Marfan Syndrome Management: Back to Basic
11:39-13:17
And then we'll get to the heart. This is my main area of expertise and interest and the two most common problems in Marfan syndrome are dilation of parts of the aorta and something called mitral valve prolapse and I'll go through this a little bit, so the aorta is this kind of candy cane-shaped-looking vessel that comes out of the heart. It gives all the blood to the heart to the body and it gives blood up to the brain and the arms and back down to the legs and normally it's shaped kind of like a candy cane but in Marfan syndrome you can see here. There's this bulge here this is called an aortic root aneurysm or in kids we call it aortic dilation. You can get aneurysms here. This is called the aortic isthmus. This can also be dilated, so we look for these and when they get too big that's when we need to do surgery and if we see dilation at all this is when we prescribe medications.
次は私の専門でもある心臓の所見です。Marfan症候群で多くみられる2つの所見は大動脈の拡張と僧帽弁逸脱です。この2つを見ていきましょう。大動脈は杖型の飴のような形をしています。心臓から出た大動脈は脳や腕に血液を送った後、背中側を下り、脚に血液を送ります。通常は杖型の飴のような形ですが、この画像からわかるように、この部分が大きく膨らんでいます。これが大動脈基部の大動脈瘤です。子どもの場合には大動脈拡張といいます。大動脈が下り始める大動脈峡部も膨らんでいます。こうした膨らみを検査で確認し、大きくなりすぎている場合には手術を検討し、膨らみがあるような場合には投薬治療を行います。
Mitral valve prolapse, so this is the heart lying on its side. This is the left atrium and left ventricle. Normally this valve, there's a valve that opens and closes should close like a v here and you see it's closing. It's more flat. It's kind of actually this area is flipping up into the left atrium. This is mitral valve prolapse and this is also commonly seen in Marfan syndrome.
僧帽弁逸脱について説明します。こちらは心エコーの画像です。右側が左心房、左側が左心室です。間にある僧帽弁は正常ならば閉じた時にV字型になるはずですが、この画像ではフラットになっており、この膨らんだ部分が左心房側に落ち込んでいます。これが僧帽弁逸脱で、Marfan症候群では一般的な所見です。
Those are the most common, so aortic dilation like I mentioned the aorta when it's dilated can cause this valve to leak. It's called the aortic valve regurgitation. Like I said mitral valve prolapse there's another valve on the right side of the heart called the tricuspid valve that can prolapse. You can have other cardiac findings like a dilated pulmonary artery that's the artery that goes to the lungs. Decreased ventricular function that's the heart muscle and ultimately aortic dissection, which we're going to talk a little bit more but this is when there's a tear in the wall of the aorta and that's what we worry about most in Marfan syndrome.
以上の2つが主な心臓の所見ですが、その他にも大動脈が拡張することで大動脈弁からリークが生じる大動脈弁逆流、心臓の右側にある三尖弁の逸脱、肺に血液を送る肺動脈の拡張、心筋が弱くなることによる心室機能低下があります。最後の大動脈解離は後ほどお話しますが、大動脈の壁が裂ける疾患で、Marfan症候群では最も注意を要します。
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。
