17 Years Old: Aortic Root Dilation

Throughout this entire journey, through all of the eye surgeries, unexplained health problems and pain, I still held out hope that maybe this was all a coincidence. Maybe I wouldn’t be in pain forever and they’d find something wrong with me other than a Connective Tissue Disorder…something curable. I know that I have a lot of the symptoms of one and that this would explain things nothing else could, and I think that deep down I knew that this was unavoidably my reality, but like I said; I still had hope.
 
A lot of people know that the biggest worry with some of the CTDs like Marfan Syndrome is the problems they can cause with your heart (and if you didn’t know now you do!). Since they know with 100% certainty I have one of these disorders they have kept a close watch on my heart to make sure that it hasn’t begun to be affected. The main concern is dilation of the aorta. This is when the wall of your aorta (your body’s largest artery) begins to weaken and stretch. This is dangerous because the more dilated your aorta becomes the higher risk you’re at for an aortic dissection, often referred to as an ER staff’s nightmare. An aortic dissection is a potentially fatal medical emergency resulting from a tear in the inner wall of the aorta which allows blood to flow between the layers of the wall of the aorta and force them apart. If the blood tears through all of the aortic walls massive blood loss will occur and the chance of survival becomes slim. Some people may remember John Ritter’s death a few years back as the result of an aortic dissection. That is why diagnosis and proper observation is vital for those with Marfan Syndrome and similar disorders. Though it is still possible those of us who are being monitored will more likely never have to face aortic dissection. Once your aorta is dilated to the point where it may become dangerous open heart surgery to replace the damaged portion is performed. Some famous figures have undergone the same type of surgery (but for other reasons), such as Barbara Walters, Robin Williams, Bill Clinton and David Letterman.
 
I’ve undergone an echocardiogram once every year since I was diagnosed with a tissue disorder to measure my aorta. It is like having an ultrasound on your heart. I am never nervous because for some reason or other -in that department- I’ve always sort of felt invincible and could never imagine the results coming back any other way than fine. I had never even thought for a second something would show up. But I was pulled back into reality last fall when my echo did in fact show that my aorta had become slightly dilated. This is no immediate concern, but it painfully shattered my denial and smacked me into reality -so to speak. I couldn’t look at my mom while we were receiving the results. I knew one look at her reaction and I wouldn’t be able to keep myself composed long enough to get the important information that I’d need and want to know. A few times while the meaning of the results was being explained to us I had zoned out. I wanted to be outside where I could scream. It felt so odd to get such personal, life altering news while confined to a room with a practical stranger. I kept thinking about my mom and how I had to be strong because I knew this news would hurt her more than me. The things I had the hardest time with was knowing that like I said; I wasn’t invincible and the fact that I might one day need heart surgery (which I should mention has a very high success rate), but regardless it is still open heart surgery. Those are some terrifying words when uttered in regards to your future treatment. I will also need to be placed on beta blockers or Losartan at some point to hopefully slow the rate at which my aorta does weaken and dilate. This will slow my body down and take some of the stress off of the artery.
 
As of now I still have a few months left before my next echo and before they will put me on one of those medications. I haven’t been fretting the next echo. I choose not to think of whether or not it will be even more dilated. I just know that for now it is only slight and I should enjoy that for as long as I can. Sometimes the aorta will stay on the outer limits of normal, or in otherwords it will become slightly dilated but never really progress further. And there are enough things ahead of me to worry about when I come to them, so for now I will try to focus on the positive. Though, at times it does sink in, for now I am living as if I had never received this news. My next appointment will arrive soon enough so until then I will pretend all is well. I’m not in denial, I’m just coping. And I think this is a positive way to do so. 
The diagram to the left is one I edited based on my cardiologist’s description of aortic root dilation. He drew where the widened part of my aorta was (in my case the root) and how it would look compared to a normal person’s. The measurement in centimetres are just to give a basic idea of the average root size and the size of one that would be considered dilated. However the size of your aorta is based on height, age, weight etc so what would be dilated to one person may not be to another. All of this is taken into consideration and calculated when measuring the aorta to help determine whether it is a healthy size or not. Mine is just outside of the average limits which is why I say it’s slightly dilated. At the moment it is 3.8cm which is larger than it should be for someone my age height and weight. Usually an aorta is not operated on until it’s 5cm-5.5cm, although it’s becoming more common to have the operation at 4.5cm to reduce the chance of dissection and aneurysm. Either way it would seem I have quite a while before I have to worry about that, depending on if and how quickly my aorta continues to expand. The speed at which it does so is unpredictable so like I said before; I try not to think about it. It isn’t the end of the world, though at first it felt like it. But I have come to accept that this will be a part of my future. 
 
