Unique Anatomy: Situs Inversus and Interrupted IVC

In March, right before my daughter’s fifth birthday, I wrote about my pregnancy with Lexie. I briefly mentioned she has situs inversus, an incomplete IVC and polysplenia. After that post, I received an email from a woman with a child with these same conditions. She commented that she had never met anyone whose child had those issues. This made me think back to the days when we found out Lexie had these conditions. I would have loved to have been able to talk or even just read up on another child with similar concerns. All I found online were medical journal which were not always easy to understand and sometimes just downright scary. I am writing this for all of those who find out their child has one or all of these conditions.

Situs Inversus

Situs Inversus is a congenital condition where the major organs are reversed or mirrored from their normal positions. This means the heart is on the right (also called dextrocardia). The stomach and spleen are also on the right while the liver and gall bladder appear on the left. This is a rare condition that happens in only 1 in 10,000 births. (There is also a similar condition –  Heterotaxy – where the organs are in different locations than the norm but not an exact reversal. There tends to be a wide variation in heterotaxy and from what I have read it appears to offer more complications.)

While Lexie’s situs inversus was diagnosed prenatally during my first sonogram, many people are unaware of their unusual anatomy until they go to the doctor for another unrelated condition or have surgery.

So what does having situs inversus mean for Lexie? Not much. It means that we need to let doctors know about her condition, so they know where her organs are during an examination. She probably should wear a medical bracelet or keep information with her that states she has situs inversus just in case she is ever unconscious and can’t relay this to the doctors.

It also means that organ transplants could be a problem in the future since most organs are donated from people with the organs in the standard layout. (situs solitus) (I will briefly talk about some of the tests they performed on her at birth related to her situs inversus in my next post.)

Famous People with Situs Inversus

One of the first things I remember reading after she was diagnosed at 16-week gestation was that actor/singer Donny Osmond also had situs inversus. It was not discovered until he had appendicitis as an adult. His doctor originally misdiagnosed it since his appendix was on his left side instead of his right.

NBA Baskeball player Randy Foye (currently a member of the Utah Jazz) and actress Catherine O’Hara (she played the mother in Home Alone) also have situs inversus.

Situs Inversus in Fiction

As an author I have to point out situs inversus can easily be incorporated into your plot. You can have someone survive being shot because their heart is on their right side. Wikipedia use to have a list of notable fictional characters with situs inversus, but it has been since taken off the webpage. I found this webpage that still contains that original list.

Interrupted IVC

At the same sonogram where Lexie was diagnosed with situs inversus, they also noted that her inferior vena cava (IVC) was what they called interrupted. (This too is rare, happening in 1 in 5000 births.) Of all the things diagnosed with Lexie, this is probably the hardest to explain without too much medical jargon.

Diagram_of_the_human_heart_(cropped).svgYour IVC brings blood from your lower extremities and should connect to the right atrium of the heart. In Lexie’s 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.

A pediatric cardiologist did a fetal echocardiogram at 27-week gestation to ensure that Lexie’s blood flow and heart were working properly. He explained it to me using math. If a normal person’s blood flow was the same as 2+2=4, then Lexie was like 1+3=4. You get the same answer but just slightly different way of getting it. In other words, everything was fine in her case.

Again, what does this mean for Lexie? Not much. Doctors will of course need to know about this prior to surgery, but it hasn’t affected her one bit so far. The worse thing has been trying to get health insurance companies to understand that and not deny her coverage which they have on two different occasions.

While neither of these rare conditions affects Lexie’s daily life now, they (along with a diagnosis of a hole in her heart) did point toward her having either asplenia (no spleen) or polysplenia (multiple spleens) which I will discuss in a separate post.

Basically, I guess what I want parents who find out their child has situs inverses to know is that it will have little effect on their child but could point to other conditions. As for the interrupted IVC, in many cases the blood still flows in a manner that works for your child but you may have to see a specialist to verify this.

If anyone has a child with either of these two situations, feel free to contact me. I am not a doctor and have no medical training but I have been there when you are feeling overwhelmed by everything your doctor is telling you.

