Abstract

It is challenging to evaluate aortopathy in congenital heart disease using conventional investigations such as brachial-ankle pulse wave velocity (baPWV). Therefore, we evaluated the cardio-ankle vascular index (CAVI), a dimension of the ascending aorta and plasma transforming growth factor-β1 (TGF-β1) level, in order to find novel noninvasive parameters of aortopathy in adults with repaired tetralogy of Fallot (TOF). Prospectively, we enrolled 42 consecutive adults with TOF (28 patients with repaired TOF were not on ARB, 8 patients with repaired TOF on ARB, 6 patients with no repair or only palliative repair) and 20 age-matched healthy controls. We measured CAVI, baPWV, plasma TGF-β1 level and a diameter of the ascending aorta using echocardiography. The mean age of repaired TOF without ARB were 31.9±9.1years. An aortic diameter of the ascending aorta, CAVI, and plasma TGF-β1 level were significantly higher in repaired TOF without ARB than those in controls, whereas baPWV did not differ. On a univariate analysis, CAVI, plasma TGF-β1 level and Rastelli procedure were important factors for an aortic diameter of the ascending aorta (r=0.56, P<0.01, r=0.59, P<0.01, r=0.39, P<0.05, respectively) in this population. There was no significant correlation with age, baPWV, NT-pro brain natriuretic peptide (BNP), repair age, shunt duration, aortic regurgitation or right aortic arch. On the other hand, patients with unrepaired or palliative TOF, who had obvious volume overload, showed no significant correlation with CAVI, baPWV or plasma TGF-β1 level. CAVI and plasma TGF-β1 level, not baPWV, correlate to aortopathy in adults with repaired TOF.

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