What this site is about

This site is to provide information about an operation, particularly for people with Marfan syndrome, who have an enlarged and possibly weak aorta.

By using computer aided design it is now possible to have made-to-measure operation.

Who this site is for

There are no restrictions.

Anyone and everyone is welcome to read it, learn from it, tell us what more you need to know and to tell us what more we should provide.

It is particularly designed to provide a shared source of information for patients and health professionals so that you are know that you are using the same language and have the same understanding of what this new idea is all about.

So if you are

we hope to provide information in plain language that can be understood by the public and professionals.

First to explain what those words mean:

The aorta

The aorta is the main pipe that carries blood out of the heart to supply the whole of the body.
The aorta is a pipe about 3cm across.
If you think of the water main coming into your house from which the plumber puts branches that go to the sink, the shower, the bath, and to each tap, the aorta does the same job giving off a branch to each part of the body.

The aortic root

The aortic root is the very beginning of that pipe where it is attached and “takes root” from the heart.

Aortic Root Support

In some people this blood vessel is weak and, with no more than normal blood pressure, can give way.
The “support” is designed to prevent that.

External Aortic Root Support

The support is “external” in the sense that it is positioned around the outer surface of the pipe without the need to cut into it.

Marfan Syndrome is the most likely reason to be interested in this site

People with Marfan syndrome typically have more than one of the following bodily characteristics.

Tall with an arm span greater than their height.
Long fingers and toes.
Very flexible joints.
Narrow jaws with crowded teeth.
A particular pattern of widening of the aortic root.

This website is particularly relevant to people with a wider than normal aortic root.

Understanding a bit more about Marfan syndrome

A “syndrome” is a word from before scientific medicine when doctors recognised a common pattern in people who seemed to have a group of features in common.
Many but not all people with Marfan syndrome have an abnormal aortic root.
There is an underlying structural problem. Again, think about a house. If the builder had a very dodgy batch of cement, problems may later come to light in the concrete foundations, the mortar supporting the bricks, the screed on the walls and the chimney stack. In Marfan syndrome the material that crops up all over the place (the spine,the eyes, the joints, the heart) is called fibrillin.
Going a layer deeper into biology, the genes, the design that programmes the growth of every bit of the body, is the source of the problem. People with Marfan syndrome do not have the genes to make good quality fibrillin – sorry!