Q: If somebody's facing elective aortic surgery should they get the vaccine first or should they hold off getting having the surgery?
大動脈手術とCOVIDワクチン接種のどちらを先にしたらいいですか？
A: I would say if you can get the vaccine whenever it's available based on sort of those risks and things get the vaccine because I don't want you to miss the chance to get the vaccine and then you should be able to schedule your surgery within a couple weeks after that.
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。
Dr. Crawford, who is one of the real pioneers from Houston, who taught, learned and taught how to fix the more downstream portions of the aorta like the thoracic, abdominal aortas, said that no patient should be considered cured of aortic dissection.
And we know that even after you have a prophylactic operation on your aortic root sometimes patients will still come back with a new dissection, not the dissection of the part that's then replaced, thank goodness, that's the most dangerous portion of the aorta that's at risk for rupture but in the downstream aorta you can still develop a type B dissection.
We looked at a series of our patients. This is unpublished but we had this collected this data. We found several patients who had a new dissection after having their ascending aorta replaced but it was only a small proportion of our patients in our series, less than one or two percent. Many of the patients had their surgery elsewhere so I don't know the denominator exactly but we know that risk is relatively low but still very real so it's important to monitor things like your blood pressure and get imaging on a regular basis.
If you have had a dissection the story is a little different. If you've had a dissection that's affected downstream parts of your aorta you will be at risk for having a re-operation on that aorta, a significant risk 15 times the risk as if the downstream aorta wasn't dissected.
We looked at patients in our center, who had surgery within a downstream, patients with connective tissue disorder who had surgery beyond their aortic root. We looked at 121 of these patients out of the 527 during that period of time, who had connective tissue disorder where their repair extended beyond the left subclavian artery.
The majority of them were chronic dissections. There were a few that did have aneurysms. They were relatively young. Most of their first operations were open but the patients did okay.
The mortality was two and a half percent. Stroke rate was two and a half percent and there were no patients with spinal cord injury in this group and so, you know, it's something that we can do well.
When we followed these patients interestingly a lot of these patients needed additional interventions. Fifteen percent of them needed two or more interventions.
Some of these were open, of course, you can see the picture on the right is a picture of an open focal abdominal aneurysm repair. It's a long section of the aorta from the top of the chest down into the pelvis with branch reconstruction of all these other vessels.
But a lot of these things can be treated endovascularly as well and endovascular treatments, I think, are very reasonable and complementary to open surgery even in patients with connective tissue disorder as long as we understand the limitations of that technology.
What we saw was that there was about a 60 percent risk at six years for patients who had a connective tissue disorder that had a single operation to go on and need some additional operations down the road.
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 what's next? What's next for how we're going to treat aortic roots and aortic valves?
大動脈基部や大動脈弁の治療に関して、今後期待できることを紹介します。
Well, there's ongoing studies that are looking at NOACs, which are novel oral anticoagulant drugs like eliquis. You sometimes see these commercials for these things. They say don't take it if you have a valve. Well, that's because it hasn't been studied in valves but it's being studied for mechanical valves now. There's a PROACT ( Prospective Randomized On-X Anticoagulation Clinical Trial) study for the On-X valve that's ongoing.
There are a couple of new artificial valves that are in development where they're looking at polymer materials for the leaflets that may be more durable than some of the biologic tissue valves but still don't require anticoagulation. So I think we're seeing some very interesting technology coming down the pike for the valves.
And when we think about what to do with the aorta, the picture on the left is something called the PEARS procedure. Maybe we can talk about that in discussion. It's very experimental and for now I think that there are a lot of unanswered questions about it but if we can do something like that in some minimally invasive way where we can change the structure on the wall of the aorta, which is what they're trying to do with the PEARS procedure I think there's a lot of mechanical, potential mechanical solutions that we can offer to people as we understand the interaction, the interface between devices and patients.