{I am not a doctor and the medical definitions and descriptions featured in this blog post do not and should not replace those of a medical professional. They are merely there to help give an idea of my situation and experiences.}

10 thoughts on “17 Years Old: Aortic Root Dilation

  1. My partner and I absolutely love your blog and find many of your post’s to be
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  2. I have 2 sons, now 19 and almost 21. They both had pectus excavatum repairs and the youngest had a spontaneous pneumothorax, lung collapse, about 1 year after his bar was placed in his chest to correct the pectus. He had gone through a big grown spurt so we thought position of the bar had changed and maybe his lung was rubbing on it possibly on a rough spot to cause it. Was in the hospital a week with a chest tube and recovering. Exactly 2 weeks later he came home from school and said ” I think my lung did that thing again”. Called surgeons office went for an outpatient x-ray and he was right. Because he is tall and skinny they checked to see if the lungs were thin at the top and causing it but that wasn’t it. After going through this and being able to check off multiple other possible Marfan’s symptoms I insisted on getting referred to the Marfan Clinic at Cincinnati Children’s Hospital.
    The first visit was the echo and ekg and visit with geneticist and cardiologist. Measurements of arm span, physical exam looking for flexibility, flat feet, high arch in roof of mouth, all the physical features. THey sent off blood for genetic testing on the youngest since he has more of the character traits. Those came back Negative for Marfans. We needed to follow up in 2 years.
    Two years later we went and the oldest who has a normal low blood pressure had some change in the enlargement of his aorta but nothing they were immediately concerned about and said to repeat visit in a year and this time to do cardiac MRI because the echo was hard to do with their size and the fact that they had a pectus repair and things were not necessarily in the “right” place due to previous intrusion of their sternum and ribs which displaced heart and lungs some.
    Next the youngest, who mind you had just had his 17th birthday they decide to run yet more genetic testing. Increase his visits to every months and start him on a beta blocker, Atenolol. This completely turned his world upside down. Don’t exercise enough to get your heart rate up high???? how? The Atenolol we later realized was the cause of him being extremely tired all the time. He had to attend the last half of his junior year and all of his senior year doing online schooling because he could not maintain a schedule due to the meds. He would wake up with chest pains, nothing of course showed on a holter monitor. He suffered with anxiety and depression and general feeling of being lost. He had been a year round athlete playing soccer and basketball before all of this. He even got to a point where he asked to go see our primary care doctor and get on medication for anxiety and depression which he continued for a few months.
    When we followed up and said he was having problems taking Atenolol twice a day due to sleepiness they switched him to Losartin and his world changed. he can have a more normal schedule although he still has some problems with chest pains and misses out on things he is restricted on doing. He is looking forward to starting college in a few weeks and wants to be a nurse. He already thinkg about when the time comes and he wants to start a family does he want to try to have his own biological children and potentially put them through this or does he want to adopt. The good news is that the Atenolol and now Losartin have put his measurements back within normal limits. He still continues meds and restricts activities but things look better. And he is back to once a year visits and MRI.

    My older son went for a check up and it showed he now has some change in his enlargement and he is being started on Losartin. Trickier with him since he has a normal low BP. He is getting 1/2 the dose of his brother and getting it at night to hopefully not feel the effects.

    The cardiologist and geneticist both agree the boys have some type of CTD but can not determine which one. All the Marfans tests come back normal and EDS doesn’t fit either. The geneticist is taking this as her challenge to find what it is they have but hasn’t gotten lucky yet.

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  3. I know this is a couple years old, but I wanted to say thank you for your encouraging words. My son was diagnosed with EDS and the Dr’s believe he has something else on top of it because he is so symptomatic. We had an echo a couple weeks ago and like you, I didn’t worry. I was thinking it would be the one thing okay because everyone listens to his heart. I was wrong! The cardiologist said he has an enlarged aorta and wants to have a new echo in 6 months and started him on a beta blocker right away. She said it is showing more like Marfans than EDS. It’s in the root just as yours. I would like to have permission to take your pic above to help better educate others and quote your site.
    Thank you for your knowledge and I am going to read more on your blog :)

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    • Hi Angela,

      Thank you for the comments!! You have my permission to use the picture any way you’d like!

      How is your son handling the beta blockers? Have you heard about the research being done on Losartan and it’s effects on slowing aorta dilation? http://marfan.org/marfan/5809/Atenolol-vs.-Losartan-More-Information
      It’s still in the trial phase but it’s very promising stuff, I see my new “adult” cardiologist in a week and will be asking her about putting me on it because I’ve been having a hard time on the beta blockers.

      I’m sorry to hear that you’re dealing with so many medical problems so early on. The only presentation I had at that age of my tissue disorder was my dislocated lenses. Once I hit 10 was when other symptoms began continually popping up.

      Every other doctor I’ve spoken to has had a different opinion from “you definitely have Marfan Syndrome”, “you definitely have EDS” to “we have no idea what you have”. It makes everything scarier doesn’t it, more unknown. It’s also made me question my doctors more than once.

      You can always email if you’d like to chat! katie.robertson@live.ca
      Good luck with everything!!

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  4. That’s scary but see your post was honest, not complaining, this is where people are getting confused…scary and honest doesn’t equal “not upbeat!”

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  5. Pingback: My Life With A Connective Tissue Disorder: A Bit About Me « Connective Tissue Disorders: My Journey

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