 

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15 thoughts on “Unique Anatomy: Situs Inversus and Interrupted IVC

  1. yasminmummy says:

    My baby was diagnosed with situs inversus totalis 4 weeks ago at my 20week scan. I was emotionally unstable when they first told be as I’d never heard of this condition before neither did I even expect anything like that to be said to me..but no one does. I immediately assumed the worst until after my referral that same day to saint George’s hospital in london to see specialists so they could examine my baby in my womb, it’s really quite amazing what they can do.

    I was told although he has situs inversus everything was working as it should be just a perfect mirror image to the normal situs solitus. Determining a good prognosis for my baby boy.
    Me & my partner were asked to attend a geneticist appointment the following week & another heart scan at 22weeks due to an expected isomerism which they just couldn’t see at the 20week scan.

    Geneticist appointment was quite pointless as neither me or my partner had any relative history of this condition in our families as far back as we could go, so although we are told its genetic we can’t find any evidence of this so believe it maybe an isolated case.
    Did they give you any explanation to why this happened ?
    Heart scan was the following week at 22 weeks which took an hour along with 5 heart specialists in the room observing the scan (I was so scared) – after the scan she took me & my partner into the consultation room and explained that she could not see any abnormalities with the heart & that it was still the same prognosis as the 20week scan – nothing had changed. Which for me was a sigh of relief as those two weeks leading up to the scan I was petrified as this is my first baby also I’m still quite young (22) & found it all really difficult as I’d never heard of this condition never the less I’m dealing with it, hoping for the best & praying my little boy will be just fine.
    It doesn’t end there though as I have another heart scan at 28 weeks then 32 also in between those appointment I have to see my normal midwife & specialist midwife.. Then once he is born 3 months later I have to go to the Brompton hospital to check his slpeen & the possible chance of (PCD) also known as Kartaganers syndrome which affects 1 in 4 cases of situs inversus. I have my fingers crossed & pray every day.

    I will do whatever it takes to give him the care he needs, as a mother because I love him very much.

    I’ve spoken to people around the world with the condition & also mothers going through it with their children, in the short time I’ve known.
    Everything I’ve heard is pretty good & one girl I spoke to from Northern Ireland didn’t discover she had situs inversus until she was 21! She has led a perfectly normal healthy life.

    I know you have been through the same as what I’m going through now so can understand my feelings, you have clearly done a wonderful job of taking care of your baby girl & I’m sure it’s not always been easy.
    I’m glad I came across your blog as they more I learn the better I can understand this condition.
    Thanks,
    Yasmin

    • Danielle says:

      Hi Yasmin! Just because you don’t know of anyone with the condition doesn’t mean they didn’t have it. I was diagnosed at 19. I had a CT scan for GI problems and voila! I was in there FOREVER by the way because the tech thought he kept messing up on the labeling. That’s something both of your kids should always be aware of. Anytime a test seems to be taking longer than normal I yell for the tech and ask, “Did the Dr. remember to tell you I have Complete Situs Inversus?” I then follow-up with the Dr. to ensure that I won’t be charged for the “extra” scans which are VERY expensive here in the good ole US of A. Anyways when we told my Grandmother about it she said, “Oh I have that!” She only has one organ switched though but she never thought to tell anybody. Maybe because the Dr. didn’t think it was important enough to tell her. I was already born when it was discovered, she was admitted to the hospital and had some tests done. One day her Dr. brings in a group of residents they surround her bed and were looking at her scans and her in amazement. After her Dr. finished his speech they started filing out of the room without so much of an explanation to her as to what it was about. But one stayed behind and asked her if she knew what was going on. When she said no she was told that she had an organ reversed and they were brought in to see. If not for that test and that student she never would have known.

  2. sherin says:

    Hi, recently a friend of mine has been diagnosed with a situs inversus totalis baby at her 20th.week scan..
    Wat all tests did u take?wer those tests really necessary?cud u share sum light on this..it would be of great help..pls

    • There really are no tests to be done due to the Situs Inverses while you are still pregnant. We had a fetal echo cardiogram done but that was because she also had the interrupted IVC. This test ensured that the blood was going to the correct place. We also did an amniocentesis to rule out other chromosomal issues because the two things (the incomplete IVC and situs inverses) pointed to the possibility of other problems.

