Secondly, I am not a scientist, MD, or otherwise, so bear with me on this.
To begin, Chloe has what is called Tricuspid Atresia {aka missing right ventricle/bottom right chamber of heart}, has a ventricular septal defect {the right and left ventricles have a hole that allow blood to flow through instead of the normal separation between the two}, and an extra superior vena cava.
Here's a quick overview from Mayo Clinic about Tricuspid Atresia and the problem it presents:
Tricuspid atresia occurs during fetal growth when your baby's heart is developing. While some factors, such as heredity or Down syndrome, may increase your baby's risk of congenital heart defects, such as tricuspid atresia, the cause of congenital heart disease is unknown in most cases. (Note: In Chloe's case, this was caused by her abnormal chromosomes/related to Trisomy)The normal-functioning heart
Your heart is divided into four chambers, two on the right and two on the left. In performing its basic job — pumping blood throughout the body — your heart uses its left and right sides for different tasks. The right side moves blood to the lungs. In your lungs, oxygen enriches the blood, which then circulates to your heart's left side. The left side of the heart pumps blood into a large vessel called the aorta, which circulates the oxygen-rich blood to the rest of your body. Valves control the flow of blood into and out of the chambers of your heart. These valves open to allow blood to move to the next chamber or to one of the arteries, and they close to keep blood from flowing backward.When things go wrong
In tricuspid atresia, the right side of the heart can't properly pump blood to the lungs because the tricuspid valve, located between the upper right chamber (atrium) and the lower right chamber (ventricle), is missing. Instead, a solid sheet of tissue blocks the flow of blood from the right atrium to the right ventricle. As a result, the right ventricle is usually very small and underdeveloped (hypoplastic).Blood instead flows from the upper right chamber to the upper left chamber through a hole in the wall between them (septum). This hole is either a heart defect (atrial septal defect) or an enlarged natural opening (foramen ovale) that is supposed to close soon after birth. If a baby with tricuspid atresia doesn't have an atrial septal defect, the baby may need a procedure to create this opening.Once the blood flows from the right atrium to the left atrium, the heart's left side must pump blood both to the rest of the body and to the lungs. The blood reaches the lungs from the left side of the heart through another natural opening between the right and left sides (ductus arteriosus) that is supposed to close soon after birth. When necessary, this passageway is kept open using medication in babies with tricuspid atresia.Some babies with tricuspid atresia have another heart defect — a hole between the lower two chambers (ventricular septal defect). In these cases, blood can flow through the hole and into the right ventricle, which pumps it to the lungs. http://www.mayoclinic.org/diseases-conditions/tricuspid-atresia/basics/causes/con-20026734
Below is an illustration from our fetal cardiologist of Chloe's heart (left) and normal heart (right). I put them side-by-side so you can see the differences in the two hearts. Click on the image to view larger.
Treatment:
1) As described by the yellow star description, Chloe will be closely monitored her first couple of weeks in the NICU once she is born to determine if and when she would need the first surgery. If that duct is staying open, then she could potentially come home with us around 2 weeks old. We would have constant contact with nursing staff at Children's to keep them posted on her stats/medications/etc., and especially to keep an eye on if she is turning blue (oxygen not getting to lungs). Otherwise, if the duct is closing as it does in most normal babies, then they would do surgery around 2-3 weeks (unless complications mean sooner) with about a 3 week recovery time (so looking at roughly 5-6 or 7 weeks total in NICU before coming home).
2) If Chloe doesn't get the shunt, next surgery would be around 6-12mo and reroute her heart. If she has the shunt and it is working, this could possibly be more like 12-18mo old by the time she'd need surgery.
3) Another surgery around 3-4 years old where they will reroute heart, closing off flow to the left side so that no purple blood exists anymore. We have a page that goes with this, but will need to scan it in yet, however it does give detail as to the 3 surgeries.
4) Around the age of 20-30 years, her heart would begin to decline and have trouble pumping, so at this point heart transplant is the treatment. Then again, they have patients who were born with this 30 years ago and are doing well with each new stage of technology. Chloe's fetal cardiologists predicts in the next 20-30 years we'll be growing hearts from stem cells. We pray the Lord grants knowledge in the area of science and this is the case!!!
In the meantime, according to her fetal cardiologist, Chloe should have a fairly normal life, and can even play some sports. She would obviously be monitored often during childhood, and up to yearly I believe after the 3rd surgery. We're also thankful that these surgeries can be done at Children's in KC, as well as her follow-up appointments in between.
Praises & Prayers:
*Praise that it sounds like if that duct stays open, she might not need the first surgery! Pray this is the case for her. Praise also that it means we can see her after she's born, and as long as there are no complications from birth, she'll have those first couple of weeks to put on more weight and grow stronger before surgery.
*Wisdom for the doctors come time that she's born as the monitor and discern if and when to have that first surgery.
*Pray the Lord would go before us and help us with the knowledge we will need to care for Chloe-medications, monitoring, etc. when this time comes.
Additional Links & Resources:
It's a long journey, Jodie, I am with you all the way. Love you, Mom
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