So beginning questions are about the studies that you presented early on, talking about the mice because people were putting those questions in while they were listening, so what was the average duration and frequency of exercise that contributed to reduction of dilatation in mice, Marfan mice?
Right, so in, in the Marfan mice this was over five months, which is in mouse terms is a fairly long period of exercise considering that mice live only a couple years and, and the, the exercise was really a treadmill or a wheel in the cage and so the mice would be running on the wheel at different speeds adjusted to different levels of intensity but because, because of the, the nature of the trial it was simply they would record the, the amount of time that the mouse spent on the wheel but there was no enforcement of that, so most, most of the mice were uniformly active but they were free to approach the wheel at any time and so the net effect of this was a period of five months of activity versus no wheel in the cage and, and just regular kind of movement in the the cage.
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
Okay, Siddharth, thank you, so these are our conclusions. I guess we could read them together but I, I guess I will read the final. First and, we will take any questions that we can here at the end, so I'm just going to move on and skip the conclusion so we have a little bit more time. I think the main message that we wanted to give today is that an individualized approach to exercise is, is necessary because there's so many differences and but, but I think the unifying recommendation is to try to be active and to choose an activity that's, that's a safe for you and can be done hopefully it's fun and that you'll continue to do and to discuss with your doctors or practitioners about what does that really look like and if the information sounds uncertain reach out there's great information. The foundation can direct you to others if you need it and there's certainly information in the literature that can help your practitioners about that and there's great web sources on The Marfan Foundation outlining, again physical activity guidelines. It's a downloadable that you can share with your practitioners, nurses, and doctors, etc.
Prakash先生ありがとうございました。こちらのスライドで結論が述べられているのですが、最後に質問の時間を多くとりたいので、手短にお伝えします。本日の重要なメッセージとしては、運動については個別性が強いことから、患者さん一人ひとり違った対応が必要となるということです。とはいっても、統一的に推奨されることは、活動的であるということ、安全な運動を選ぶということ、楽しめる運動であるということ、継続できる運動であるということです。医師などに運動の内容について相談してもよいでしょう。医師からの情報に不安を感じる場合には、The Marfan Foundation が別の医師を紹介することもできますし、文献の中にも専門家が参考にできるような情報があります。また、The Marfan Foundation のホームページにある運動ガイドラインをダウンロードして医療者らと共有することもできます。
This is, these, these, there's one thing that Mary Shepard who is a aortic specialist in Kentucky, who wrote a very nice paper about exercise in heritable disorders and Mary said that, that exercise may help improve the aortic biomechanics and potentially buffer the hemodynamic effects of the inevitable stressors like giving a lecture or running through the airport or other things that can get your heart rate and blood pressure up in life for all of us and and I couldn't agree more and I'm really hopeful that the work that Dr. Prakash is doing and others are doing around the world in this very important area, will improve quality of life and well-being for, for all of us and especially, those who are haven't been exercising because of concerns and so we'll stop there and to turn it over to Eileen, who may moderate some Q&A. Thank you very much.
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
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 and helpful but proof of point. So without further Ado, I'm going to turn this over to Dr. Prakash. He's going to talk about a very exciting clinical trial that he's leading.
Thanks, Alan, yes so our focus has been to extend the results that we that I showed you with our patients who are clinic patients to, to really evaluate what are the independent effects of exercise and in order to do this in a rigorous way we chose a clinical trial format because this is the way that we can use randomization to show that the intervention, in this case, exercise, has a significant effect over everything else that's going on in our patients' lives, which is very complicated. As we all know there are many factors that are going on and it makes it hard to separate the effect of one from the other, so I hope that I can convince you that this format will allow us to do just that and provide some real rigorous objective evidence that this is not only safe but hopefully effective as well for a variety of conditions related to your aortic dissection and health. Next slide, please.
