Scimitar Syndrome: Anyone else have this?

@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.

Hola! Tengo una sobrina de 38 años de edad, a ella también hace poco le diagnosticaron el Síndrome de la Cimitarra, le realizaron una operación donde le pusieron una especie de manguera en la vena pulmonar, la operación no fue del todo exitosa porque ahora presenta trombos por lo que no la pueden volver a operar.
Quisiéramos saber a ti que te hicieron o dijeron los médicos de las posibles soluciones a este Síndrome.

I have scimitar. I had vein relocation in 2018. I’m 44 and they didn’t figure it out until I was 30. Tons of complications since the relocation.

Hello @mrsneel and welcome to Mayo Connect. This seems to be a rather rare type of heart defect, that is usually found in infants. In reading through some of the posts here, it appears that others, like you, did not know they had this until adulthood.

Here is a link to an article about one woman's experience with this issue,
https://www.heart.org/en/news/2019/02/08/new-workout-routine-helped-uncover-womans-rare-heart-defect
I would like to invite @lisagold70 to discuss this with you as well.

This sounds like a disorder that would be best followed up with an expert cardiologist, and probably one from a research-oriented health care institution, like Mayo Clinic (here is a link for appointment information to Mayo Clinic, http://mayocl.in/1mtmR63) or perhaps a university medical school.

How are you feeling, @mrsneel? What are your most difficult symptoms?

@hopeful33250 Thankfully I am lucky enough to have all of my cardiology care at the Adult congenital clinic at Mayo Scottsdale. I’m a full time patient. My most difficult complication is that I have a very hard time breathing since the vein relocation. Unfortunately I got really bad pulmonary vein stenosis where they attached the pulmonary vein in my left atrium. I never had low oxygen levels before surgery but I was sent home from the hospital on oxygen and still have to have it. They had to place a stent to get blood flow back and I’ve had several surgeries after to open the stent more because of restenosis causing a blockage again. Now I also have severe hypocapnea that took years and lots of tests and different types of doctors at Mayo to figure out. Finally someone decided to refer me to Aerospace medicine and an amazing expert there was able to identify the problem right away. As a result of all of this I’ve also suddenly become intolerant of elevation and I have lots of fainting and horrible breathing side effects from the lack of carbon dioxide. Now I’ve got a new issue with my diaphragm which could be a consequence of relocating the scimitar vein. It’s really disappointing because all of my medical team was so excited to fix my vein and close a PFO. They all kept saying that my QOL was going to be drastically better but it made things drastically worse unfortunately. But I’m that rare person that gets all of the rare diseases that I have almost no chance of getting so it should’ve been a sign that I would get the extremely rare complications that they never see. On top of scimitar syndrome I also have a handful of other very rare diseases and disorders. I see seven different types of specialists regularly at mayo and I have had weekly appointments at the clinic since 2011. My biggest health issue has been that I was diagnosed with Polycythemia Vera when I was 16 and I’m highly symptomatic from that. So the cancer fatigue on top of the lack of oxygen, lack of carbon dioxide and the struggle to breathe combined with type 1 narcolepsy and scimitar syndrome just keeps me constantly wiped out and run down.

I am glad to hear that you are being seen at Mayo. The combination of rare disorders is difficult to deal with, I'm sure.

Do you have limited mobility? How do you keep occupied during the day? Are you able to sleep at night?