Imagine having a condition that effects your heart and knowing that a necessary major surgery is imminent and will be followed by taking blood thinning medication for the rest of your life. Now, imagine deciding not to undergo the recommended surgery and coming up with your own, customized heart implant. This is the path that the innovative engineer Tal Golesworthy took when he avoided the prescribed heart surgery for his Marfan syndrome, invented the Personalized External Aortic Root Support (PEARS), and was its first recipient in 2004.
Marfan syndrome is a genetic connective tissue disorder. For patients with Marfan syndrome, the tissue made of proteins that holds organs in shape and in place can develop problems due to a genetic protein defect. The eyes, joints, bones, lungs, and other areas of the body can be affected, including the aorta of the heart. The aorta is the main vessel that pumps blood out of the heart. With Marfan syndrome, it can become stretched out instead of relaxing after each pump and eventually burst. This is the situation that Golesworthy was potentially facing. The BBC explains that the complex prescripted surgery “includes replacing the stretched segment of the aorta with an artificial graft. Sometimes surgeons also have to put metal valves inside the heart to replace ones that are cut out,” along with the need for blood-thinning medication. Blood-thinners, which can sometimes make even slight falls dangerous, are often unpopular with active individuals and Golesworthy was both generally active and a skier. He shared with us that he also enjoys clay pigeon shooting, deer stalking, music, 20 century European history, and has been married for 35 years to Teresa.
Personalized External Aortic Root Support
(public domain image)
Golesworthy is a Chartered Engineer, Information Scientist, and Member of the Royal Society of Chemistry with decades of experience in research and development, which helped him to come up with a creative solution to his dilemma. His background includes working with combustion systems, cyclone development, and intelligent filter controller, among other specializations. He has won two SMART (Science, Mathematics and Research for Transformation) Awards for developing low-cost pollution control solutions for domestic scale coal combustion in Poland. Despite his extensive expertise, he is able to describe the concept for his PEARS invention in very relatable terms: “If the hosepipe is bulging, I must get some insulation tape and wrap it round the outside of the hosepipe to stop it bulging.”
More specifically, Golesworthy envisioned and developed a customized sleeve tailored to his aorta which is sewn in place to secure and support the aorta and keep it from growing. But first, Golesworthy had to line up funding and willing surgeons. He started a company called Exstent as part of his efforts to find investors and he pitched his PEARS concept at a Marfan Association annual meeting. There, he met and intrigued Tom Treasure, who was a surgeon at Guy's Hospital London at the time and now works with the Clinical Operational Research Unit at University College, London. Golesworthy, Treasure, John Pepper of the Royal Brompton Hospital London, and engineers from the Imperial College London, among others, collaborated for four years to develop the PEARS process. Golesworthy told us that first, they had to choose an image acquisition system “for the feasibility period of the project;” either Magnetic Resonance Imaging (MRI) or X-ray Computer Tomography (CT). Here’s more on that choice and their early challenges, in Golesworthy’s words:
“As image resolution for MRI and CT was similar when the PEARS project began, and knowing that some considerable time was going to be required in developing a scanning protocol for PEARS, it was decided that MRI would be the safer option. In the event some 30 patient hours were spent in the CAMRIC CMRI scanner at the Royal Brompton Hospital by the author before an appropriate scanning protocol was finalized…the critical imaging resolution was related to the coronary arteries that emerge from the aortic root…Correctly identifying and placing these structures on the aortic model is a critical function as it ensures that the finished implant will not impose pressure on the coronary arteries and compromise coronary blood flow and it informs the surgeon as to the position of the coronary arteries on the aortic root; a critical step in ensuring the safe mobilization of the left coronary artery (LCA) prior to implantation of the ExoVasc® device.”
Another challenge that comes up when imaging the heart is that it is always moving. To avoid the image distortions this can cause, cardiac gating, similar to stop-motion photography, was used, and the patient (Golesworthy) also held his breath to minimize movement. The scans that were taken of Golesworthy’s heart were then converted through CAD and 3D printing into a 3D model. The model was immersed in polymers and the sleeve shape was molded. Mosaic Science explains that the sausage-shaped sleeve is woven out of polyethylene terephthalate, a thermoplastic polymer resin. In 2004, Golesworthy underwent the successful two-hour operation at Royal Brompton Hospital, which he said was “the scariest day of [his] life.” Since the operation, his aorta has been functioning well. Here’s how Golesworthy felt about the successful procedure and healthy resulting lifestyle:
“I am living a life so ‘normal’ that all the usual banalities have crowded in to irritate: work, money, the health of other family members, etc., but I have a freedom and emancipation that only an experience as profound as cardiothoracic surgery (or similar) can bring. Nothing frightens me anymore, I feel able to express myself without let or hindrance and I can live as I will.”
Mosaic also shares that PEARS is not the right solution for all patients; it could be too risky for those with an extremely enlarged aortas but is a good choice for those in early stages of the disease. Golesworthy says “the scanning, CAD modelling and manufacture of the ExoVasc® has only changed in respect of detail changes in the CAD modelling software and the cleaning regime for the textile implant itself. In essence, the process used is the same today as it was for P1 in May 2004.” Numerous patients have now received successfully been treated with PEARS (over 20 in 2016 and 87 total) and you can read about some of their experiences in their own words here. Andrew Ellis, for example, is an athlete who underwent the procedure in 2008 and whose biological father died of the same heart condition in his twenties. This is an excerpt from his reflection on the experience:
“It means I will not have to take Warfarin [blood thinner] for the rest of my life, as I would have had to do if I had undergone the existing, recognized Bentall procedure. For me, there can simply be no price, value, or words attached to that which could possibly sum up what that means.”
In December of 2011, PEARS won The Engineer’s Medical & Healthcare innovation award at the Royal Society. Patient Innovation is a website that enables patients who develop their own illness solutions to share their ideas and experiences. Golesworthy was one of their first guest speakers and won one of their annual Innovation Awards in 2015. Golesworthy shared in November of 2016, “We’ve got new surgeons and new centers. We’ve just done four patients in New Zealand, and they’re really pleased. We’ve got centers in the Czech Republic, a couple in Poland are about to start, and we’re getting two more in the UK.” He explained that his hopes for his invention, which has now evolved into three types of procedures, are that “as many patients as possible get access to PEARS as a surgical option: their choice of PEARS, Total Root Replacement (TRR), or Valve Sparing Root Replacement (VSRR) is theirs to make but they deserve to be offered all 3 principal surgical options.”
You can learn more about PEARS at the Marfan Aortic Root Support site.