It has been developed in consultation with the clinicians who will be using it, and so is responsive to their needs. The display allows the user to judge where a particular patient's root diameter places them in relation to the whole Marfan population. The display of a patient's echo data over time shows how echo measurements can vary and highlights the difficulties in relying on a single cut-off point for what is usually a variable echo history. Comparison with a normal population gives little insight. The prototype computer system that has been developed displays serial aortic root measurements, standardised for height and age.
Further development is planned to add facilities to aid clinical decision making.
Although the system has considerable potential, it is as yet unproven. We intend to assemble serial echocardiogram data on 500 Marfan syndrome patients from several centres. We will use these data to calibrate and test the system's performance in determining when patients are at imminent risk of aortic dissection and in urgent need of surgery. As part of this programme of research, there will be a need to improve the precision and scope of the reference ranges used to adjust for age and height. Other potential factors affecting aortic root diameter may need to be taken into account.