Scimitar Syndrome: Anyone else have this?

Posted by lisagold70 @lisagold70, Aug 30, 2021

I was diagnosed with scimitar syndrome in 2015 @ the age of 45 and have not found a support group or discussion page for survivors. So if anyone else is a survivor or going through it please comment!

Interested in more discussions like this? Go to the Heart & Blood Health group.

Hello @lisagold70 and welcome to Mayo Clinic Connect. I appreciate your post and question about scimitar syndrome. As I had never heard of this before, I "Googled" the term and found that this is a rare disorder. Here is some information that I found on the National Institute of Health's website. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2813113/.

From your first post, I see that you were diagnosed with this in 2015. If you don't mind sharing more, could you relate what symptoms you were having that led to this diagnosis and what tests were done to confirm it? Have you had surgery or other treatment?

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@hopeful33250

Hello @lisagold70 and welcome to Mayo Clinic Connect. I appreciate your post and question about scimitar syndrome. As I had never heard of this before, I "Googled" the term and found that this is a rare disorder. Here is some information that I found on the National Institute of Health's website. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2813113/.

From your first post, I see that you were diagnosed with this in 2015. If you don't mind sharing more, could you relate what symptoms you were having that led to this diagnosis and what tests were done to confirm it? Have you had surgery or other treatment?

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Yes happily: I was diagnosed in 2015 prior to my diagnosis the only symptoms I was experiencing was shortness of breath and fatigue. I was employed as a CMA for a family practice physician who suggested I use an inhaler before my workouts. I changed employment and was employed for a cardiologist. At the time as I'd room patients I'd identify myself to there symptoms however trying not to be a hypochondriac I brushed off my symptoms. However, my workouts where becoming more difficult because my SOB and fatigue was getting worse. Soon after I started to retain a lot of fluids and gaining weight rapidly. I had a ECG and a ECHO in the office and even though my ECG showed RT heart enlargement the cardiologist wasn't to concerned. By the time I seen my own cardiologist I had another ECG, ECHO, TEE and showed nothing, it wasn't until I requested a cardiac MRI that I was properly diagnosed. Within 5 months of that diagnosis I was in heart failure and needed open heart surgery to create a baffle to allow proper flow from the RT to LT. It followed 2 other open heart surgeries due to stenosis in the baffle that was placed and numerous procedures such as right heart caths, balloon etc. Thankfully Dr. Derani at Mayo Rochester, which I had to fly to from PHX, performed my 3 and hopefully final open heart surgery in 2017!

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@lisagold70

Yes happily: I was diagnosed in 2015 prior to my diagnosis the only symptoms I was experiencing was shortness of breath and fatigue. I was employed as a CMA for a family practice physician who suggested I use an inhaler before my workouts. I changed employment and was employed for a cardiologist. At the time as I'd room patients I'd identify myself to there symptoms however trying not to be a hypochondriac I brushed off my symptoms. However, my workouts where becoming more difficult because my SOB and fatigue was getting worse. Soon after I started to retain a lot of fluids and gaining weight rapidly. I had a ECG and a ECHO in the office and even though my ECG showed RT heart enlargement the cardiologist wasn't to concerned. By the time I seen my own cardiologist I had another ECG, ECHO, TEE and showed nothing, it wasn't until I requested a cardiac MRI that I was properly diagnosed. Within 5 months of that diagnosis I was in heart failure and needed open heart surgery to create a baffle to allow proper flow from the RT to LT. It followed 2 other open heart surgeries due to stenosis in the baffle that was placed and numerous procedures such as right heart caths, balloon etc. Thankfully Dr. Derani at Mayo Rochester, which I had to fly to from PHX, performed my 3 and hopefully final open heart surgery in 2017!

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How wonderful that you persisted and found an answer, @lisagold70. I'm glad that you found a good surgeon at Mayo Clinic in Phoenix. I get the impression that you had to ask for the heart MRI. Is that correct? What made you ask for that?

If you will share more, what was different in the Mayo surgery that makes you think you will not have to have more open-heart surgeries? Also, are you currently taking medications for heart failure?

I hope that your SOB and fatigue are resolved now. Are you able to exercise without symptoms?

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Hello again @lisagold70

Another mentor in Connect, @johnbishop, found a very interesting article about a woman who shares your diagnosis. I found the article very interesting and I think you might as well. Here is the link, https://www.heart.org/en/news/2019/02/08/new-workout-routine-helped-uncover-womans-rare-heart-defect

REPLY
@hopeful33250

How wonderful that you persisted and found an answer, @lisagold70. I'm glad that you found a good surgeon at Mayo Clinic in Phoenix. I get the impression that you had to ask for the heart MRI. Is that correct? What made you ask for that?

