The Low Down on Baby K's Heart

On Monday, Dr G noticed a problem with Baby K's heart during our amnio. His preliminary diagnosis was Hypoplastic Left Heart Syndrome. You can read more about it here. But basically, he noticed the left side of his heart was significantly smaller than the right side, mostly the left ventricle. With this defect Baby K would not have been able to survive without immediate intervention which included open heart surgery shortly after birth (and 2 more open heart surgeries over the next few years). Joe and I were beside ourselves with worry. We were referred to a pediatric cardiologist to get an official diagnosis.

We saw Baby K's new cardiologist today, Dr P (yes, he will need a cardiologist, probably for his whole life). We had a fetal echocardiogram and a consultation afterwards. He has not ruled out HLHS since he does have most of the markers for it, but says he is optimistic that baby boy does not have it. Here is what he thinks the problems are and what we would do for them (Dr P drew the pictures for us, I added the arrows so it would be easier to explain):

Normal baby's heart (while in utero)

Baby K's heart

Red Arrow: Hypoplasia of the left ventricle, basically his left ventricle is significantly smaller than the right. The left ventricle is responsible for getting oxygenated blood to the body. The good news is that the left ventricle is completely developed and is functioning, it's just smaller and will have to work much harder. The hope is that after birth, it will be able to continue functioning and be able to support the body's needed blood supply.

Green Arrow: Hypoplasia of the mitral valve, this is the opening through which blood travels from the left atria to the left ventricle. Because his valve is small, less blood can get through to the ventricle. Dr. P does not see this as a major issue right now, and will just keep an eye on it.

Blue Arrow: Aortic Stenosis, the valve going from the left ventricle to the aorta is slightly small, but the main problem is that it isn't always opening completely. This restricts the blood coming from the ventricle and causes the ventricle to work harder than normal. If this remains a problem, the valve would need to be catherized. His valve is also bicuspid, which means it has 2 flaps instead of the normal 3.

Purple Arrow: Hypoplasia of the aorta, baby boy's aorta is narrower than normal, but Dr P feels that is still an adequate size and is hoping that by correcting some of his other issues that the aorta will eventually get larger.

Yellow Arrow:Coarctation of aorta, this is a severely narrowed part of baby boy's aorta. Because his aorta narrows some of the oxygenated blood is flowing backwards (Turquoise Arrow). The location of the narrowing will severely affect the blood flow to the lower portion of his body after he is born (about arm pits down). So he will receive prostaglandins (a med) to keep his PDA open. Some of you may remember Julianna had to have medication to close her PDA after birth, how odd this baby will have meds to keep his open! Permanently fixing this would require surgery. An incision would be made under his arm and they would go in to correct the problem that way.

So, this is the plan as of right now...Dr P is contacting Dr O to decide when I should deliver (Dr P wants to wait longer for baby boy, but Dr O doesn't want to wait because of my risk for rupture). Once Baby K is delivered, he will immediately be transferred to A.C. Hospital for observation. At this time a better diagnosis can be made (since they won't be going through me to see his heart). If Dr P is correct about the above diagnosis, then baby boy will have those interventions and will hopefully only have a 5-10 day stay at the hospital. If they determine that it is indeed HLHS, then he will have the first of his open heart surgeries and be at the hospital for much longer. The more optimistic diagnosis would leave Baby K with very few life-long issues, HLHS would mean a lifetime of meds, possible surgeries and the worry of complications.

So even though Dr P is "very optimistic for this best case scenario" (his wording) that it is not HLHS, please keep praying for Baby K because we will not know for sure until after he is born.

(Sorry for the giant paragraph, Blogger is being dumb tonight!)