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





I have a few questions about PEARS procedure, which we didn't talk about. So Alan, come back. I see you want to answer one of these but is there anything new on the PEARS procedure, which we'd have a talk about it last summer at, at our international meeting. Is there new data? Is it being done in the US? Will it be done in the US?






Well, the PEARS procedure could be performed anywhere. It's, it's for those of you who don't know what it is, it's personalized external, does it mean, aortic root support, right? It's essentially wrapping the aorta and the idea is you're trying to squish it and prevent it from expanding. Now that, that, that's good for sort of preventing aneurysms that will rupture because it's, it won't grow but in theory that will not prevent a dissection because a dissection occurs in the wall of the aorta, so if the wall of the aorta is weakened, necessarily that may not work and sure, it can be done and it's generally done in very, very small aortas that, generally, we wouldn't operate on necessarily at the current recommendations but it is something that to be considered. 


Dr. Moon:

PEARSは場所問わずどこでも実施することができます。ご存知ない方もいらっしゃると思うのでPEARSの説明をしますが、PEARSとは、Personalized External Aortic Root Supportの略で、本質的な部分は大動脈を包むことで圧迫し、拡張を防ぐことにあります。大動脈が膨らむことはなくなるので、動脈瘤の破裂を防ぐ効果はありますが、大動脈解離を防ぐことは理論上できません。というのは、大動脈解離は大動脈の壁で起きるからです。そのため、大動脈の壁がもろくなっていると効果を発揮できないかもしれませんが、施術自体は可能です。現行のガイドラインで手術適応とならない太さでPEARSを実施することになっていますが、これに関しては検討が必要です。


And I'm going to give you a historic example, Albert Einstein had an aneurysm. He went in to get a gallbladder repaired and they found an aneurysm of the aorta and they wrapped it in cellophane, I think, is that right, Wes? 


Dr. Moon:





Dr. Ohman:



They wrapped it in cellophane and two years later it ruptured, so, you know, the wrapping is, is there is some merit to it and certainly it can be done but I think it's not our sort of our standard therapy to get rid of the diseased aorta. Alan, what, what do you think about that? 


Dr. Moon:



I think, I think it's an interesting concept. As Dr. Moon said we don't actually have the exact PEARS protocol. That's done in Western Europe, predominantly England and others, where it was developed, where a three-dimensional mapping of your own aorta is done and personalized, that's why it makes it personalized, surgery is done where the aorta is surrounded by this graft and in that series with very careful, carefully selected subjects they've had really very good outcomes in the, in the short term follow-up. 





And it's just not FDA-approved in the United States right now, so it is currently not available even if Dr. Moon said I want to do that for you he would have to come up with a different way to do it. It's just not an FDA-approved procedure right now for root replacement in Marfan and Loeys-Dietz but it is being done very carefully and as Dr. Moon said generally the aorta's smaller four and a half centimeters or so without a lot of aortic valve disease and again in the, in the follow-up so far in those patients they've actually done very well. 





Looking for something, we're always looking for something less invasive but equally effective and that's what Dr. Ohman and I are just moving forward in that direction. 


Dr. Moon:



Along the same lines, what about the sleeve, I know I'm in Florida, so a lot of people do have what they call the Florida Sleeve, so can you comment on that as well? 



同じような質問になりますが、Florida Sleeveと呼ばれる術式を受けた方が大勢いらっしゃいます。それについてコメントをお願いします。


Yeah, I know, the Florida Sleeve is essentially just a version of, of root replacement with specific techniques and the surgeon who developed that in Gainesville, Tom Martin was a good friend of mine, so it's just the way he does it and this is just a description of the technique so it's, it's, it's essentially a, aortic root replacement with various minor maneuvers. 


Dr. Moon:

Florida Sleeveとは、大動脈基部置換術の方法の一つです。考案したのはゲインズビルのTomas D. Martin医師で、私の親友です。彼が用いた方法がFlorida Sleeveと呼ばれているだけです。大動脈基部を置換する方法には細かい技法が様々あり、そのうちの一つということです。


Okay, great. Thank you very much.





Very effective.


Dr. Moon:



Okay, good to hear, good to hear. 





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。