Does the dissection ever heal so that surgery isn't necessary or does it stabilize, does it ever heal?
Eileen:
大動脈解離が自然に治って手術が不要になったり、安定するということはありますか？
A:
So we do see, we see patients with chronic dissections patients that have survived a dissection but again, if you look at that old graph of survival after an acute dissection it's very rare without treatment but there are patients that do survive dissection. I, I, it's, it's unusual. What do you think Dr. Ohman?
Going in, all going back, all the way to medical school in Houston, which also is a major aortic center, I've seen one patient with a type B dissection that we would consider completely healed with medical therapy and that's really, I think, the exception that proves the rule is that this is not a disease that reverses, this is a progressive disease with dilatation and it's all about preventing those downturn, downstream events for as long as possible with medical therapy.
Yeah, it certainly can stabilize though with good medical therapy. We, we have patients, we followed for 20 years whose type B dissections have not grown over time.
That's great. Do you want to add anything that, Alan?
Eileen:
素晴らしいですね。Braverman先生から付け加えることはありますか？
The only thing I would add was, is that the variant known as intramural hematoma where there's bleeding in the wall. About half the cases it does stabilize and just like a hematoma or a bruise anywhere else it does resorb but those in connective tissue conditions are still fraught with concern but there, that's one that can heal or, or, or the false lumen can thrombose so it does seem to stabilize and, and may not progress as much if there's a big contained opening there but, but it usually is a lifelong illness.
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.
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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 your patients?
In the, in the very early period postoperatively or after the event whichever it may be, we typically get one scan at one month and as long as that look, or when they leave the hospital and then one month. If that looks roughly stable then we go to six months and if that looks stable and aorta's kind of proven that it's cooling down and it's not misbehaving and they're not having episodes of pain with exertion we may stretch that out to a year.
And one of the things we've started doing here particularly with patients in their 20s and 30s is that they can tolerate lying flat for an MRA or for a MRI of their aorta and MR aortogram that's where we go because there's no radiation, the con, it uses a different type of contrast, it doesn't stress the kidneys as much.
And so the the follow-up changes a little bit depending upon how stable the aorta is. Now if they've had a large jump in size we may say we're going to go to another month or we're going to go to three months. There's no absolute criteria that we use but to Dr. Moon's point it's more than just imaging. It's the history of the patient and their family as well as what they're exhibiting in between those points in time.
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。
People want to know, I mean, they see obviously that it's better to have surgery prophylactically than emergently. Besides the size, which they would know themselves, are there any other signs that a dissection is going to happen?
Unfortunately, not. The, there's not really preemptive, you don't sort of have low grade pain and then sort of develop symptomatology. I mean it's rare. Sometimes that can occur or it, it sheds a light on maybe this patient's at higher risk.
But I think the most important thing is the family history. If you, if your parents or your uncle had a dissection at a very young age and your aorta is sort of borderline that might be a situation where you'd want to consider an earlier, earlier treatment approach.
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。
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-Dietz, there's teenagers who might need this. Can you comment on that at all?
A: Yes, we, we see a handful of patients every year with a more, a much more aggressive or malignant aortopathy. There's been a handful of patients in their teenage years that have undergone significant thoraco, thorax, and, or chest and abdomen aortic replacement even though they're not technically adults. I remember doing one when I was finishing training with Dr. Moon and one of my other partners.
And so they're, they still require close follow-up with the pediatric cardiologists and the, Dr. Braverman can probably speak to the handoff between the pediatric and the, the adult cardiologist but for the most part a lot of the surgical triggers, criteria, techniques are roughly the same between the, the two. The grafts may be a little bit smaller. We may leave a little bit more redundancy in them to allow for future growth but the, the overall themes of trying to get the patient through the operating room and out of the hospital with an eye towards the future, even if that for, future maybe several decades longer, still applies.
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 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 procedure, which we'd have a talk about it last summer at, at our international meeting. Is there new data? Is it being done in the US? Will it be done in the US?
Well, the PEARS procedure could be performed anywhere. It's, it's for those of you who don't know what it is, it's personalized external, does it mean, aortic root support, right? It's essentially wrapping the aorta and the idea is you're trying to squish it and prevent it from expanding. Now that, that, that's good for sort of preventing aneurysms that will rupture because it's, it won't grow but in theory that will not prevent a dissection because a dissection occurs in the wall of the aorta, so if the wall of the aorta is weakened, necessarily that may not work and sure, it can be done and it's generally done in very, very small aortas that, generally, we wouldn't operate on necessarily at the current recommendations but it is something that to be considered.
And I'm going to give you a historic example, Albert Einstein had an aneurysm. He went in to get a gallbladder repaired and they found an aneurysm of the aorta and they wrapped it in cellophane, I think, is that right, Wes?
They wrapped it in cellophane and two years later it ruptured, so, you know, the wrapping is, is there is some merit to it and certainly it can be done but I think it's not our sort of our standard therapy to get rid of the diseased aorta. Alan, what, what do you think about that?
I think, I think it's an interesting concept. As Dr. Moon said we don't actually have the exact PEARS protocol. That's done in Western Europe, predominantly England and others, where it was developed, where a three-dimensional mapping of your own aorta is done and personalized, that's why it makes it personalized, surgery is done where the aorta is surrounded by this graft and in that series with very careful, carefully selected subjects they've had really very good outcomes in the, in the short term follow-up.
