First cardiology appointments - diagnosis changes

Today we had appointments with pediatric cardiologists at Seattle Children's hospital and Swedish Pediatric Cardiology - and two fetal echocardiograms done. It was a GOOD day.

As a result of these detailed scans and lots more investigation they have given us a much better prognosis than the full-blown HLHS discussed at our first appointment.

It now seems likely that the reduced left-heart measurements are due to an aortal problem - possibly an obstruction or narrowing. The baby has a full aorta (arch etc) but it is half the diameter they would like it to be (2.5mm v 5mm). If the aortal problem is the only one and is the case and the left ventricle and aortal valve remain at approximately 66% of the norm, then the baby will face either a heart catheterization or an aortal reconstruction (a single open-heart procedure) shortly after birth. If things go really well, he may not need the cath till a few months old but the ped card. said this was unlikely because there is quite a lot of turbulance in the aorta - suggesting some obstruction to flow. We may not know how much surgery he needs till after his birth so he will go to the NICU and be watched with a cardiologist on-call to do a diagnostic if/when he shows signs of heart stress. This is a MUCH better prognosis than any we were given before because after the procedure he would have a relatively normal 4-chamber heart and the odds for the surgery are 90%+ success.

The Children's Hospital cardiologist thinks that Seattle Children's Hospital surgeons would be fine to do this surgery but we are going to get other opinions on that when we are closer to delivery. Stanford remains an option if things are not their best. A surgeon in Portland and one at Mary Bridges, Tacoma, were also recommended - depending on the diagnosis nearer my due date.

Now, the worst-case scenario is that the left heart continues to fall behind and/or the aorta do not grow. Then we are back in the HLHS 3-stage open heart procedure. This is possible but LESS LIKELY NOW with the revised measurements!!!

We are going back in a month to 6 weeks for new scans and are desperately hoping that the left-heart measurements are holding up.

Also, he does not have digeorge syndrome! Yay. I am terribly terribly tired (each appointment took 2 and a half HOURS) and am going to bed early.

One week later.. information overload

After my amnio came back fine I thought I could finally relax about this pregnancy. Unfortunately, at a follow-up 21 week u/s the radiologist came in and told us our baby has a serious left-side heart defect and we need to consult a pedictric cardiologist and discuss surgery at birth.

It was a horrible shock. I feel we are in an alternative universe and no-one really gets how this feels.

I have read a lot about HLHS and am feeling terrified but kind of okay about our odds with that diagnosis. Then the PC (whom we will see this Friday) called after reviewing the tapes and said that the HLHS is "not presenting typically" because "there is more left heart than they would expect" but "he can't see the arch properly" etc etc. To cut a long story short he postposed our appointment until this Friday when the best Echo technician will join him to run another study and see what is really going on.

I am just dreading a WORSE prognosis. All these terms I had never heard until last week are rushing through my mind "diGeorge syndrome", "aortic stenosis", "interupted aortal arch". Nothing sounds good.

The only bright moment has been getting in touch with a heart-kids family support group which met on Sunday. I met many families who have survived this and 2 whose kids have had the first two open-heart surgeries of the HLHS three-stage procedure. They were among the sickest kids and had most feeding problems but their parents said they should improve a lot after the Fontan is done in the next year.

Sorry this is so long. I just feel completely overwhelmed with information but have had this awful wait to find out any more about our little boy and it feels good to blurt it all out.