Seraph's Heart

Seraph was born with an unbalanced Atrioventricular Septal Defect (AVSD)(aka CAVC - complete atrioventricular canal defect) with hypoplasia of the right ventricle. Also, her heart is mirrored - so she has a left-sided right atrium and a right-sided left atrium, etc..

Stay with me - it's not as hard as it sounds.
"Atrio" - from atria which are the upper chambers of the heart
"ventricular" - from ventricles which are the lower chambers of the heart
"Septal" - from septum which are the dividing walls or the heart

"hypoplasia of the right ventricle" - meaning her right ventricle is unusually small compared to the rest of her heart.



Here's a normal heart:













The blue blood goes out (thru the pulmonary artery) to the lungs where it picks up oxygen.


Here's a heart with AVSD (Atrioventricular Septal Devect) aka CAVC (complete atrioventricular canal defect):





















Remember: Seraph's heart is flipped. So her heart is a mirror image of these pictures. Also, her right ventricle is too small compared to the rest of her heart.



Seraph's first surgery - the Norwood Procedure (11 days old) (not shown in picture - hole between the two ventricles)

a--improve the flow of red blood (oxygenated blood) around the body by attaching the base (Trunk) of the lung artery (Pulmonary Artery) to the body artery (Aorta).

b--provide a blood flow to the lungs through a passage (Shunt), creating a link between the lung artery (Pulmonary Artery) and the body artery (Aorta). The passage is made out of soft plastic (Gore-Tex).

c--I don't think they had to enlarge the atrial septal defect, but I'm not positive.


Seraph's second surgery - Cavo Pulminary Connection (4-6 months old)(not shown in picture - hole between the two ventricles)

--redirect the flow of blue blood (deoxygenated blood) to the lungs by attaching the upper body vein (Superior Vena Cava) directly on to the lung artery (Pulmonary Artery).

The Gore-Tex shunt that was inserted at the last operation will be taken away.








Seraph's third surgery - Fontan Procedure (2-5 years old)(not shown in picture - hole between the two ventricles)
--aims to separate the blue (deoxygenated) blood supply and the red (oxygenated) blood supply

--create a wall (baffle) in the right collecting chamber (Right Atrium) and then attach the chamber to the base of the lung artery (Pulmonary Artery)

All the returning blue (deoxygenated) blood will now be flowing to the lungs, without a pump behind it. This causes an increase in pressure within the lung blood vessels, so, to aid circulation, a small hole (fenestration) may be created in the wall (baffle). This acts as a pressure release valve whilst the child’s body adjusts to their new circulation.


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They monitor the oxygen levels in her blood--when it's too low they know it's time for the next surgery. She'll end up with just one ventricle pumping oxygen-rich blood to her body. Her deoxygenated blood will flow to her lungs without a pump.

She may need a heart transplant in her 20's.

Fetal Echocardiogram...

....which is just a more in depth ultra sound. It was long, but cool. More and more doctors kept coming in the room. Colors flashes on the screen to show you which way the blood is flowing.

Anyway, they confirmed what we already suspected and then talked about treatments and implications. Baby B's heart has 2 problems rolled into one.

The heart on the left is a normal heart. The heart on the right has an atrioventricular septal defect (AVSD). There's a hole between the 2 bottom chambers of her heart. So, the oxygenated blood mixes with the de-oxygenated blood and isn't sent to the right places. ADDITIONALLY(not shown in the picture), her left ventricle (which pumps oxygen rich blood to the body) is too small to work properly.

We already knew the left ventricle was too small. We didn't know about the hole between the ventricles. The small ventricle is the more serious problem. The surgeries correcting that will correct the AVSD, too.

She'll need 3 surgeries - the first before she comes home from the hospital, the second when she's 4-6 months old, and the third when she's 2-3 years old.

She's fine as long as she's still in my tummy because she gets extra oxygenated blood through the placenta. She'll be blueish when she's born. She'll be taken to Primary Children's NICU and cared for there.

-45% of kids with down syndrome have AVSD - ...so we'll put that in the maybe category.
-for reasons unknown to me, kids with congenital heart defects have to brush their teeth more often to prevent infections in the heart valves.


As an added bonus, the cardio team threw in heterotaxy (situs ambigous)--some of her organs are on the wrong side of her body. My perinatologist looked carefully at it today, and thinks it's more likely that she has situs inversus which is where all her organs are reversed - a mirror image of where they should be. This would make our twins "identical mirror twins". We just keep hitting all the low odds! (Haqon wants me to buy lottery tickets!)

heterotaxy would require surgery to make sure there's no danger of intestinal blockage. Situs inversus is kinda cool but not a problem - lots of people have it w/out even knowing it. The biggest problem is your symptoms are on the wrong side of your body. So you could be telling your doc about pain from appendicitis, and he might think it's nothing serious because your pain is on the wrong side for it to be appendicitis.


So...they explained all of this to us.....and then asked us if we'd take a test to determine our stress level. LOL. I don't know about Haqon, but I scored pretty high :)

Thank you for your support and prayers--it means a lot :).
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