So the objectives of this, this study were are foremost to assess the psychosocial impact of exercise on patients with thoracic aortic disease, as you heard depression and anxiety, PTSD are extremely common after aortic dissection and exercise may be, based on other data, one of the most effective ways to improve those areas of mental health. We would also like to, to rate to use validated tools to rate anxiety, depression and confidence to engage in activities before and after exercise and then we're also going to find out, you know, how, how much time do people actually need to achieve the benefit. One of the concerns would be, is that taking away time from other activities or other, other things that they might want to do and so we can, we can get some as a sense of that from this trial when we're recording the time that they spend. Next slide, please.
So the rationale for this is really that if we, if we provide a guided exercise program to patients after aortic dissection it could increase their confidence but also reduce anxiety and depression and then compared to their usual care. We, we hypothesize that it can also reduce blood pressure as we show, as we discussed before and improve measures that we care about in terms of aortic health. In this case I'll be showing you some measures of aortic stiffness. The wall stiffness really reflects the composition that Dr. Braverman showed you in the beginning of these different layers of the aorta. If a healthy aorta has is less stiff it's more elastic because it has those elastic fibers and maybe exercise could help build that up. Mata, and then we want to show, of course, that moderate intensity exercises are safe and can be performed independently by, by almost anyone who survived a dissection but because potentially this strategy could expand the benefits of guided exercise to people who may not have access to cardiac rehabilitation. As we, as we showed and, you know, we in the, in the current guidelines rehab is now recommended for after all for almost everyone who has survived an aortic dissection and we want this benefit to be available to as many people as possible. Next slide.
So the inclusion criteria for the trial is, are as simple as possible, so anyone who survived a thoracic aortic dissection, it could be a type A or type B, it could be operated on or intervened on or not, at least three months prior to enrollment will be eligible for inclusion. The reason we chose three months is, is to allow time for the acute healing to occur and for patients to complete any immediate rehabilitation that would be required after discharge from the hospital. Next slide, please.
So that there's a raft of exclusion criteria which we will assess but in most cases this has to do with the availability to participate in in-person programs, which would be required for this particular trial because we really want to observe people exercising, provide guided feedback and also measure their blood pressure during exercises I showed you earlier. Next slide.
So this is a complicated slide and I'm just going to summarize. I'm happy to answer questions about it later. You can see on top really, what we're showing from top to bottom is what happens to an individual patient.
So there is one study visit when you would come to clinic physically, be enrolled, and fill out a consent form and questionnaires we would measure your blood pressure and we would randomize you, so you would either be randomized, just a flip of the coin to the guided exercise arm on the left or the control group on the right.
Now if you're in the guided exercise group you will attend several sessions. "1 in-person exercise training session," where we show you we demonstrate how to do the moderate intensity circuit of exercises and this would be very similar to the ones I showed you that we're doing in our, with our clinic patients and then several virtual check-in sessions at home where we assess your, your exercise setup at home and provide feedback and encouragement during the course of the 12 months of the trial, so this is a 12-month trial from enrollment to the final study visit.
And in, in contrast the control group is only going to receive the standard kind of advice that we give at clinic visits which is based on the guidelines that Dr. Braverman shared with you, pamphlets and brochures to discuss the importance of physical activity and exercise. We will ask them how they're doing at their study visits but, but we will not do any in-person or virtual visits, specifically to encourage them to exercise.
And then at the final visit they're going to fill out the same questionnaires to assess mental health and quality of life and we'll check their blood pressure again with the hypothesis that both will be different in individuals who had the intervention. Next slide, please.
That, this is the overall timeline. The, the trial is set to begin in January 2023 and I have to, and thank the John Ritter Foundation for, for us funding. This trial, it is a, is a year-long trial and the, the goal is for each participant to spend a year in the trial but the overall time is 18 months as you see because we will be recruiting in the first six months of 2023. Once recruitment is complete there will be 12 months, up to 12 months of follow-up and the data analysis should be available by June of 2024 when we're hoping to publish the results and you can see the different survey study visits on the bottom at 1, 3, 6 and 9 months. Each individual will have contact with the study team.
