対象となる疾患と背景 対象は、マルファン症候群、Loeys-Dietz症候群、血管型エーラスダンロス症候群など、遺伝性大動脈・血管疾患をもつ子どもです。 これらの疾患では大動脈拡大・解離リスクが高く、血圧や心拍数上昇を引き起こしうる刺激薬の使用が懸念さ…

What You and Your Healthcare Professionals Should Know About Marfan Syndrome - Webinar 1:04:21-1:05:08 Q: What are the protocols for someone with Marfan who is unconscious? Is it okay to start CPR? Q: 意識不明のマルファン症候群患者に対する…

What You and Your Healthcare Professionals Should Know About Marfan Syndrome - Webinar 57:41-59:14 Q: My son is currently at 4.5. We have been told repeatedly not to wait until 5.0 to start the surgery process. We would like to be proactiv…

What You and Your Healthcare Professionals Should Know About Marfan Syndrome - Webinar 55:23-56:44 Q: Is there a best beta-blocker for individuals with Marfan syndrome? Should they go the cardio-selective route? What’s the best approach th…

What You and Your Healthcare Professionals Should Know About Marfan Syndrome - Webinar 53:53-54:49 Q: My daughter is four and has only had two echoes. Does she also need a CT? Q: 娘は4歳ですが、心エコー検査を2回しか受けていません。CT検査も…

What You and Your Healthcare Professionals Should Know About Marfan Syndrome - Webinar 35:09-40:06 So, now let's talk a little bit about pregnancy and Marfan syndrome. It's known, obviously, miscarriages happen, but women with Marfan Syndr…

What You and Your Healthcare Professionals Should Know About Marfan Syndrome - Webinar 25:38-31:29 Now that we’re doing a better job of diagnosing people with Marfan syndrome and have better imaging, so we can carefully follow these aneury…

What You and Your Healthcare Professionals Should Know About Marfan Syndrome - Webinar 19:29-25:36 So let’s look very closely to see what exactly an aortic dissection actually looks like. So in this figure, let’s focus first on the left-ha…

What You and Your Healthcare Professionals Should Know About Marfan Syndrome - Webinar 18:35-19:29 What are potential cardiovascular complications? So, first, an aortic aneurysm, approximately 90% of people with Marfan syndrome will have a…

What You and Your Healthcare Professionals Should Know About Marfan Syndrome - Webinar 16:26-18:34 The next topic is medications. The best evidence for medical therapy in people with Marfan syndrome is to use of beta blockers―usually ateno…

What You and Your Healthcare Professionals Should Know About Marfan Syndrome - Webinar 13:46-16:22 So let's now turn to what I’m calling non-surgical therapies. The first category is what we call lifestyle interventions and theses include …

What You and Your Healthcare Professionals Should Know About Marfan Syndrome - Webinar 5:59-13:44 This is a diagram of the aorta, but how do we actually see it in the body? There are three main imaging modalities or tests we usually use. O…

What You and Your Healthcare Professionals Should Know About Marfan Syndrome - Webinar 3:30-5:59 So I have been tasked to tell you what you should know about cardiovascular health, genetic testing, pregnancy and reproductive health, vision…

Staying Healthy & Exercising Safely 43:27-44:47 Q: Should folks monitor heart rate during exercise, and are there general goal recommendations? 運動中に心拍数を測る場合、一般的な目標値はありますか？ A: That’s a great point, and a lot of pe…

Staying Healthy & Exercising Safely 42:13-43:24 Q: Someone is sharing that they're having a difficult time getting family and friends to understand what they can and can't do from a physical activity perspective. It makes them not want to …

Staying Healthy & Exercising Safely 40:29-42:07 Q: Someone wrote in saying that they are a year and a half post-dissection and always tired. They try to walk 30 minutes a day, but they see others post-dissection who are able to do more, an…

Staying Healthy & Exercising Safely 36:25-37:46 Q: For someone with VEDS and POTS, it's very hard to exercise with both conditions. Do you have recommendations on activities that might work? VEDSとPOTS（体位性頻脈症候群）の両方をお持ちの患…

Staying Healthy & Exercising Safely 34:56-36:22 Q: There are lots of questions around specific exercises. If you're willing, I'll try. Okay, the first one: for individuals with VEDS, are abdominal movement-based exercises like crunches, mo…

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…

Staying Healthy & Exercising Safely 20:20-28:15 So, that was the rationale for a randomized control trial which is wrapping up this year. The goal here was really to determine how exercise affects blood pressure and mental health in people…

Staying Healthy & Exercising Safely 15:41-20:19 So, we designed an exercise circuit to test the safety of exercise for patients, initially in our clinic and then in a randomized trial. I'll go through the exercises that we used briefly, bu…

Staying Healthy & Exercising Safely 9:12-15:41 We do have some data coming out. These are observational studies of people who survived dissections, both in IRAD and in Europe, showing that if you are supervised during cardiac rehab after a…

Staying Healthy & Exercising Safely 6:20-8:08 And we also know that not all exercises are created equal, so it may not make sense to put a blanket ban on certain types of activities. For example, blood pressure responds differently to diff…

Staying Healthy & Exercising Safely 4:35-6:19 We know that anecdotal reports have linked some types of exercise, primarily heavy weightlifting, with aortic dissection. And as I mentioned, there isn't a lot of evidence about the safety of e…

Marfan Basics: Taking Care of Your Aorta 1:16:41-1:19:21 So, our studies have shown that athletic activity after diagnosis is possible. There are even people doing competitive athletic activities―these are not officially sanctioned, but th…

Marfan Basics: Taking Care of Your Aorta 1:15:46-1:16:40 So, during our clinical trial, we found that people did generally very well, but the number of, um, kind of problems they had during exercise seemed to be directly related to the num…

Marfan Basics: Taking Care of Your Aorta 1:14:55-1:15:46 So there are really three types of high blood pressure that we're talking about when we talk about aortic disease. One is the condition of your aorta and blood vessels ― so in other …

Marfan Basics: Taking Care of Your Aorta 1:12:22-1:14:55 So, uh, the other thing to consider is that the type of exercise that you do affects the way your blood pressure responds to exercise and your aorta responds to exercise. So we talk …

Marfan Basics: Taking Care of Your Aorta 1:10:11-1:12:20 And so the two kinds of medications we recommend are ARBs or Angiotensin receptor blockers, which you probably have heard of, like Losartan, and beta blockers, like Metoprolol or Ate…

Marfan Basics: Taking Care of Your Aorta 1:09:33-1:10:09 So, again, before starting exercise, it's really important to make sure that your blood pressure is controlled. This is from the guidelines. We typically say, you know, for high bloo…