You take the good with the bad....

Della had her first appointment with her new cardiologist yesterday.  We learned some potentially good news and we learned some bad news.  I'll start with the bad news to get it over with. 

When the cardiologist first listened to her heart he mentioned that he thought he heard a small murmur but that considering that it hadn't been noticed before (and she has an excellent pediatrician) it was most likely due to the amount of Albuterol she is on (and no, I have no idea why that would cause a murmur?...should have asked).  But after taking her history and talking to us about her symptoms he wanted to run a transthoracic echocardiogram.  This test was not at all painful for her but she wasn't too happy about it either.  Even the Baby Einstein movie they had showing wasn't interesting enough to keep her from trying to get away. 

The bad news is that Della has an Atrial Septal Defect (or ASD for short). 

(You can read more about this here)

Della's ASD is considered "moderate" and will be monitored.  Over time if it is left untreated the right atrium will become enlarged due to the extra blood flow into that chamber.  Most likely when she is 3 or 4 years old they will go into the heart with a catheter inserted in an artery in her leg to fix the hole.  This will be relatively easy and will not involve a stay in the hospital.  In the cardiologists words "She'll go in for the day and go home fixed with a bandaid on her leg."  That is pretty amazing.  So really, the "bad news" wasn't really all that bad.  

As for the potentially good news.  While the doctor was looking at her heart he said that he was not able to locate a left aorta.  What this means is that she may not, after all, have a double aortic arch.  He said that doesn't mean it isn't there, just that he couldn't see it with that test.  There is definitely something causing all these troubles to happen.  Based on what he was able to see he is suggesting it might be something he termed "Right arch with aberrant left subclavian artery".  This is still a type of vascular ring defect it's just different from a double aortic arch, and as I understood it, involves a much less invasive surgery to be corrected.  

(Image credit)

If this is the defect that Della has they will still have to fix it surgically but they will only have to go into her chest and cut ligamentum arteriosum (which you can see in the picture above).  Cutting this will allow the left subclavian artery the flexibility to move back and stop putting pressure on her esophagus.  This is obviously much easier than having to go in and remove a piece of the aorta.  

So where do we stand right now?  Basically we are still waiting.  We have the doctor who ran the Barium Swallow test who thinks she saw a double aortic arch with the x-ray, and we have the doctor who ran the echo test who thinks he saw only a right arch with aberrant left subclavian artery.  We don't really know for sure either way.  Both tests have large error margins.  We also don't know for sure, but we were told the doctor who did the Barium Swallow thought part of the aortic arch was going between the esophagus and the trachea rather than around them both.  So we don't know what will be involved if the left subclavian artery is between those structures?  Surely it will have to be removed, but how?  And how risky is that?  We simply don't know.  

From here we will have to wait a couple of weeks until we can have the CT with contrast run which will answer all these questions for us.  It is so incredibly frustrating feeling that you finally have an answer from doctors who don't make it sound like there is a question as to what her diagnosis is, to going back to not knowing what is wrong with her. 

Silly baby, just playing with her shoes and waiting for the doctor!