臨床試験の全体的なタイムラインがこちらです。試験開始は2023年1月です。1年におよぶ臨床試験に資金提供していただいた John Ritter Foundation には感謝しています。試験の目的は参加者に1年間関わっていただくことですが、最初の6ヶ月は登録者の募集期間なので、全体的な期間は18ヶ月としています。登録完了の時点から12ヶ月間フォローすることになります。データ解析の結果は2024年6月までには入手できるはずで、結果は公開したいと思います。スライドの下に示されているように、登録完了から1・3・6・9ヶ月目に様々な調査が行われます。各参加者は、研究チームと連絡がとれるようにします。
Now we're doing this at three sites, you know, our place in Houston, Washington University in St. Louis with Dr. Braverman and the University of Michigan, so for this trial it will be important that the participants can access one of the three sites in person for some of these or some of these in-person study visits that I mentioned. Other than that we'll be contacting the participants virtually so during that one year you will be responsible for up to three in-person visits only and the rest of it we, we can do from, from home through our virtual connection. Next slide.
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
I'll end my discussion with just one slide from the 2022 ACC/AHA guidelines on the diagnosis of management of aortic disease, which was published this last week and for the first time in these aortic guidelines it's listed what some of the recommendations are for physical activity and quality of life and, and I think they're reading from our playbook, which is really, really wonderful.
So people with significant aortic disease, education and guidance should be provided about avoiding intense isometric exercise, burst exercise and collision, so yes, we want to talk about that and then we want to move on. For people who have had surgery for aneurysm or dissection again for the first time post-operative cardiac rehabilitation is being recommended and that's going to really help with payers and insurance and all the things that we want to improve quality of life and exercise tolerance and again exercise prescriptions are here for those with thoracic and abdominal aneurysms and again emphasizing whose blood pressure is adequately controlled, it's reasonable to encourage 30 to 60 minutes of mild to moderate aerobic exercise several days a week and to screen for quality of life issues, mental health issues and provide support to optimize this.
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
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 interested in. I know Dr. Prakash has been interested in this a long time.
People who, who have experienced an aortic dissection that, that leave the hospital we expect most to live long lives and, and we want them to have the best quality of life possible. And this, this is a condition that brings a lot of fear and anxiety and frankly post-traumatic stress for individuals and their families and certainly for caregivers who are not familiar with what is considered safe and what's not considered safe and a lot of the fear is really produced by things that we might say or others might say in our position to somebody because of our uncertainty about what's safe, so that's a lot of area of active research that we think can improve quality of lives.
There's some information out there. We tried to write papers about this, providing what is expected with blood pressure with physical activities. That would sort of work that Dr. Prakash is specializing in to provide general information about safe level activity for individuals after aortic dissection.
This is a survey that was done out of the University of Michigan a few years back of 300 people with aortic dissection about lifestyle, what side of exercise they're doing, what emotional state you're in and you can see that a third of people in this survey had mental health concerns after aortic dissection, depression, anxiety and a quarter we're doing no exercise at all. Many were no longer sexually active because of concerns about safety but interestingly as, as you're learning and we're learning the theme of tonight is those who did exercise routinely had better quality of life and and lower blood pressure and this sort of, of information is being reproduced in other studies now.
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
Great, yes, so the first study I'd like to discuss is a pilot study from the Netherlands, again involving children ages 6 to 18 years old, primarily with the diagnosis of Marfan syndrome but there were some as you can see with with vascular EDS and Loeys-Dietz.
The training they put these children through a training regimen with a trainer that consisted of high intensity or power training for 12 weeks, three times a week, in 45-minute sessions and I wasn't able from the abstract to understand exactly what the, what the sessions consisted of but they were not all the same. It depended upon the individual's physical ability. There was an assessment and then each, each program was standard, was, was crafted for the patient's capacity and, and goals. The feasibility of this approach was measured by the percent of sessions that were attended. They were looking again at 36 total sessions and they asked the children and the parents separately to rate the effects of the sessions. The physical function was measured at the beginning and end to assess the training effects. Next slide please.
