米国マルファン症候群患者団体The Marfan Foundationからの情報を中心に、マルファン症候群や関連疾患についての海外情報を翻訳して発信します。


Aortic Root Surgery in Connective Tissue Conditions - Surgery Series (2/10/21)




If it does dissect how long does somebody have to get to the hospital if it's an emergency situation?



You know, it's not quite, it's all individual like, we don't know how many people die at home so there's no, there's no magic number to that question.



But it's a little different than like a heart attack and a stroke so like for heart attack or stroke we talk about how you got to get somewhere within an hour, they talk about the golden hour or 90 minutes or something from, you know, from heart attack door-to-balloon time and all that stuff.



With the aorta usually we try to sort of get someone taken care of if it's ascending aorta, I mean, the sooner the better, of course. Usually we have kind of a window of several hours, like I don't like to see the sun rise or set on someone with a dissection. If they have some extenuating circumstances we have to do some additional testing to do the operation safely and the patient's otherwise stable, you know, we can do that.



If they've got a bunch of blood that's collecting around their heart or one of the blood vessels downstream is compromised not getting good blood flow we got to move faster.



Right, but they should put that timing on you, they should get to the hospital as soon as possible....



Absolutely, get to the hospital as fast as possible and if you have any history of connective tissue disorder even if a family member had sudden death.



It should be on the, you know, we should be thinking about aorta and we've been, we've been doing, you know, with these aortic awareness projects not just, you know, educating the masses but we've been, you know, doing our best to make sure that emergency medicine docs and people in the medical field are also thinking about that.



It tends to, it probably set lower on the differential diagnosis for many years than it should because it sounded like a rare thing. I think it's just way underdiagnosed and as we're starting to get better at diagnosing it it started to kind of rise into the minds of folks and it's got to be on your mind if you're going to make the diagnosis, got to think about it.



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