10:18-15:56
So what, what issue am I trying to resolve now? Marfan syndrome obviously is a multisystem disorder. It affects the eyes, it affects the spine, it affects the muscular-skeletal system. In fact, it affects many parts of the body. Some might say it affects every organ system to some extent, sometimes just trivially but sometimes quite seriously but I think that the effects on the, the the cardiovascular system are the, you know, by far the most serious in terms of impacting a patients longevity, how long they're going to to live.
マルファン症候群では、眼、背骨、筋骨格系など複数の部位に問題が生じます。程度の差はあれ、全ての器官系に問題が生じるという意見もあるかもしれませんが、寿命の観点から何より真剣に捉えるべきは、心血管系に対する影響です。
Now there are patients with Marfan syndrome whose life expectancy is unaffected by Marfan syndrome. You know, I have at least one patient I can think. I've got one one chap who comes to my clinic, who is 81 years of age, who has definite Marfan syndrome and he has never had any heart surgeries. He's never had any surgeries on his blood vessels. He was treated, you know, for the first 50 years of his life I don't think he had any medical treatment and even in later years he didn't have much medical treatment, probably just a beta blocker, so, you know, we haven't done very much to him. He's, he's lived for 81 years without much treatment, so it's certainly possible to live to very advanced ages with Maran syndrome and not have serious cardiovascular disease but many other patients do need to have quite, you know, either they need medications or they need surgeries or they run into complications and so, so that's what we're trying to deal with.
マルファン症候群の患者さんでも、この病気が寿命に影響しない方もおられます。私が担当する81歳の男性患者さんは、間違いなくマルファン症候群なのですが、心血管系の手術を一度も受けたことがありません。50歳までは内科的治療を受けたことがないと思いますし、高齢になってからも治療はβ遮断薬のみですが、81歳まで長生きしておられます。マルファン症候群であっても、心血管系の問題を起こすことなくこのように長く生きておられる方もいらっしゃるわけですが、多くの患者さんは、薬物治療や手術が必要になりますし、合併症を起こすこともあります。我々はこうした患者さんの治療に当たることになります。
You know, I focus on the cardiovascular manifestations and in particular I focus on problems of the aorta, so I know that many, probably most of you already know this, most of you do but I guess it's just possible someone on the call has just recently been diagnosed and this hasn't come up yet and I think that perhaps something that people don't fully appreciate is that there are, this is, this usually begins and mainly affects two specific parts of the aorta and the most vulnerable part is this part here, so here's the aorta in the chest, it is of course a gigantic blood vessel people often are very shocked to learn how big it is. It's, I always say, it's the biggest part of your body that no one's ever heard of and, you know, it starts here just at the heart. It then loops up around just under the neck and giving off these branches to the arm and to the to the head and then it loops around like this, this is the arch of the aorta and then it loops down through the chest. That's about a foot long or, you know, maybe that kind of thing and then it sort of goes down through the diaphragm into the abdomen then through the pel, and then it sort of halfway down the abdomen, it's splits around about where the the navel or belly button is here and then splits into these two big blood vessels here.
このウェビナーでは、心血管系、特に大動脈にフォーカスして話を進めます。ご参加の皆様の大部分が既にご存知の内容かもしれませんが、最近診断されたばかりで、よくわからない方もおられると思いますので、最初から説明します。マルファン症候群によって影響を受けやすい大動脈の部位は二箇所あり、最も影響を受けやすいのが大動脈の始まりにあたるこちらの大動脈基部です。左側は大動脈全体の図です。巨大な血管ですので、皆さんその大きさにびっくりされます。身体の中の最も大きな器官と言えます。大動脈は心臓を出て、首の下で腕と頭への血管を分岐させ、ループ状になります。この部分を大動脈弓と呼びます。その後、胸部を経由して下がります。胸部の部分の長さは足のサイズくらいだと思ってください。横隔膜を通り腹部へと入ります。腹部の真ん中あたり、というのはヘソのあたりですが、ここで足に向かう二股の大きな血管に分かれます。
And usually here it is of taken out and this is the aortic valve and then this very first part here is called the aortic root. These two very, very important blood vessels: the coronary arteries come off here. This is what causes, you know, in the generally older people causes coronary artery disease. That's not a feature of Marfan syndrome. That's different and then but this is the part that tends to swell in Marfan syndrome and if it gets too big that's when there is a risk of tearing of the aorta here and this is where the risk of tearing is very serious because it can disrupt the aortic valve, it can disrupt the coronary arteries and it can run back towards the heart, so that's what we want to avoid and then there's another area of vulnerability just here where the aortic arch meets the descending aorta.
