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。

大動脈解離の治療における進展 ~血管内治療(マルファン関連疾患患者の場合)~

Advances in the Treatment of Aortic Dissection




Most of the experiences in the literature are single study or single center institution reports with a handful, maybe, two dozen at best and you can see for patients in both dissections as well as aneurysms. 



There's a significant amount of reintervention in terms of both open surgery, maybe percutaneous interventions and there's still some amount of mortality associated with these types of repairs. 



We are writing up our experience and if we do have one of the largest series but you can see we still have a six percent stroke risk, compare that to Dr. Moon's previous statement. Three percent risk of the paraplegia. That's spinal stroke. Endoleaks are where we have either an incomplete or an inadequate repair and you can see that there's about 40 percent of or so of our patients and we are very high volume, very experienced center. A lot of, six percent of patients have another procedure within 30 days of their implant and then even after that 30 day mark there's 30 or so percent of patients go on to require another intervention and there's this idea of this stent graft-induced new re-entry tear, so even if we try to cover the tear with a stent graft and redirect flow out of that false lumen only into the true lumen the stiffness of the endograft is such that can tear the that very mobile, verifiable dissection flap and allow perfusion to re-enter the false lumen and so we see that in about one-fifth of patients. 



Shifting to large database series looking at national registries here in the US over 100 patients were identified meeting diagnostic criteria of either Marfan syndrome, vascular Ehlers-Danlos, or Loeys-Dietz and these were done for roughly equal aneurysms, acute, and chronic type B dissections and what they saw was still a fairly significant risk of an endoleak and reintervention downstream. Their overall mortality was five percent at roughly 15 months after follow-up and three of them have retrograde type A dissections requiring emergency cardiac surgery.  



So it's not the magic bullet like I said and it's it really has to be used judiciously and selectively.



I mentioned delayed aortic events are real and I don't mean within the first 30 days. Here's a patient that we treated here, who had her endograft placed elsewhere eight years ago. She was fortunate. she had remodeled her aorta well at that stented segment but she presented with a inter, excuse me an intramural hematoma within that stented segment and she ended up requiring open conversion because of intractable pain and enlargement at that section and so it's not a, it's not a fix even over the long term. 



You still require long-term follow-up and then open surgery for downstream events and even events within the stented segment and the, the way we repair patients who had a previous stent graft open is we try to incorporate this stent graft as much as we can. We think of it as more as a pledge it or something to reinforce the suture line rather than something that's a definitive repair and so we do end up removing a significant amount of the stent graft when we do our conversions for downstream events whether it's aneurysmal dilatation or a change in the dissection flap that ends up causing malperfusion. 



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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