Congenital heart disease
Progression of Aortic Dilation in Children With a Functionally Normal Bicuspid Aortic Valve

https://doi.org/10.1016/j.amjcard.2006.04.022Get rights and content

Adults with bicuspid aortic valves (BAVs) are at risk for progressive aortic dilation independent of valve function. The evolution of aortic dilation in children with functionally normal BAVs has not been studied. In this study, ascending aortic diameters were assessed in a group of children with functionally normal BAVs (peak gradient ≤16 mm Hg) to determine whether progressive dilation occurs during childhood. A cohort of 101 patients and 97 controls were studied with echocardiography. There were no differences in mean age (9.0 ± 4.8 vs 8.7 ± 6.1 years, p = 0.7) or body surface area (1.1 ± 0.4 vs 1.1 ± 0.5 m2, p = 0.9). Patients had significantly greater aortic dimensions than controls in all measured regions of the aorta, with the most striking discrepancy in the ascending aorta (2.3 ± 0.6 vs 1.8 ± 0.5 cm, p <0.0001). The size discrepancy was present from birth and persisted throughout childhood. Repeated measurements were performed in a subgroup of 28 patients and 25 controls. Patients had significantly greater increases in ascending aortic dimension than controls per year of follow-up (1.2 ± 0.08 vs 0.6 ± 0.08 mm/year, p <0.0001). In conclusion, BAV is complicated by progressive aortic dilation beginning in childhood.

Section snippets

Methods and Results

An institutional review board–approved echocardiographic and clinical analysis of children with isolated BAVs and healthy controls was undertaken. Patients with isolated BAVs were those with no greater than trivial aortic insufficiency and peak transvalvar aortic gradients of ≤16 mm Hg. Our aim was to determine whether a functionally normal BAV is a benign lesion or whether such patients require more frequent follow-up because of aortic disease progression early in life. Patients and controls

Discussion

Although aneurysmal aortic dilation has been documented in adults with isolated BAVs,5 this study supports the postulate that BAV is a disease of the entire aorta, with an associated aortopathy present from birth.6 In the absence of even minor hemodynamic alterations, we have shown aortic dilation to be present and progressive throughout childhood in patients with BAVs.

Cited by (0)

View full text