Though they reported that of these 10 children 9 out of 10 completed at least 90 percent of the training sessions, there were no adverse events which they defined as anything that would require medical attention or stopping the exercise program. An 8 out of 10 achieved children did achieve their training goals stated at the first session. Parents and children both rated the training program as very feasible and they did show that their physical function scores on these standardized performance evaluations at the beginning and end did improve significantly as they were hypothesizing. The next slide.
So the conclusion of this pilot study again with a very small, small number of kids is that this high intensity training program is feasible and did improve physical functioning over a short time 12 weeks in children with heritable connective tissue conditions. The numbers are really too small to conclude that the exercises are completely safe, again these, this is high intensity and so it may not be appropriate for many kids who have musculoskeletal or other impairments to, to these kinds of activities and there were also no standardized criteria for their participation. They simply required the permission of their cardiologist, so with that in mind it is a useful first step but there's a long way to go to make this more standardizable and, in larger populations.
So in this group from Stanford University in California the question was can a simple intervention to increase the amount, number of steps a day that individuals with Marfan syndrome take slow aortic root dilation over a period of six months, so this was a prospective study of a six months program and 24 children, 14 boys and 10 girls, 8-19 years old with Marfan syndrome and they compared them to age-matched controlled Marfan patients who did not receive the intervention but went to the same clinics at Stanford.
So the intervention consisted of, of cues over these, over smartphones and, and through periodic visits for them to take 10,000 steps per day. Each of the children was given an activity tracker where they could log their steps and of course the study team had access to their their data. The primary outcome between, beginning of the study in six months was the rate of change in their aortic root Z scores, again looking to see if the individuals who were exercising actually had slower aortic dilation similar to that Marfan mouse study that I showed you. The next slide.
So the results were at baseline they of course found that most children were relatively sedentary and that's consistent with the, with the data that Alan reported, however 22 out of 24 did complete the intervention for the six months and those that did walked an average of 7,700 steps per day. They wore their activity trackers on 93 of all possible days and they did find that there was a significant decrease in the aortic root z-score in the change between zero and six months in the group that had the exercise intervention compared to the sedentary controls, which didn't really change at all, so this is very encouraging, however it is not, it is not definitive because again it's a small number and there were lots of other factors that could have played a role. Next slide.
The conclusion was, however, that a simple activity intervention in children did appear to be feasible and may be effective to decrease the aortic dilation rate. They are planning to verify this in a randomized controlled trial because it's not clear if the children who are studied as controls may have had different characteristics than the individuals who did participate in the activity since this was not randomized and they were using individuals in different circumstances, so next.
So in a similar vein there was a pilot study on the effects of moderate intensity exercise in a program on children and young adults with Marfan syndrome. This was at Baylor College of Medicine in Houston and so this was a prospective study of an eight-week supervised moderate intensity exercise protocol similar to what we do with an adult with, with adults but in children and teenagers ages 12 to 21. There were only seven patients enrolled. There were five of them were, were women with the diagnosis of Marfan syndrome and, and most of these exercise sessions were held virtually due to the pandemic. The primary outcome was the change in their capacity of, to do exercise is assessed by standardized tests at the beginning and end of the of the study. They also measured heart function and mental health using questionnaires. Next slide.
As a result, they, they tried to hold in-person exercise sessions but they switched in middle, in the middle of the study to virtual due to non-attendance. This was in the early stages of the pandemic, so all, all seven patients in the protocol did end up completing the virtual exercise sessions for eight weeks. There, they did note that the exercise capacity increased and their systolic blood pressure decreased between the beginning and end of the study. They, they there was no change in aortic root z-scores in the group that, you know, over the, over the time but it was a very short trial as I said only eight weeks and it was a very small number of participants so that's not surprising. Next slide.
