Living a Life with LDS (September 12, 2020) E3 Summit 21:38-24:36 What about the distribution of the occurrence of those genes? ではLDSのタイプ分布を見てみましょう。 The most common one is is still TGF-beta receptor 2, which is about half …

Living a Life with LDS (September 12, 2020) E3 Summit 19:56-21:37 Hal made this, a very nice comparative table. We will not go into all the details. I will just point at the main differences. この比較表はDietz先生が作成したもので、非常にわ…

Living a Life with LDS (September 12, 2020) E3 Summit 19:03-19:54 So overall we do have now at least five established genes. Receptor 1, receptor 2, SMAD3, TGF-beta 2 and 3. これまでの研究から、LDSの原因遺伝子として少なくとも5種類（TGFBR1…

Living a Life with LDS (September 12, 2020) E3 Summit 18:07-19:02 A condition that resembles Marfan syndrome and Loeys-Dietz syndrome is called Shprintzen-Goldberg syndrome. Marfan症候群やLoeys-Dietz症候群に類似したShprintzen-Goldberg症候…

Living a Life with LDS (September 12, 2020) E3 Summit 14:48-18:05 So soon after we described it clinically we identified the TGF beta receptors as the causal genes, which we published in 2005. As you can see here on the figure the TGF beta…

Living a Life with LDS (September 12, 2020) E3 Summit 13:15-14:47 Like Marfan syndrome we noticed that there could be skeletal findings including scoliosis and which is a curvature of the spine, chest wall deformities with either the chest…

Living a Life with LDS (September 12, 2020) E3 Summit 9:54-13:15 Hello, good afternoon for the American side. Good evening for the European sides. It's my pleasure to welcome you on behalf of Hal. We, we will give a very brief introduction…

Surgery Series: Aortic Arch (2/22/21) 1:12:49-1:13:42 Q:When you, when you're, when you, when you replace the aorta, this is a good great question, I never saw this one before, when you replace the aorta with the dacron how, what's the dia…

Surgery Series: Aortic Arch (2/22/21) 1:10:22-1:12:49 Q:Can you talk a little bit more about the aortic size, which you mentioned before and you said it's different, different number based on the person's bigger or smaller, so it's so, if …

Surgery Series: Aortic Arch (2/22/21) 1:09:23-1:19:20 Q:So what likelihood is there for scar tissue to develop after a full arch replacement and how would scar tissue impact the patient? 弓部全置換後に瘢痕組織が生じる可能性はどのくらいでし…

Surgery Series: Aortic Arch (2/22/21) 1:07:35-1:09:19 Q:Is this, so this surgery, the aortic arch surgery is just always done in an open open way? Is there any way to avoid open surgery? 大動脈弓部の手術には開胸手術以外の方法がありますか？…

Surgery Series: Aortic Arch (2/22/21) 1:06:30-1:07:31 Q:So if we can talk about Loeys-Dietz for a second. You mentioned that patients having elective root replacement surgery, you generally recommend a total re-implantation of the arch at …

Surgery Series: Aortic Arch (2/22/21) 1:05:13-1:06:20 Q:Just about the vocal cords we can go back to that for a second. Does that, does that, does it go back? （反回神経の損傷による）声帯麻痺は治るのでしょうか？ A:So some patients, it's a …

Surgery Series: Aortic Arch (2/22/21) 1:02:58-1:05:08 Q:We're gonna go to Jill, who asked her a question before we started the presentation tonight. She says her native arch is still intact but she has dacron, dacron everywhere else. Is th…

Surgery Series: Aortic Arch (2/22/21) 1:01:33-1:02:56 Q:If people can't go to hog, I mean, how do people find an experienced surgeon? What should they ask, like they don't know where to go, they need to at least another question so they ca…

Surgery Series: Aortic Arch (2/22/21) 1:00:24-1:01:28 Q:And so and just going back to that brain perfusion right near the end of your talk. You said that mortality was lower if that was done but you didn't give any numbers. People are aski…

Surgery Series: Aortic Arch (2/22/21) 58:03-1:00:23 Q:How long does that surgery take the aortic arch surgery or does it matter which one of, the ones that you showed us. It looks like it looks complicated. 大動脈弓部の手術にはどのくらいの…

Surgery Series: Aortic Arch (2/22/21) 56:17-58:02 Q:So, you know, you talked a lot about the need for aortic arch surgery after somebody presented with dissection. Unless so if it was prophylactic surgery but what are the indications for t…

Surgery Series: Aortic Arch (2/22/21) 52:57-54:11 So what does the future look like? Well in the field of aortic surgery we need to, we need to clean up our act a little bit and there is a consensus statement that tried to do that and that…

Surgery Series: Aortic Arch (2/22/21) 52:34-52:54 Post-operative imaging is critical. With that we need to, we're going to be following echoes, we're going to be following CT scans, some patients can be followed with MRIs. 術後の画像検査も…

Surgery Series: Aortic Arch (2/22/21) 50:50-52:34 And with that the left recurrent laryngeal nerve, which hopefully you guys can see this pointer, but off the vaso, vegus the left recurrent laryngeal nerve wraps around the arch and it usua…

Surgery Series: Aortic Arch (2/22/21) 49:05-50:49 Obviously in the post-op setting there's a variety of things that we're looking for. 術後に確認すべき項目は様々です。 We obviously need a monitor for bleeding when we get up to the ICU. We …

Surgery Series: Aortic Arch (2/22/21) 47:25-49:04 As in medicine there's, if there's something good there's always some bad to it so the colder you go the bigger problems you have with bleeding because the proteins that form coagulation or…

Surgery Series: Aortic Arch (2/22/21) 46:41-47:23 So cerebral monitoring is crucial to achieve our task and that is there's a variety. 大動脈弓部の手術において、脳のモニタリングは極めて重要であり、様々な検査法を用いて行われます。 There's B…

Surgery Series: Aortic Arch (2/22/21) 45:31-46:40 One of the ways we can do that, as we showed, is the right axillary perfusion so this is where we're instilling flow usually through a graft from the heart-lung machine. 先ほど順行性脳灌流…

Surgery Series: Aortic Arch (2/22/21) 43:38-45:29 But hypothermia is not the end-all and be-all. You need adjuncts and adjunct is perfusion to the brain and this is a concept that we've has been around and this is kind of like religion in …

Surgery Series: Aortic Arch (2/22/21) 41:09-43:36 So back to the original question, "Why is arch surgery different?" Well, we're balancing perfusion to the heart as well as blood flow to the brain and then blood flow to the rest of the bod…

Surgery Series: Aortic Arch (2/22/21) 39:13-41:07 Here's a picture and some video of a 19-year-old Marfan's patient, who came in a while ago. He had a root aneurysm that hadn't been operated and you can see his initial presentation was a t…

Surgery Series: Aortic Arch (2/22/21) 37:36-39:12 The sun procedure so this is, you know, one thing that's talked about in the aortic community and I gotta say it's a common procedure that a lot of us do for aortic dissections. How that's …

Surgery Series: Aortic Arch (2/22/21) 35:44-37:35 The elephant trunk procedure, this is, this is important for our connective tissue disorder patients, especially Marfan’s and Loeys-Dietzs where they, they have risk for intervention on the…