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。


Advances in the Treatment of Aortic Dissection




Dr. Ohman, you mentioned, you said that but the endovascular repair not being for connective tissue disorder patients. There's so many questions about that. Can you explain why and what you worry about and why that's not what you do?



So even as I outlined in my talk, even in the best case scenario for patients who don't have a fragile aorta we're not really seeing the great effect in terms of reduction of mortality, reduction of future events or surgery out until five years in a best case scenario. 

Dr. Ohman:



And what we're seeing really more is a lot of changes for the negative either at the attachment site, Dr. Moon mentioned, the landing zone and that's really critical in terms of when you think about an endograft. You have to find a way to kind of tack it at one place proximately one place distally. 




These devices are still relatively stiff and so that outward force, even if you size it one to one or a millimeter larger than the aorta, you still have this chronic radial force in a, in a weakened blood vessel that can induce a pseudo aneurysm or localized rupture at that site. We've seen that. That can induce tears in the dissection that change the true versus of the false lumen profusion and actually make things downstream in terms of the legs, the intestines, the kidneys worse off than they were before. 




And so, you know, as one of the larger centers in the world for treating patients with connective tissue disorders with stent grafts, yes, I think there's a role for it but it's really, you have to be really thoughtful, so if the patient won't survive open cardiac surgery and we use it as a temporizing measure, yes, that should absolutely be done. If it's a patient who has a surgical graft for their, let's say, they had an interposition for, for a type B dissection done open and they've had the visceral segment replaced and I just need to span to fix an aneurysm between the two surgical grafts that is a reason or that is an indication as well because you're landing in non-threatened tissue, you're landing in dacron that Dr. Moon has sewn in and so you're you're minimizing a lot of the risks but once again those patients aren't out of the woods because we're still, we've still seen aortic events in that stented segment that looks great up to eight years out and so it really has to be judicious.




I know it sounds very attractive and appealing that it's a much less invasive procedure but it's fraught with complications that do require either bigger operation or in some cases an emergency operation after the stent craft has been put in.




Thank you for that a lot of people asking. You're on mute if you want to add something, Dr. Moon?




Yeah, I just wanted to say one thing and, and that's, what's important to have a aortic team that works on these issues so that it's not one or the other but we work together to sort of determine what's the best for that individual patient.




The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.

The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。