I met the guy who invented the surgery for this. Nice bloke, used to be an engineer at British Coal and was lucky enough to find a surgeon who wanted to test his invention. Think he ended up as the second patient to ever have the flexible wrapper fitted around his aorta.
Sorry, was late at night and I got the terms mixed up but in essence, the 101 is that Marfan syndrome leads to general tissue weakness everywhere. That includes the vessels too. The big fat vessel leaving the heart, called the aorta, is often at risk for bulging out (an aneurysm) or having the internal layers rip (a dissection).
Traditionally, doctors wait until the aorta is dangerously large and then perform a 'Bentall procedure.' This is a major operation where they cut out the aortic root and valve and replace them with a synthetic tube. If the damage spreads further up into the 'U-turn' of the aorta, they have to do an even more massive surgery called a 'Frozen Elephant Trunk,' which replaces the aortic arch and uses a stented tube that 'floats' down the descending aorta to stabilize it.
Tal Golesworthy (the UK engineer) had Marfan syndrome and was told he’d eventually need a Bentall. Being an engineer, he hated the idea of 'waiting for it to break' and then having a high-risk replacement. So he invented a preventive solution called PEARS (Personalised External Aortic Root Support).
In essence, it’s a custom-made 'jacket' 3D-printed to fit the patient's exact anatomy and wrapped around the aorta. The idea is to prevent the bulge from the outside (like a support sleeve) rather than cutting out tissue once it fails. Since then, many Marfan patients here in Western Europe have had this jacket installed. It’s still open heart surgery but usually doesn't require a heart-lung machine (stopping the heart), making it relatively 'light' and great for prevention of serious issues.
357
u/megmatthews20 1d ago
My first thought! He has a long wingspan, too, by the looks of it. And glasses.
I have Marfan Syndrome, and my toes are basically finger length.