海外マルファン情報

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。

心血管手術

心臓手術後の運動について

Staying Healthy & Exercising Safely 31:16-32:43 Q: This is a recurring theme, so um, there seems to be a lot of guidance around pre-surgery. What about post-surgery? Does surgery significantly reduce the likelihood of adverse events? 繰り…

マルファン症候群の基礎 〜大動脈手術についてのまとめ〜

Marfan Basics: Taking Care of Your Aorta 56:42-58:39 Another aspect, before pregnancy, if you're thinking about getting pregnant, this is an absolutely important consideration to have. And if you're somewhere in the border, you know there'…

マルファン症候群の基礎 〜大動脈手術について〜

Marfan Basics: Taking Care of Your Aorta 52:51-56:30 The common interventions are typically aortic root replacement, specifically valve-sparing aortic root replacement, which is the most common intervention that we see in patients with inh…

マルファン症候群の基礎 〜大動脈手術のしきい値〜

Marfan Basics: Taking Care of Your Aorta 51:41-52:51 So, when you need surgery, you will see a lot of numbers being thrown around. And you will see these numbers ― you know, these are guidelines, obviously. A lot of this comes from Europea…

マルファン症候群の基礎 〜大動脈手術に向けて大切なこと〜

Marfan Basics: Taking Care of Your Aorta 49:08-51:41 Now what matters, the institution or the hospital matters, and the surgeon matters. But more importantly, I would say the surgical team, which means the anesthesiologist, the critical ca…

マルファン症候群の基礎 〜大動脈手術のための準備〜

Marfan Basics: Taking Care of Your Aorta 46:54-49:08 So your patient or your loved one needs surgery, and you may have genetically inherited aortopathy or some sort of aortic syndrome. So what are your options, right? And so I'd say first …

大動脈画像検査の頻度について

Marfan Basics: Taking Care of Your Aorta 40:37-43:30 Q: Somebody is asking, what advice would you give a person whose aorta does not yet measure 4.5 cm? Like, how often should they follow up imaging? They obviously want to prevent an emerg…

マルファン症候群の基礎 〜マルファン症候群における手術適応基準〜

Marfan Basics: Taking Care of Your Aorta 34:25-35:29 So, how does imaging help guide surgical timing? Basically, if you have Marfan syndrome and a diameter of your aortic root more than 5 cm, surgery is recommended. If you have an aortic r…

A型解離とB型解離の違いについて

Managing HTAD: Key Insights from the 2024 ESC Guidelines 1:05:34-1:07:24 Q: What’s the difference between a Type B and a Type A dissection and how is it related to pregnancy? A型解離とB型解離の違いについて教えてください。また、妊娠との関係…

上行大動脈の手術閾値について

Managing HTAD: Key Insights from the 2024 ESC Guidelines 51:58-53:39 Q: For ascending aortic aneurysms, do the ESC guidelines take us back from as 5 cm threshold for high-volume regional centers towards 5.5 cm as a recommended threshold, e…

2024 ECSガイドラインについて 〜外科手術〜

Managing HTAD: Key Insights from the 2024 ESC Guidelines 38:04-42:10 And you can see this in our guidelines. So surgery is indeed indicated in patients with Marfan syndrome who have aortic root disease with a diameter of 50 millimeters. Th…

胸部手術後の心血管系症状の感じ方について

Tromsø Aorta 2024 Recap with Dr. Kim Eagle 44:10-45:49 Q: After surgery, are the symptoms of vascular disease diminished? 心臓や血管の手術をした後では、心血管系の症状が出ても感じにくくなるのですか? A: Oh, that's an interesting question. S…

Tromsø Aorta 2024 からの報告 ~PEARS~

Tromsø Aorta 2024 Recap with Dr. Kim Eagle 26:49-30:26 There was a really interesting presentation looking at how we approach aortic surgery and there's a fascinating project led by an engineer in Europe who has developed something called …

小児における大動脈疾患の診断と管理に関するステートメント 〜予防的外科手術〜

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 17:38-18:26 And then there's a table on surgery, when to do surgery. Again, the lighter blue is standard risk. If there are high-risk featu…

