It’s been a whole year, well more than a year now – Raegan just turned 16 months old. I can’t believe it has gone by so fast! Her first words are forming and she is so close to walking now. A little over a year ago we were in a bit of strife. Raegan was diagnosed with left atrial isomerism which is a condition where the organs are not in their correct positions and the heart has some deformities, her heart was looked at from before birth by a Paediatric Cardiologist and we were told that her heart is mid-lined in the body, and that there was issues with IVC, the vein from the heart that drains to the lower half of the body, being blocked.
Long before she was born, at my 20-week scan our doctor used the ultrasound along with an amnio to determine what issues that Raegan may be facing. We were lucky that things weren’t as bad as initially – we did have a bit of worry in the beginning.
Today we had our one year check up with the Paediatric Cardiologist, he gave us the green light and said he’d follow up again in 1-year.
Her IVC, which brings blood from your lower extremities and should connect to the right atrium of the heart. In this case, instead of continuing to the heart, this vein joins another vein (hemiazygos) and continues to the superior vena cava which then connects to her heart.
Somehow in utero when the IVC got blocked, her body made up this new way to connect. Apparently it is still working with no problems.
Left isomerism, is something I’ve been trying to explain here on the blog since before Raegan’s birth. It is the mirror image of the structures on the left side of the chest along the left-right axis of the body, i.e. patients with isomeric left atrial appendages frequently have bilobed lungs bilaterally and each with a long bronchus. Additionally, they commonly have polysplenia and pulmonary veins connecting to both atrial chambers.
Left isomerism, also called polysplenia, is a laterality disturbance associated with with paired leftsidedness viscera and multiple small spleens. Left isomerism, heart congenital abnormalities and gastrointestinal malformation are strongly associated. Raegan has 3 spleens. Many times with polysplenia the multiple spleens do not work properly and it causes a case of immunity to bacterial infections. It can be a very serious condition, but after year of testing we have found that the spleen that is broken into three is still working for Raegan. I couldn’t be more pleased.
In the below diagram, (A) a regular set up for the organs, (B) Raegan’s set up which is a mirror image of the norm.
And in this diagram it shows how the IVC is interrupted or cut off and the new azygos and hypoplasia that the body created to circumvent the problem.
Thanks for checking in on my little blog. I hope that now things have settled, we can concentrate on raising our beautiful daughter and worry less about these issues. Rae is a wonderful, happy, and loving little girl, and the love of my life.