If you will share more, what was different in the Mayo surgery that makes you think you will not have to have more open-heart surgeries? Also, are you currently taking medications for heart failure?

I hope that your SOB and fatigue are resolved now. Are you able to exercise without symptoms?

Jump to this post

Yes I did have to ask for the MRI, I figured all other tests preformed, showed nothing, which I didn't mention before I had numerous Chest CT w/contrast prior to diagnosis because ER Dr's thought and kept diagnosing me with pneumonia even though My lungs where clear, but they couldn't explain my SOB. So no test showed anything substantial or heart related and I just knew in my gut there was something wrong and needed answers. Dr. Derani from Rochester was a life saver! There's not a day that doesn't go by that I don't think I'll need another surgery, however at this point I'm happy this has been the longest I haven't needed one! I really think the experience and confidence in Dr. Derani made me believe and I'm hopeful I won't need another surgery but if I do I feel like I'm in the right place in the best hands possible. I am followed by the wonderful team here in phx for my congenital heart issue. I'm also monitored by the heart failure/PH team. Yes I ended up also getting diagnosed with pulmonary arterial hypertension in 2019 due to my congenital heart defect. I am currently taking 2 medications for PH, 2 medications for heart, 1 medication for acid reflux, 1 medication for heart failure. I am able to exercise almost daily, although I do experience some SOB and sleep with O2 I am able to workout. I have attended cardiac rehab and pulmonary rehab at the Mayo so I know my limits. I'm very blessed to be surrounded by such knowledgeable Doctors and staff at the Mayo

REPLY
@hopeful33250

Hello again @lisagold70

Another mentor in Connect, @johnbishop, found a very interesting article about a woman who shares your diagnosis. I found the article very interesting and I think you might as well. Here is the link, https://www.heart.org/en/news/2019/02/08/new-workout-routine-helped-uncover-womans-rare-heart-defect

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That is an awesome article!! It is soo rare I've yet to meet someone with my defect, even tho I know there out there !! Thank you for the article it did bring tears to my eyes just reliving it all through her experience.

REPLY
@lisagold70

Yes I did have to ask for the MRI, I figured all other tests preformed, showed nothing, which I didn't mention before I had numerous Chest CT w/contrast prior to diagnosis because ER Dr's thought and kept diagnosing me with pneumonia even though My lungs where clear, but they couldn't explain my SOB. So no test showed anything substantial or heart related and I just knew in my gut there was something wrong and needed answers. Dr. Derani from Rochester was a life saver! There's not a day that doesn't go by that I don't think I'll need another surgery, however at this point I'm happy this has been the longest I haven't needed one! I really think the experience and confidence in Dr. Derani made me believe and I'm hopeful I won't need another surgery but if I do I feel like I'm in the right place in the best hands possible. I am followed by the wonderful team here in phx for my congenital heart issue. I'm also monitored by the heart failure/PH team. Yes I ended up also getting diagnosed with pulmonary arterial hypertension in 2019 due to my congenital heart defect. I am currently taking 2 medications for PH, 2 medications for heart, 1 medication for acid reflux, 1 medication for heart failure. I am able to exercise almost daily, although I do experience some SOB and sleep with O2 I am able to workout. I have attended cardiac rehab and pulmonary rehab at the Mayo so I know my limits. I'm very blessed to be surrounded by such knowledgeable Doctors and staff at the Mayo

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You must feel so happy to have this resolved, @lisagold70. Please keep in touch and post when you can. I would love to hear how you are doing.

Will you keep in touch?

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@lisagold70

That is an awesome article!! It is soo rare I've yet to meet someone with my defect, even tho I know there out there !! Thank you for the article it did bring tears to my eyes just reliving it all through her experience.

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I'm glad it was helpful!

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@hopeful33250

You must feel so happy to have this resolved, @lisagold70. Please keep in touch and post when you can. I would love to hear how you are doing.

Will you keep in touch?

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Yes

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@hopeful33250

Hello again @lisagold70

Another mentor in Connect, @johnbishop, found a very interesting article about a woman who shares your diagnosis. I found the article very interesting and I think you might as well. Here is the link, https://www.heart.org/en/news/2019/02/08/new-workout-routine-helped-uncover-womans-rare-heart-defect

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@hopeful33250 Hi Terrsa that was an interesting article Thanks for sharing

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@hopeful33250 I have been wondering if you had your surgery and how things went. I just can no longer go through all of the comments to keep up.

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