Aortic stiffness and aortic dilation in infants and children with tetralogy of Fallot before corrective surgery: evidence for intrinsically abnormal aortic mechanical property.

OBJECTIVE

The present study tested the hypothesis that there is an intrinsic abnormality of aortic elasticity in infants/children with tetralogy of Fallot (TOF) before corrective surgery. The study also determined the independent and quantitative effects of aortic volume load on aortic dilation in this group of TOF patients.

METHODS

Aortic stiffness (pulse wave velocity; PWV) and aortic volume load (aortic volume flow) were measured during catheterization in 37 infants and children with TOF before corrective surgery and in 55 control subjects.

RESULTS

PWV was significantly higher in TOF patients than in controls, irrespective of age, sex, hemodynamic burden on the aortic wall, and existence of aorto-pulmonary shunt. Aortic diameter was also significantly greater in TOF patients than in controls. Multivariate regression analysis identified aortic volume load as an independent determinant of aortic dilation (aortic diameter = 0.72 aortic flow + 26.1 body surface area + 2.79, r(2) = 0.58, p < 0.001). Increased aortic-wall stiffness correlated with the increase in aortic diameter in patients with dominant left-to-right shunt (without aortic volume load); aortic diameter = 0.007 PWV + 13.5 body surface area (BSA) + 6.3 (r(2) = 0.73, p < 0.05).

CONCLUSIONS

The present study highlighted the intrinsic abnormality of the mechanical property of the aortic wall as a feature of aortopathy in TOF. The study also indicated that aortic volume overload and, to a lesser extent, intrinsically high aortic stiffness correlated significantly with aortic dilation in TOF.