      Most of the other tests that were done because of the Situs Inverses were done after birth. They did stop her heart briefly to take a picture but I don’t recall if this was because of the hole in her heart or the situs inverses. I do recall they had her do a test on her intestines and digestive system before feeding her. This required her to drink barium solution and then have several scans to watch the process of the barium. They wanted to make sure everything was in order and what went in would eventually come back out – the way it should.

      Just remember there are people out there with situs inverses who don’t even know they have it. If there are no other health concerns, then situs inverses is really no big deal though will be noted on health forms at doctors’ offices and of course when the baby enters school in case there is ever an emergency.

  3. […] I have mentioned numerous times on my blog, Lexie has a variety of medical issues. The only ones affecting her daily life right now are her eczema and […]

  4. Jill Hobin says:

    To shed some light for some . Im 20 years old and I was born with situs inversus. I also was born with kartageners syndrome. As a child i had a lot of infections ( ears and nose) i got sick alot! As i got older my immune system gradually increased. I still get sick more than the average person. My mom how ever found a system to help me not get sick as often. Having a healthy life style and taking things from the health food store such as acidophilus , grape seed extract , vitamin c , fish oils. Eating a diet full of antioxidants! Making those small adjustments has help me so much . I live a very normal life , I work a physically demanding job , and I train for being a runner . The only difference between me and everyone else is my organs are reversed and i get sick a couple more times a year than the ‘normal’ person. So for moms or dads having a baby or whom have had a baby with this condition chances are your baby will live a normal life and probably forget sometimes that they are backwards just like I do . Its a hell of a conversation starter and seems more severe than it really is !

  5. Diane McLeod says:

    My 4 year old son has Situs Inversus with Levocardia. He has a hole in his heart, multiple spleens, and had bowel surgery at 4 weeks old. It has very much affected his life. I think it needs to be clear of the differences between Situs Inversus with Dextrocardia and that of Levocardia. Yes, dextrocardia can leave no long term issues and can go overlooked for years, however my son would have died due to his condition had it not been known at our 20 week scan. I’m fighting to raise awareness of this condition as all I can find online is of people living normal lives with Dextrocardia. People tend to miss Levocardia or Dextrocardia off their condition when actually that’s the part that makes the difference.

    • Ju says:

      Yes my daughter born in 2005 has Levocardia with situs inversus (only 1 in 2 million have this), thankfully she was in the 5% of no congenital heart abnormalities and has a normal functioning spleen with odd mesentary ones.
      Shockingly not picked up on any prenatal scans, had a late scan at 37wks and the radiographer found something, fetched her colleague and they talked themselves out of what they were seeing ie stomach not below heart! So I was oblivious until within 24hrs after her birth she was rushed to special baby care as sick with yellow bile & after tests discovered blocked bowel – diagnosed with congenital malrotation causing the blockage and situs inversus… Major op & 3 wks in hospital and now lives a completely normal life.
      Also, a childhood friend of mine had a baby boy with dextrocardia situs solitus and has had major life-limitations & life-threatening episodes. He’s had major heart surgery throughout his life wasn’t expected to live beyond infancy, then beyond 10yrs, beyond teenage years but advances in medical science and prayer have enabled him to reach young adulthood.

      • Wow. I am glad everything worked out for your daughter. I’m sorry to hear your childhood friend wasn’t as lucky with her son. It is amazing how different each case can be. I am still thankful that everything turned out well with my own daughter.

  6. Sara Rajpoot says:

    Can a person live longer with dextrocardia? ? Does it have any side effect? ? Please do reply me

    • Dextocardia simply means the heart is on the right side verses the standard position on the left. It really doesn’t effect how long you live. As long as everything (veins, heart chambers) are all in order, then there would be no side effects. I found out several of my friends’ kids had dextocardia after Lexie was born (though none of them have situs inversus) . They are all doing fine too.

  7. I have read the S.I. is not uncommon with identical twins–one being normal and the other S.I. Is this true?

    • I have seen mention of “mirror” twins but I still think it highly uncommon. I have read of a few cases where both twins have it or just one but most identical twins don’t have S.I.

  8. […] does have Situs Inversus Totalis, which means her organs are flipped as a mirror image (left to right) of the average person. Her […]

  9. […] On the parenting, I typically look at what is happening with my children – birthday parties, health problems, believing in the tooth fairy and topics like these. I write about what our Parent-Teacher […]

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