右側の図を見てください。こちらは大動脈を取り出した図になります。四角で拡大された部分が大動脈弁、大動脈の始まりの部分が大動脈基部です。大動脈基部からは冠動脈と呼ばれる二本の重要な血管が出ています。一般に高齢者になると、冠動脈で病気を起こしやすくなるのですが、冠動脈疾患はマルファン症候群の特徴ではありません。マルファン症候群では、大動脈基部が膨らむ傾向にあり、大きくなりすぎると裂けるリスクが生じます。大動脈基部が裂けることで、大動脈弁や冠動脈が損傷したり、血液が心臓に逆流する可能性もありますので、リスクのある状態は回避しなければなりません。マルファン症候群で影響を受けやすいもう一方の大動脈の部位は、大動脈弓から下行大動脈に切り替わる部位です。
And when they have a problem here it tends to cause tearing called a type A dissection and when you have tearing here it tends to cause what's called a type B dissection, generally speaking, so that's the issue that we're trying to prevent and we're trying to slow down the growth of the aorta here and here.
一般に、大動脈基部が裂けると、上行大動脈の裂傷を引き起こすA型解離になりやすく、下行大動脈が裂けるとB型解離になりやすい傾向にあります。これらの大動脈解離を防ぐことが我々の仕事であり、そのためには大動脈の拡張速度を遅くする必要があるのです。
Now we get these things called aneurysms which are sort of swellings of the blood vessel and here's one type of aneurysm and here's another type of aneurysm and you can get aneurysms anywhere along the aorta but this is the kind of aneurysm that you tend to see in Marfan syndrome and, and also other conditions like Loeys-Dietz syndrome and, and it's right down at the root of the aorta. You don't see this, you see this another conditions, you see this like this. It's like a flask that we used to use in school in chemistry lessons like that and that's what it looks like.
動脈瘤についてのお話をします。血管が膨らんだ状態を動脈瘤と呼びます。こちらのスライドにある二種類の動脈瘤は種類が違います。動脈瘤は大動脈のどの部分にもできる可能性がありますが、マルファン症候群やロイス・ディーツ症候群で見られるのは、大動脈基部が膨らむ左側のタイプです。学校の理科の実験で使うフラスコのような形状をしています。
And if I go to the next slide this is what the echo cardiogram looks like. Now this is the same aorta but it's just been tilted 90 degrees on its side. This is what a normal aorta looks like. Here's the heart, here's the aortic valve, here's the aorta and here's the little flask. This is what a normal one would look like and here is what one would look like in a patient with Marfan syndrome where the aorta has been allowed to grow far, far too large. Each one of these markings here is one centimeter, so this is probably one, two, three, four, five, six, seven, eight, eight and a half centimeters. That's that's crazy, too big, so and here's the aortic valve and this whole thing has got, got far too big, so that's, that's what a Marfan aorta would look like on an echo scan.
次のスライドは心エコー検査の画像です。90度傾いた大動脈が写っています。左側の画像が正常な大動脈でフラスコのような形をしています。大動脈の左側に心臓があり、大動脈の根本に大動脈弁があります。右側はマルファン症候群の患者さんで見られるような大動脈の画像です。かなり大きくなっています。点と点の間隔が1cmですので、この患者さんの大動脈基部の太さは8.5cmあります。根本に大動脈弁がありますが、全体的にかなり拡張しています。マルファン患者さんの大動脈基部は心エコー検査ではこのような画像になります。
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。