In conclusion the moderate exercise did appear to be safe and well tolerated. There were no adverse events that caused people to quit or injured, injuries did not occur. They did show that exercise improved cardiovascular fitness in standardized tests but we're not able to show that it, that it improved aortic root dilation, however, they did not measure aortic root enlargement during the study, either. They may not have been able to show a change because the patients were older and the intervention was shorter compared to the previous study that I talked about.
I'm going to end with the last abstract from the sessions in Paris, so an exercise research and Oliver Milleron and his group from the hospital in Paris. He shared some of his slides with this. This is a very interesting approach, so they have developed over the last three years a web-based tool, so they look at the kids in primary school and examine them, look at their physical characteristics and then look at what sort of exercise is prescribed for the children in the school and then they rate those depending upon what you might expect blood pressure and in particular blood pressure response and it can differ in men, sorry, girls and boys and then they give a checklist and recommendation for yes or no or in and this and then the parents can choose to use this if they wish to help with the activities that are part of the school.
Science in Parisで公開された論文の紹介はこちらで最後にします。Oliver Milleron先生の研究グループによる非常に興味深いアプローチです。研究グループは直近の3年を費やし、オンラインツールを開発しました。一方、小学校児童の身体的特徴および学校で行われている運動を調べ、想定される血圧、特に血圧の変化に基づき、これらの運動をランク分けしました。血圧変化は男女で異なる可能性があります。さらに、推奨される運動のチェックリストを作成し、お子さんが学校で行う運動について親御さんが選べるようにしました。
So the skeletal features like how hypermobile they are, do they have certain limitations that might impact the type of physical activities based on their phenotype, do they have certain eye features which might affect levels of exercise, and they also look at different genotypes, do they have the FBN1 Marfan gene or do they have genes in the Loeys-Dietz family and that might predict different responses for different levels of physical activity and then they thought about what's the skeletal effect and what's blood pressure response and they use of standard data sets based on age and predicted responses. They didn't actually measure the blood pressures in the kids. They just use what would be expected so probably a more practical approach for large groups when they're thinking about this.
But still this is what it kind of looks like, so of course this is in French for our French reading audience and then they have like mid distance running, the hurdle, and other things like that and then what sort of response, Level 1: no or minimal increase in blood pressure would be expected; Level 2: mild, and Level 3: sustained and high increase in blood pressure, the sort of thing that Dr. Prakash talked about earlier in, in the exercise tests and some of the exercise protocols in some of the patients he was seeing, so Level 3s were recommended against and then Levels 1s and 2s were dependent upon the person, their age, and the type of activity.
So they use this tool for the kids and then they generate the report and then they, it's really very yes or no, so again not a lot of gray areas for some of this and that can be kind of problematic for some and how to adapt that but still I think it's a useful way of thinking about this that might be helpful for some. We don't have such a thing at our institution and not, not all people use this in, in Paris but they've generated 168, that's 165 such reports in the last three years.
And here might be one of them, so for instance, you know, Aerobic "OUI" so that's YES, so here's I can't imagine why badminton is "NON" but it's NO, so that must be some a person for which badminton is not recommended, basketball "NON" etc., so this is a sort of paperwork that's generated for a school level child through their clinic, so trying to be helpful and to try to individualize the exercise recommendations, emphasizing what is OUI for the individual so they can participate in physical activity because it's important.
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
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, from regular aerobic exercise compared to the mice that sat on the couch, so that's very informative, instructive and, and could translate into, into information that's helpful in people and that's what we're going to talk about now about what do we know about people.
Your studies also is to emphasize that it is important to think about the blood pressure response to different activities for different people because it can vary but in general it looks super promising that the blood pressure response to this sort of physical activities is well within the normal range from, for most and that they can continue that and the more aerobic activity they do on a sustained manner, the less the blood pressure response. It's a training effect, very interesting.
So what we're going to move on to now is into we're going to review these seven papers or abstracts. I'm going to talk about really the background information from some of the European studies, which look at physical capabilities and physical activity in children with heritable disorders setting, the background for why it's important to think about this and to actually prescribe exercise and physical activity for quality of life and, and, and really physical fitness, so I'm going to set up that and then Prakash is going to talk about the benefits of this.
