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。

循環器

大動脈の画像検査 ~MRAでみる大動脈解離~

Aortic Imaging: Your Questions Answered 18:22-19:57 How does dissection look by MRI, so this is an example on the right, left side top you see this cartoon with blood flowing through it and you're acquiring this angiogram multiple times an…

大動脈の画像検査 ~各種MRAによる大動脈撮影~

Aortic Imaging: Your Questions Answered 15:38-18:21 How is it done on MRI, which is MRA, MRA is a form of MRI but it's just magnetic resonance angiography where you give contrast and you can basically image the aortic root like here and th…

大動脈の画像検査 ~心電図同期CT~

Aortic Imaging: Your Questions Answered 14:15-15:35 One important point if you take anything from this presentation is, this is probably the most important point. A lot of times the scans are done without gating. What do I mean by that? So…

運動に関する最新研究 ~大動脈解離後の運動に関する臨床試験~

Exercise and Marfan, LDS, and VEDS: The Latest Research 49:27--57:20 So again, I think it's a great time now to study this in a prospective manner and gather information that can help prove this sort of information, is not only effective a…

運動に関する最新研究 ~大動脈解離後の運動に関する研究~

Exercise and Marfan, LDS, and VEDS: The Latest Research 46:08-48:06 Now we're going to move on to the last part of our discussion before we open up the questions and this is a topic that's very unique and something that we're all very inte…

運動に関する最新研究 ~小児の運動に関する研究(Science in Paris 2022)~

Exercise and Marfan, LDS, and VEDS: The Latest Research 27:26-34:46 OK, Siddharth, that was outstanding, very, very informative and to summarize the animal model studies of genetically programmed Marfan-like mice, aorta's benefit from, fro…

運動に関する最新研究 ~大動脈解離を発症した集団の運動に関する結果~

Exercise and Marfan, LDS, and VEDS: The Latest Research 25:22-26:02 We, we also looked at the aortic dissection subgroup. I mentioned that was a minority of patients but we wanted to make sure that was safe and effective for them. We did n…

運動に関する最新研究 ~大動脈疾患患者の運動に関する研究~

Exercise and Marfan, LDS, and VEDS: The Latest Research 17:48-25:19 So our group has for the last two years studied the safety of exercise for our patients who come to clinic with thoracic aortic disease of all types and we started to do t…

運動に関する最新研究 ~血管に関する基礎知識~

Exercise and Marfan, LDS, and VEDS: The Latest Research 6:04-8:15 So as a bit of background this is a cartoon of a blood vessel wall like the aorta or another blood vessel and if, the thin lining layer is called the intima an endothelial c…

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) ~産後の授乳による大動脈解離リスク~

Family Planning and Pregnancy: US and Europe Perspectives 59:11-1:01:14 But one, one area of risk that has been focused upon is breastfeeding, so oxytocin and is a chemical released endogenously in each person that stimulates both labor an…

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) ~産後の画像検査~

Family Planning and Pregnancy: US and Europe Perspectives 57:41-59:10 Overall the majority of aortic complications occur in the third trimester but it's also the postpartum period and that's something we haven't discussed at all yet, so wh…

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~

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…

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) ~妊娠中の心エコー・MRI・CT検査~

Family Planning and Pregnancy: US and Europe Perspectives 43:50-44:56 So all our patients with aortic disease should be seen on a regular basis throughout the pregnancy for evaluating the aortic root and echocardiography is the preferred d…

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) ~現行のガイドラインによる治療の転帰~

Family Planning and Pregnancy: US and Europe Perspectives 42:24-43:49 So does it pay off? Well, there are a few studies who actually evaluated the safety and the course of pregnancy in patients who are treated according to the current guid…

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) ~妊娠中の服薬~

41:21-42:23 So one of the previous speaker already discussed the use of the prophetic use of beta blocks in pregnancy. Well, some studies actually observed a reduced rate of progression of aortic dilatation, so according to the European gu…

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) ~妊娠中の画像検査~

Family Planning and Pregnancy: US and Europe Perspectives 39:56-41:20 So patients with aortic pathology should be overseen in specialized centers and supervised by both cardiologists and obstetricians who are actually aware of the potentia…

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) ~妊娠中の血行動態変化~

Family Planning and Pregnancy: US and Europe Perspectives 38:42-39:55 So there is an increased risk for aortic dissection in pregnancy, which is the result of both hemodynamic, hormonal but also from thromboembolic changes and actually pre…

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) ~医療チーム~

Family Planning and Pregnancy: US and Europe Perspectives 37:12-38:41 I will continue this webinar with the next topic, "the follow-up during pregnancy," so in the coming minutes I will discuss when we should see our patients and who actua…

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) ~循環器科のまとめ~

Family Planning and Pregnancy: US and Europe Perspectives 34:58-36:47 And then to end this presentation I just want to mention that every woman wanting to become pregnant should have a cardiac ultrasound done prior to pregnancy to look at …

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) ~前向き研究のデータからわかること~

Family Planning and Pregnancy: US and Europe Perspectives 34:13-34:57 So very briefly some prospective data and I'm going to skip this slide because I know that Martijn is going to address this one but I just want to mention with you that …

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) ~大動脈基部置換手術後のリスク~

Family Planning and Pregnancy: US and Europe Perspectives 32:58-34:12 So even after aortic root replacement because if you recommend replacement before pregnancy you should be aware that you are still not in an absolutely safe zone, so the…

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) ~予防的大動脈基部手術の基準値~

Family Planning and Pregnancy: US and Europe Perspectives 29:49-32:57 So how can we reduce the risk? As I mentioned knowing the diagnosis is really, really paramount. Pre-pregnancy counseling as Melissa has nicely explained. Follow-up duri…

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) ~リスク推定~

Family Planning and Pregnancy: US and Europe Perspectives 28:52-29:49 So how can this risk be estimated? We use risk estimations and this is the most widely used risk estimation. The, WHO risk estimation where you have categories going fro…

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) ~大動脈解離のリスク因子~

Family Planning and Pregnancy: US and Europe Perspectives 26:21-28:53 So risk factors in the setting of, of, of Marfan disease and related heritable thoracic aortic diseases diagnosis of the aortopathy is really crucial. Most women in seri…

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) ~大動脈解離のリスク~

Family Planning and Pregnancy: US and Europe Perspectives 22:52-26:21 I will be addressing firstly what is the risk that we are talking about, how can we estimate the risk, how can we reduce the risk and I'll be addressing prophylactic sur…

大動脈疾患に関する研究報告(Science in Paris) ~二尖大動脈弁~

Aortic Disease Research (9/19/22) 34:55-38:43 So bicuspid aortic valve is a very common inherent disorder of the aortic valve and a certain percentage of patients 10 to 35 percent also have an abnormal, abnormal aorta. I think it's estimat…

大動脈疾患に関する研究報告(Science in Paris) ~大動脈解離サバイバーが望んでいること~

Aortic Disease Research (9/19/22) 20:42-23:20 So, this was a very interesting analysis. Sherene Shalhub and others led a large group of patients to try to understand from patients and families, what, what are the research priorities that y…

大動脈の画像検査 ~CTでみる大動脈解離~

Aortic Imaging: Your Questions Answered 13:28-14:14 The other image I want to share here is how does a dissection look on CT. So this is basically what we call a snake view of the aorta, whole of the aorta. On the top is the root as you co…