At 2 years old, my mother took me to our local doctor for tonsilitis. During my check up he noticed a heart murmur and after referrals to a paediatrician and a cardiologist, I was then on annual reviews to monitor ‘the murmur’.
At 9 years old, my cardiologist looked at my 4 foot 11inch tall mother, looked at me, a much taller 8 year old, and started posing the idea that I might have Marfan Syndrome.
This was the real start of my journey as a person with Marfan Syndrome (or a Marfling as I refer to myself as).
The only fatal risk of Marfans is the dissection of your aorta. For context, a “normal” aorta is <35mm. When a person’s aorta gets to between 45-50mm you are considered at a high enough risk of aortic dissection and a full aortic root replacement is required. Traditionally, when the time comes for an aortic root replacement, the options are limited to an extensive surgery, requiring full heart/lung bypass and the aorta to be replaced with:
- Total Root Replacement: mechanical valve + polyester aortic graft – requiring a life of warfarin.
- Bio-Bentall: tissue valve + polyester aortic graft – that needs replacement roughly every 10-15 years.
- Valve Sparing Root Replacement: retaining your own valve leaflets within a polyester aortic graft – a longer operation with a significant re-operation rate.
At 36 years old, none of these options are ideal.
This is where the Personalised External Aortic Root Support (PEARS) procedure comes in. I first discovered PEARS when I was researching options to help my leaking spinal fluid (another joy of Marfans and a story for another day). It probably helps here to know that I am the kind of person who needs to know. Everything. So I read journal articles, spoke to my doctor, my cardiologist, I even called the surgeon in Australia who does the procedure and reached out to Tal Golesworthy, the pioneer of the PEARS procedure (who to my absolute astonishment answered me!) to ask all the questions.
What I learnt, in summary, was that Tal, a process engineer by qualification and a fellow Marfling, came up with PEARS when he too was wanting to avoid the legacy issues of the valve repairs currently available.
Working with a team of experts, Tal has provided what is now, to my knowledge, the only viable preventative option of reinforcing the aorta from the outside. This essentially seeks to reduce (or eliminate) the risk of dissection and prevent the need for a valve replacement – meaning no warfarin or valve replacements every 15 years!
After my research, I committed to the procedure – and thank goodness I did. Up until recently my aorta had been getting about 1mm bigger each year. In July 2022 year my aorta was 43mm. Then, in my pre-operation scans in April 2023, it showed that my aorta went from 43mm to 46mm – in just 8 months. Further, when my surgeon conducted my PEARS procedure he noted that my aortic wall was thinning, and that it appeared lighter in colour indicating limited blood flow.
I was much closer to aortic root replacement, or even dissection than I thought.
Yes, PEARS is open chest surgery but no bypass means less stress on the body. Yes, the first week post operation isn’t fun but upon writing this, I am 19 days post operation and am recovering really well. Much stronger than after the open heart surgery to repair my mitral valve.
I am beyond grateful for the option to have the PEARS procedure done. I am just the 774th person in the world to have this done and as a result, my aorta went from 46mm to 35mm. Now, because of PEARS it is not foreseeable that I will need aortic root replacement PLUS the risk of dissection has been significantly reduced. I truly believe that the PEARS procedure has saved my life and given me quality of life.
If you are researching this procedure, and are unsure, I implore you to speak to your doctor or cardiologist about it. If they aren’t supportive, keep asking questions, get a second opinion. This is a genuine life changing option for anyone whose aorta is compromised. As someone with a chronic health condition I have learnt that you have to be your own advocate. No one knows your condition or body like you do. If you feel this is an option for you, be your own advocate to get the answers you need to inform your decision.