Abstract 19063: TGF-β Levels Are Associated with Aortic Root Dilatation, Aortic Root Growth Rate and Clinical Events in Patients with Marfan Syndrome

Abstract

Background: Marfan syndrome (MFS) is a connective tissue disorder with variable aortic root dilatation. Recently, increased transforming growth factor beta (TGF-β) signalling emerged as a new potential biochemical pathway leading to aortic disease. The aim of this study was to investigate whether TGF-β is correlated with aortic root dilatation, exploring the role of TGF-β as a prognostic biomarker in MFS. Methods: In this correlation study between aortic root diameters and TGF-β, 86 MFS patients were included. Twenty-five patients had previously undergone aortic root replacement. Total plasma TGF-β was assessed at baseline in patients and 22 healthy controls. Aortic root diameters were measured by means of echocardiography with a mean follow-up of 32 months. Clinical events, including aortic dissection and aortic root replacement were evaluated. Results: At baseline TGF-β levels were higher in MFS patients as compared to controls (112.6 vs 33.5 pg/ml, p=0.019). Patients with previous aortic root replacement had significantly higher TGF-β levels compared to patients without aortic root repair (168.7 vs 89.7 pg/ml, p=0.008). Higher plasma TGF-β levels correlated with larger aortic root diameter at baseline (p=0.047) and at follow-up (p=0.006). Moreover, there is a correlation between higher TGF-β levels and faster aortic root growth rate (p=0.022). After 32 months of follow-up 17 events were observed: aortic root replacement in 13 patients and type b dissection in four patients. Patients with an event had higher aortic root diameters at baseline (45.6 vs 39.6 mm, p=<0.0001) and higher TGF-β levels (160 vs 100 pg/ml, p=0.014) compared to patients without event. Conclusion: TGF-β levels are higher in MFS patients than in healthy controls. Higher TGF-β plasma levels were correlated with larger aortic root diameters, faster aortic root growth and aortic root replacements or type b dissections. Plasma TGF-β might serve as a prognostic biomarker in Marfan syndrome.