Background: The Marfan syndrome is a disorder of connective tissue, affectinq 1 in every 3000-5000 newborn. In Marfan patients poor survival is mainly determined by aortic dilation and dissection at a relatively young age. Prognosis is favourably influenced by prophylactic surgery of aortic aneurysms. Yet, unanticipated aortic dissection and rapid growth of small aortic aneurysms may occur both in uncomplicated Marfan patients (non-surgical group) and in prophylactically operated patients (surgical group, Bentall procedure). The expected 10 year-complication rate in both groups is approximately 30%. In 1996 we studied aortic elasticity in a non-surgical group of 73 Marfan patients, using magnetic resonance (MR) techniques. In 70% of these patients aortic elasticity was significantly decreased. In 8 patients (11%) with severely decreased aortic elasticity a major event (surgery for excessive aortic growth or dissection) was already evident after 2-3 years of clinical follow-up. These preliminary results suggest that aortic elasticity may contribute to risk stratification in Marfan patients and therefore justify 1) a more elaborate follow-up of the non-surgical group and 2) extrapolation of the study protocol to the surgical group.
Hypothesis: Aortic elasticity may serve as an additional risk determinant for excessive aortic growth and dissection in Marfan patients.
Study objectives: To assess the prognostic value of aortic elasticity on excessive aortic dilation rate (> 1 mm/year) and the occurrence of aortic dissection in non-surgical and surgical Marfan patients.
Methods: The predictive value of aortic elasticity on the occurrence of aortic dissection and excessive aortic dilation rate will be assessed both in non-surgical (n= 73) and surgical patients (n=75), after a follow-up period of 3 and 6 years, respectively. Aortic elasticity and aortic dimensions will be assessed by means of MR techniques, combined with MR-adapted Finapres ® continuous blood pressure measurement.
Expected results: Decreased aortic elasticity is an early marker of aortic disease and offers additional clinical value in the prevention of future aortic complications in Marfan patients, both before and after aortic surgery. |
