Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy
18:30-20:57
And these are the different sections that are included in the paper. So you might imagine that the sections on Marfan syndrome, Loeys-Dietz syndrome, vascular EDS and ACTA2 are much longer than some of the others where there's less information to talk about. So in addition to the general sections in the front of the paper, there are specific sections for each of these conditions.
前半では、マルファン症候群やロイス・ディーツ症候群、血管型エーラス・ダンロス症候群などに大きなページが割かれ、それ以外の情報の少ない疾患についてはあまり記載がありませんでしたが、後半では、それぞれの疾患についてのセクションを設けました。
And towards the bottom of the table, we talk about a very common congenital heart defect called bicuspid valve. So there's specific guidance related to that diagnosis, and then there's also a section for what to do in a situation where we know that there is aortic enlargement but we don't know what the genetic cause is.
また、先天性心疾患で非常に多くみられる二尖大動脈弁についても診断に関する指針を設けました。さらに、大動脈拡張がみられるものの遺伝的要因がわからない疾患についても同様です。
And because of the strict word limit that the actual paper allowed, only seven tables, I think we included seven figures and tables, we included an additional 14 tables and figures in the supplement which is easily accessible on the website, the Circulation website. So we talk about things like how these genes present during childhood. We can just go through the different tables. I won't go into all the specifics here.
厳しい語数制限がありましたので、掲載できた図表は7点のみだったと思いますが、付録に14の図表を追加しました。付録はCirculationのウェブからダウンロードできます。こちらには、原因遺伝子別の所見の一覧表などがまとめられています。
There's figures on how to do the echo, how to do an MRI, how to take the measurements, recognizing that it's important to do these measurements in a consistent way. Probably the most useful supplemental table, I think it's the next one, Shaine. This one? Yes. Yeah, so the second to the last one, we give there's a table devoted to when to do CT and MRI. So it's generally less common to do - oh there's the table - yeah, it's less common to do it for Marfan syndrome, much more common to do cross-sectional imaging either CT or an MRI with a Loeys-Dietz diagnosis, particularly type 1 and 2, or a vascular Ehlers-Danlos diagnosis. And then we have specific guidance for how to use the medications as well.
また、付録には、エコーやMRIでの各部の測定方法も載せました。測定には一貫した方法が必要だと考えたからです。最も役立つと思われる表は、CTとMRIの断層撮影に関する表です。マルファン症候群ではあまり行われませんが、特にロイス・ディーツ症候群の1型と2型、血管型エーラス・ダンロス症候群で多く行われる検査です。また、薬の投与方法についても詳細な記載があります。
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。
