgery at 4.5 months old. Reading your story has hit very close to home and in my research I haven’t found too many kids with both double outlet right ventricle and transposition of the great arteries along with Greyson’s other few heart related defects. Looking at Greyson you would never know he has such a complex heart defect and it hasn’t slowed him down at all. In most things developmentally he is ahead of the game. Please read Greyson’s blog I really think his story will set your mind at ease. I blogged nearly everyday of his nicu stay and surgery. Don’t bank on it but we were home in 14 days after birth but we also didn’t have surgery right after birth either. I’ll be praying for you and your family and hope everything goes as well for Alayna as it has for Greyson.

  3. Neysa says:

    At least you have a diagnosis and a plan, now! Big hugs!!!

    We faced the decision of where to deliver, as well. My local hospital has a level IV NICU and my son was not predicted to need immediate intervention. We live 4 hours from MUSC and I really did not want an induction, so I chose to deliver at our local hospital. While Mason was pink at birth, he had a couple of surprises that made me wish we had delivered at MUSC (although our local hospital did a wonderful job with him).

    A big factor in my decision to deliver locally was my (then 18mo old) son. I wanted to keep his life as normal as possible.

    The amnio. We also were encouraged to receive one since Downs, DiGeorges, and CHARGE syndrome often accompany the particular defect my son has (Tetralogy of Fallot). I chose to go through with it because I’m a planner and I really needed to know if there were more to Mason’s defect than just the heart. I still sometimes shudder at the thought of possibly having lost him via the amnio.

    One thing about amnio’s, the safety is directly related to the office that performs them. You need to make sure yours is done via the specialists at MFM. While amnio risks tend to be 1:400, the risk at a specialists office is more in the 1:1000 range. My husband took off work for mine (of course) and when we got home, I got in bed with the laptop and a book. I did not move for an entire day. Even with never moving, I still had some cramping (which is considered normal, but is still very scary).

    You have a large community rooting you on and praying for your sweet girl! Please use us for any and every question you think of! We all had the same questions at one time or another!!!

  4. Joye says:

    I can imagine the strange sense of relief you’re feeling about having a diagnosis. One one hand, it’s nice to have a plan – but, on the other, it’s a plan no mama wants for their child.

    Check out these blogs, if you want … http://whenlifehandsyouabrokenheart.blogspot.com/ – Logan has a very similar heart defect and … thehuegelfamily.blogspot.com/ – Andrew was born 6 weeks early. Both boys are around 4-5 now and doing well!

    Prayers for an uneventful remainder of your pregnancy, and stable ultrasounds.

  5. Tanya says:

    So glad you were able to get some encouragement today; although it is scary, it sounds like you are in very good hands. I will pray that everything goes as planned and for wisdom for you and the doctors throughout your pregnancy, delivery, and after. (((HUGS)))

  6. Andrea says:

    I know having the diagnosis is scary but you are doing the right thing by researching/educating youself and forming a plan. You are a wonderful mama! Since Max’s heart defect went undetected there was never any time (or need) for us to do any research. As far as we knew he was HEALTHY! We literally went from thinking he was healthy to hearing the words “heart failure” all in the blink of an eye. A lot of times I sit back and think about how much “easier” it would have been had we known at birth. We lived with a “healthy” child for 2 months and then had to adjust to life with a child battling a complex congenital heart defect. We were thrown right in and never even given time to accept it. Max was in surgery yet I barely even understood what was wrong with him. It happened that fast… Take advantage of knowing and surround yourself with information. Connect with other parents, build a support system, and grieve over the loss of the healthy child you dreamed of having.. Knowledge is power! *HUGS*

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