Reminiscing on scenes from a symposium held in Liverpool last week, where some of the world’s greats in aortic surgery and genetics assembled for Aorta: Structure to Rupture. Liverpool hosts this aortic conference biennially and the Marfan Trust was represented by Joanne, our nurse specialist, and Jessica, one of our in-house researchers, who mingled with clinicians, nurses, patients, and visitors from the US including Dr Duke Cameron, Dr John Elefteriades and Dr Dianna Milewicz.
Amongst the many lectures and discussions unfolding across the symposium was fascinating conversation around how AI may begin to improve the diagnosis of connective tissue disorders (CTDs) by recognising some of the facial features associated with different CTDs. Technology is also playing an increasingly important role in developing our understanding of how and why the aorta dilates and sometimes tears, while exciting work on the flow dynamics of blood in the aorta is helping researchers detect and, hopefully in the future, predict where problems may arise.
Yet perhaps the symposium’s most memorable moments came when patients with Loeys-Dietz syndrome were given the chance to meet the very clinicians behind the name. Following their keynote lectures, Dr Bart Loeys and Dr Hal Dietz hosted a lunchtime forum for patients and we were fortunate enough to join them. The duo were extraordinarily generous with their time, discussing treatment options, fears and concerns, and the ongoing research taking place in the field.
Their take-home messages included:
- The importance of providing advice within the context of the specific gene change
- Considering family history when thinking about surgery and the risk of dissection, getting as much family history information as possible is really helpful
- Dr Dietz emphasized the importance of medication within the Angiotensin Receptor Blocker group, e.g. Losartan, Irbesartan. He commences medication in patients at a young age, regardless of aortic size. He also aims to get individuals to the highest tolerated dose possible. For example, it is better to be on a good dose of either beta blocker or ARB than a very small dose of each.
- Both doctors highlighted the importance of physical activity and remaining active at all stages of life.
Fresh from the conference halls!! Dr Loeys and Dr Dietz shared with us the upcoming publication of their newly updated guidelines for the care of people with Loeys Dietz. Watch this space as the guidelines will be free to access and we will share once they are in print
