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

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…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~予防的外科手術の目安~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 20:53-25:55 Dr. DeAnda is going to talk about some of the surgical thresholds. では、DeAnda先生から外科手術のしきい値についてお話いただきます。 Some of this has c…

大動脈疾患の診断と管理に関する新ガイドライン(ACC/AHA)

2022年11月3日 THE MARFAN FOUNDATION 大動脈疾患の診断と管理のための新しいガイドラインが、昨日、Journal of the American College of Cardiology と Circulation に掲載された。この ガイドライン は、ACC(米国心臓病学会)とAHA(米国心臓協会)を代表…

妊娠・出産について(アメリカ・ヨーロッパの専門医の視点から) ~妊娠中管理のまとめ~

Family Planning and Pregnancy: US and Europe Perspectives 50:03-50:59 So I came to the to my final recommendations and as we have seen that pregnancy in patients with aortic disease should be managed by a multidisciplinary team and an expe…

妊娠・出産について(アメリカ・ヨーロッパの専門医の視点から) ~ロイス・ディーツ症候群患者の場合~

49:01-50:02 So to conclude I would also make some, some discuss some aspects of pregnancy in patients with Loeys-Dietz syndrome although the data is much scarcer in this group and of course we know that the risk of aortic dissection during…

妊娠・出産について(アメリカ・ヨーロッパの専門医の視点から) ~妊娠中の心血管手術のタイミングに関するメタアナリシス~

Family Planning and Pregnancy: US and Europe Perspectives 47:35-49:00 Of course, the question of the timing has been evaluated a little bit in more detail in this recently published meta-analysis and in this analysis they analyzed 386 pati…

妊娠・出産について(アメリカ・ヨーロッパの専門医の視点から) ~妊娠中の心血管手術~

Family Planning and Pregnancy: US and Europe Perspectives 44:57-47:34 So I will continue with the final part of my presentation and discuss surgery during pregnancy and of course this is something we want to avoid but unfortunately still i…

大動脈手術の将来について

Advances in the Treatment of Aortic Dissection 58:00-1:00:10 Q: How do you feel about this the state of aortic surgery right now. What are you looking forward to in the future? What do patients have to look forward to? Eileen:大動脈手術の…

大動脈解離手術後の画像検査について

Advances in the Treatment of Aortic Dissection 54:46-56:09 Q: And I'm on the other end, Dr. Ohman, you talked about long-term surveillance after the surgery, so how frequently with what kind of imaging, you know, what do you recommend for …

10代の大動脈手術について

Advances in the Treatment of Aortic Dissection 52:29-53:56 Q:So I know you you both do a surgery on adults, correct? and so some people are asking about some of this information pertains to children as well. Obviously in Marfan and Loeys-D…

PEARSについて教えてください

Advances in the Treatment of Aortic Dissection 48:50-52:29 Q: 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 proced…

ロイス・ディーツ症候群の血管手術において覚えておくべきこと

Advances in the Treatment of Aortic Dissection 46:57-48:47 Q: Are there different recommendations for surgery if a patient is Loeys-Dietz compared to Marfan? Eileen: ロイス・ディーツ症候群の血管手術において、マルファン症候群と比較して、特…

大動脈解離の治療における進展 ~血管内治療(マルファン関連疾患患者の場合)~

Advances in the Treatment of Aortic Dissection 37:01-40:39 Most of the experiences in the literature are single study or single center institution reports with a handful, maybe, two dozen at best and you can see for patients in both dissec…

大動脈解離の治療における進展 ~選択的修復における標準治療(GenTACの声明)~

Advances in the Treatment of Aortic Dissection 34:04-35:31 And so I have to be clear. GenTAC has a statement and, and this is true for patients who are stable and are undergoing elective repair open surgery remains the gold standard. GenTA…

大動脈解離の治療における進展 ~B型解離の手術タイミング~

Advances in the Treatment of Aortic Dissection 31:53-34:04 So when do we make the decision to go to the operating room? If it's an acute, malperfusion issue with solely a type B and they're relatively stable I want to be very clear ―― the …