Applanation tonometry for Marfan syndrome: a novel tool to improve timing of prophylactic aortic root operations?

Background: Non-invasive applanation tonometry (APT) is useful to assess aortic stiffness and pulse wave reflection. Moreover, APT can predict outcome in many conditions such as arterial hypertension. In this study we test whether APT measurements relate to progression of aortic disease in Marfan syndrome (MFS).

Methods and results: We performed APT in 50 consecutive, medically treated adults with MFS (19 men, 31 women aged 32±13 years), who had not undergone previous cardiovascular surgery. During 22±16 months of follow-up, 26 of these patients developed progression of aortic disease, which we defined as progression of aortic root diameters ≥5mm/annum (18 individuals), aortic surgery ≥3 months after APT (7 individuals), or onset of acute aortic dissection any time after APT (1 individual). Univariate Cox regression analysis suggested an association of aortic disease progression with age (P=0.001), total cholesterol levels (P=0.04), aortic root diameter (P=0.007), descending aorta diameter (P=0.01), aortic root ratio (P=0.02), and augmentation index (AIx@HR75; P< .006). Multivariate Cox regression analysis confirmed an independent impact on aortic disease progression exclusively for baseline aortic root diameters (HR=1.347; 95% CI 1.104–1.643; P=0.003) and AIx@HR75 (HR=1.246; 95% CI 1.029–1.508; P=0.02). In addition, Kaplan-Meier survival curves analysis illustrated significantly lower rates of aortic root disease progression both with lower AIX@HR75 (P=0.025) and with lower PWV values (P=0.027).

Conclusions: We provide evidence that APT parameters relate to aortic disease progression in medically treated patients with MFS. We believe that APT has a potential to improve timing of elective aortic surgery in MFS patients.