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。
At the Cleveland Clinic in 2020 we did 1,381 operations only a couple percent lower than 2019. We were affected by the pandemic but fortunately people understood that their cardioaortic disease was problematic and we were able to maintain safety in our institution and continue to provide care for patients over the last year and we hope to continue to do so going forward.
We also when we look at the valve operations that we do because again I think you have to think about the root and the aortic valve together. You can see that in the over three and a half thousand valve operations that we did. Many of them are combined and in that 58 of combined valve operations. A lot of those include the aorta patients.
This is our volume of valve re-implantation procedures. Dr. Svensson really built the program here in the early 2000s and I joined him in 2005 and then after gaining some experience and helping to train some other surgeons we've really ramped it up and we're consistently doing over a hundred operations of these valve re-implantation or David procedures every year.
And with that experience, it allows us to do even more complex roots like this patient. You see the green arrow shows a valve that's just really stretched out by the aneurysm. It looks like a like a bow string but the tissue on the cusps was healthy and the rest of that root gets reconstructed when we do a David's procedure and so we were able to save that valve that living valve and re-implant it in the root and make it look like this. This is a patient of mine that valve's still working pretty well after six or seven years later.
And when we looked at all four of those different kind of root operations I talked about the mechanical/biologic composite valve grafts, the homographs and the valve preserving procedures over this period of time with nearly a thousand patients. The overall mortality was less than one percent the stroke rate was also low. Remember some of those are older patients getting these procedures
And I thought that this was important to share and demonstrates that a experienced center with a lot of volume and experienced surgeons can have even better results.
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 showed you the quick survival curve from our series but we looked at 178 patients, who underwent that David's re-implantation procedure in our institution and 84 percent of them had Marfan's, the other 16 percent had other connective tissue disorders, about a third had really quite leaky aortic valves. In a proportion of them we did extended repairs into the arch plus some mitral valve repairs.
But we also looked at freedom from re-operation and in the follow-up of these patients there was a freedom for re-operation of better than 90 percent six years and freedom from valve operations 93 at at five years.
It's important to also when you're choosing a surgeon and a team if you have a chance to do so, it's an elective surgery, to appreciate that experience matters.
In this assessment of a database from the society of thoracic surgeon they looked at over 13,000 operations that involve the aortic root or combination of the valve and the ascending aorta at 741 centers.
Interestingly 25 percent of the operations were performed at three percent of the centers. A large volume center was defined as one upper quartile did more than 30 cases a year.
If it was a large volume center the mortality was half of what it was at a low volume center. Three percent versus six percent and so that experience is critical.
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。
Hopefully we'll find other ways to guide us besides just the size of the aorta but what I think is important to also understand is that survival is improving for patients with Marfan syndrome and other connective tissue disorders.
In this, you know, paper back in the 70s the average age of somebody with Marfan's was only about 45. Another paper, which is still is pretty old 25 years ago, we saw a lot of patients where the life expectancy was extending above 70 and now we're doing even better than that.
And hopefully improving the quality of life for people as well as we can offer safer and better operations on segments of the aorta that are involved or other parts of the body that are involved by connective tissue disorders.
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。
Unfortunately, you know, the guideline of how to do this is based on the size but we see in this data here that a lot of aortas will still develop, this acute aortic syndrome, a dissection or tear at a smaller size than the recommended size.
So the general recommendations are that the aorta should be replaced when an aneurysm gets to five and a half centimeters or larger. We typically lower that threshold for most patients because most patients we see are younger might even though they might not necessarily have a connective tissue disorder we're still suspicious that they do if they're young and I consider everybody less than 70 young. And so because we can do this operation safely we typically will lower that threshold down to five.
And if someone has a non-connective tissue disorder with a higher risk genetic abnormality like some of the Loeys-Dietz syndrome sort of abnormalities we may even lower that threshold to 4.2, 4.3, four and a half centimeter range.
A lot of that depends also on your families. You had a family member had a dissection. You knew how big the aorta was when that happened. We don't ignore that. That's what I was talking about earlier about this sort of personalized care we really take all the elements of you into consideration when we take care of somebody.
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。