兄がつなぐ命

2024年2月9日 カレン・ブルックス 謎の診断 未知との戦い 喪失と学び 調査と希望 意義ある目的 私は生まれた時にマルファン症候群と診断されました。突然変異による発症だった父から、4人きょうだいのうちの2人がマルファンを受け継ぎました。今年の夏で82歳…

大動脈基部および上行大動脈の散在性大動脈瘤手術

Surgery for Sporadic Aortic Aneurysms of the Aortic Root and Ascending Aorta On behalf of the GenTAC alliance I'll be speaking on surgery for sporadic aneurysms of the aortic root and ascending aorta. My name is Chris Malaisrie. I'm a card…

ロイス・ディーツ症候群における大動脈基部および上行大動脈置換術に対する手術閾値

Surgical Thresholds for Root and Ascending Aortic Replacement in Loeys- Dietz Syndrome Hi, I'm Alan Braverman. I'm the director of the Marfan syndrome and aortopathy center at Washington University in St Louis school of medicine and on beh…

ロイス・ディーツ症候群における大動脈基部および上行大動脈置換術に対する手術閾値

Surgical Thresholds for Root and Ascending Aortic Replacement in Loeys- Dietz Syndrome Hi, I'm Alan Braverman. I'm the director of the Marfan syndrome and aortopathy center at Washington University in St Louis school of medicine and on beh…

太陽が昇るまで ― A型解離からの生還

2023年9月23日 The Marfan Foundation 「人生が一変したあの瞬間…」 2020年7月31日。僕の人生が一瞬で変わってしまった日。早めの夕食を済ませて帰宅すると、左腕が突然感覚を失った。家はひんやりとしていたのに、汗が吹き出してくる。左腕の感覚はない。妹…

LDSガイドラインの改訂に向けて ~血管の外科手術~

LDS Guidelines: Then vs. Now 39:07-40:01 So in terms of vascular surgery as well we really want to make sure in the upcoming guidelines that we get our vascular surgery colleagues involved. This is a slide again. My, some of you might have…

LDSガイドラインの改訂に向けて ~大動脈手術~

LDS Guidelines: Then vs. Now 7:54-10:15 So one of the big things that differentiated Loeys-Dietz syndrome from Marfan syndrome in the very beginning was this recognition, right? that aortic diameters could show dissections or tears at smal…

PEARS(Personalised External Aortic Root Support)について

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 1:05:49-1:07:41 Q:There's a lot of questions about PEARS surgery. Abe, are you comfortable talking about the PEARS? PEARSについての質問を多くいただいています。DeA…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~大動脈弓部の動脈瘤に関する推奨事項~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 53:22-54:33 And and then finally some patients will have an arch, aortic arch aneurysm and they may have symptoms that are attributed to the aneurysm. If, as long…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~A型解離に関する新たな追加項目~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 51:56-53:18 And then finally and these were some of the biggest talking points that we added on to the, to the regulations for type A dissection. When there is de…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~急性A型解離に対する推奨クラス2Aの推奨事項~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 50:55-51:51 And finally, the, there's class 2A recommendation when it comes to type A aortic dissections and this, this did come from a lot of discussion and that…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~A型解離に関する推奨事項~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 46:22-49:18 For the class, I'm going to go through this fairly quickly. These are the class one recommendations for type A aortic dissections and I've included th…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~妊娠・出産について~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 40:54-46:17 Okay moving on to pregnancy. As Maya commented earlier that the emphasis on shared decision making and the importance of counseling, genetic counselin…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~血管型エーラス・ダンロス症候群に関する推奨事項~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 38:08-40:48 Moving on to vascular Ehlers-Danlos syndrome in, and in vEDS. In imaging the aorta and the branches is very important because it may identify arterial…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~ロイス・ディーツ症候群に関する推奨事項~

30:17-36:57 This, this slide just emphasizes a little bit of difference in management and Loeys-Dietz syndrome, so in Loeys-Dietz syndrome imaging because aneurysm disease is more widespread than in Marfan syndrome that baseline MR or CT a…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~大動脈基部手術の基準値(マルファン症候群)~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 27:36-30:06 Dr. DeAnda is going to walk us through surgical thresholds in Marfan syndrome aortic root enlargement. では、DeAnda先生からマルファン症候群の大動脈基…