So first paper was from the Netherlands and this was a study of 56 children, average age of about 12 years old, 59 percent male and they had Marfan, vascular EDS, and Loeys-Dietz syndrome followed in this university and really what they, what the authors wanted to do is understand these children living their lives with the recommendations for physical activity they were had been given, they wanted to say what is a physical capacity and what's the muscle strength and the motor proficiency and physical activity and compare that to standard controls and see what's the background.
So this was the results, which were fairly striking, that the children with heritable connective tissue disorders that's what they labeled this group. They had reduced cardiovascular endurance and mild to moderate reduced muscle strength compared to controls. Motor proficiency, the use of, of muscle skills wasn't different but really physical activity was reduced in compared to daily functioning other children of similar ages even though the kids were active four hours a day, inactive more often and average steps were about 9,000.
They concluded that there was reduced physical capacity and physical activity in children with heritable connective tissue disorders and they thought that the main reason for the reduced cardiovascular endurance was deconditioning as, as opposed to a primary problem with the, with the individual is really this, not being as active in combination with perhaps genetic factors in some.
Very similarly, a group from Toulouse, France examined fitness capacities in children and young adults, predominantly Marfan but also some related disorders, sorry, related conditions and these authors examined 28 individuals, similar age 12 years a little bit a bigger range up to 20 years with a heritable aortopathy condition, again assessing what's the physical capacity and what might be a cause for limitation compared to age match controls.
There was mild aortic enlargement, so this would be aortic z-score of 2.4, which would be just barely over the normal range. They did not have any significant vascular disease, valvular disease and importantly the vast majority were on beta blocker therapy. That's important because beta blocker therapy can blunt the results of cardiovascular exercise testing because the heart rate response isn't as brisk and they did cardiopulmonary exercise testing to assess performance in these kids.
And they found that the maximum exercise performance and the cardiopulmonary performance was reduced in children with Marfan and related conditions and they can measure that by oxygen consumption with a sophisticated set of testing and it was about two-thirds of that expected for age match controls and again these authors had similar conclusions they thought it was peripheral muscle deconditioning, in other words, not being as active being more sedentary and the effect of the beta block or reducing the heart rate, so some of it was a little bit artificial but some of it was related to deconditioning, not related to a primary problem from the hearts, lungs of these individuals and interestingly when the quality of life measures were obtained by standard questionnaires it was lower in the kids in this population compared to controls.
They concluded the deconditioning in Marfan syndrome may contribute to this decreased quality of life and should not be neglected, emphasizing the importance of physical activity in school to begin at a young age. Very, very forward thinking.
Very similar study will be the last one that I'm going to talk about in this session. The authors propose the concerns about aortic risk in genetic aortopathy conditions like Marfan, vEDS and others, especially the concern about aortic dissection has kind of shifted the pendulum and the worry from the importance of exercise in quality of life to the concern about risks. There has to be a balance there.
They analyze the impact of a genetic aortopathy condition on quality of life measures from young age to adulthood and correlated that with the results of cardiopulmonary exercise testing, so again these authors wanted to understand what's the quality of life and what's your physical fitness and there is a relationship between the two in individuals with a genetic aortopathy.
So 63 individuals average age of 12, 5 to 21 years old with Marfan or a related connective tissue condition. They did quality of life questionnaires, socio-demographic parameters and then cardiopulmonary exercise testing was performed in just a subset about half with Marfan syndrome in this population.
And very similarly, the maximum exercise performance was reduced almost the same amount as in the prior study, 64 percent of that expected and they also found a correlation between quality of life measures and the results of cardiopulmonary exercise testing.
So similarly, they emphasize the importance of deconditioning and genetic aortopathy and that the care of individuals with genetic aortopathy should include adapted physical activity programs beginning at a young age to try to address these factors and perhaps improve quality of life measures.
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