And it's just not FDA-approved in the United States right now, so it is currently not available even if Dr. Moon said I want to do that for you he would have to come up with a different way to do it. It's just not an FDA-approved procedure right now for root replacement in Marfan and Loeys-Dietz but it is being done very carefully and as Dr. Moon said generally the aorta's smaller four and a half centimeters or so without a lot of aortic valve disease and again in the, in the follow-up so far in those patients they've actually done very well.
Looking for something, we're always looking for something less invasive but equally effective and that's what Dr. Ohman and I are just moving forward in that direction.
Along the same lines, what about the sleeve, I know I'm in Florida, so a lot of people do have what they call the Florida Sleeve, so can you comment on that as well?
Yeah, I know, the Florida Sleeve is essentially just a version of, of root replacement with specific techniques and the surgeon who developed that in Gainesville, Tom Martin was a good friend of mine, so it's just the way he does it and this is just a description of the technique so it's, it's, it's essentially a, aortic root replacement with various minor maneuvers.
Dr. Moon:
Florida Sleeveとは、大動脈基部置換術の方法の一つです。考案したのはゲインズビルのTomas D. Martin医師で、私の親友です。彼が用いた方法がFlorida Sleeveと呼ばれているだけです。大動脈基部を置換する方法には細かい技法が様々あり、そのうちの一つということです。
Okay, great. Thank you very much.
Eileen:
わかりました。
Very effective.
Dr. Moon:
非常に効果の高い方法です。
Okay, good to hear, good to hear.
Eileen:
ありがとうございました。
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。
Well, I think, you know, I forgot what year it was but 15 years ago so maybe, Loeys-Dietz was sort of not even on the horizon and Dr. Braverman was very critical in bringing that to light that, that is actually a little bit more aggressive disease process than or syndromic process than Marfan syndrome. In that patients with Loeys-Dietz can have abnormal arteries in places that Marfan patients don't usually have and also they can have tears and, and, and ruptures and things like that at a much smaller size.
Dr. Braverman, maybe you can comment on that because we're much more aggressive, I think, in a Loeys-Dietz patient prophylactically than, than a patient with Marfan although we're aggressive in both.
Dr. Moon's right. The and, it's an important question Loeys-Dietz, of course, now has five types and we're learning more. As Dr. Ohman said about how the genetics predict the outcomes and how we are going to be making surgical and vascular surgical decisions based on what the genotype is but in general for Loeys-Dietz type 1 and 2 or TGFBR1 and 2 prophylactic surgery that are much smaller size than Marfan syndrome is recommended. For some of the newer varieties we just don't have as much information and even amongst the ones we know about we're learning more that some genes behave even more aggressively than others in the same family. So a lot of work is being done here and it does take a multi-disciplinary approach with a lot of shared decision making about what's right for you.
And, and your family history is very important. When someone in the family has an aortic dissection we think about you much differently. We're much more aggressive about doing earlier surgery and that's the borne-out in most type of aortic disease.
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。
Q: Dr. Ohman, you mentioned, you said that but the endovascular repair not being for connective tissue disorder patients. There's so many questions about that. Can you explain why and what you worry about and why that's not what you do?
A: So even as I outlined in my talk, even in the best case scenario for patients who don't have a fragile aorta we're not really seeing the great effect in terms of reduction of mortality, reduction of future events or surgery out until five years in a best case scenario.
And what we're seeing really more is a lot of changes for the negative either at the attachment site, Dr. Moon mentioned, the landing zone and that's really critical in terms of when you think about an endograft. You have to find a way to kind of tack it at one place proximately one place distally.
These devices are still relatively stiff and so that outward force, even if you size it one to one or a millimeter larger than the aorta, you still have this chronic radial force in a, in a weakened blood vessel that can induce a pseudo aneurysm or localized rupture at that site. We've seen that. That can induce tears in the dissection that change the true versus of the false lumen profusion and actually make things downstream in terms of the legs, the intestines, the kidneys worse off than they were before.
And so, you know, as one of the larger centers in the world for treating patients with connective tissue disorders with stent grafts, yes, I think there's a role for it but it's really, you have to be really thoughtful, so if the patient won't survive open cardiac surgery and we use it as a temporizing measure, yes, that should absolutely be done. If it's a patient who has a surgical graft for their, let's say, they had an interposition for, for a type B dissection done open and they've had the visceral segment replaced and I just need to span to fix an aneurysm between the two surgical grafts that is a reason or that is an indication as well because you're landing in non-threatened tissue, you're landing in dacron that Dr. Moon has sewn in and so you're you're minimizing a lot of the risks but once again those patients aren't out of the woods because we're still, we've still seen aortic events in that stented segment that looks great up to eight years out and so it really has to be judicious.
I know it sounds very attractive and appealing that it's a much less invasive procedure but it's fraught with complications that do require either bigger operation or in some cases an emergency operation after the stent craft has been put in.
Yeah, I just wanted to say one thing and, and that's, what's important to have a aortic team that works on these issues so that it's not one or the other but we work together to sort of determine what's the best for that individual patient.
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。
