Stefek HA, Lin KH, Rigsby CK, Michelena HI, Aouad P, Barker AJ, Robinson JD.
Pediatr Cardiol. 2019 Dec 19. doi: 10.1007/s00246-019-02264-3. [Epub ahead of print]
Select item 31858200
Take Home Points:
- In patients with a bicuspid aortic valve (BAV), root size and cusp fusion pattern is associated with disease outcomes
- This retrospective study characterised aortic sinus symmetry in adult and paediatric patients with a BAV and association to valvulopathy and aortopathy.
- Using CMR studies, 149 adults and 51 children with BAV were characterised and compared to 40 adults and 20 children with normal aortic valve morphology.
- In both adults and children with a BAV, the non-fused aortic sinus was larger than either fused sinus.
- Sinus eccentricity in BAV patients was significantly greater than in patients with a normal aortic valve.
- Degree of eccentricity varies with valve dysfunction and BAV phenotype but, does not relate to degree of aortic root dilation.
- In adults, degree of eccentricity does not correlate with ascending aortic dilation.
Commentary from Dr. Damien Cullington (Liverpool, UK), section editor of ACHD Journal Watch:
The BAV is the commonest congenital cardiac lesion and seen in approximately 0.5%-2% of the population. Congenital cardiologists will frequently encounter the patients with the long-term consequences of BAV – namely, aortic stenosis, aortic regurgitation, a combination of both with and without an aortopathy. It is challenging to predict disease progression in the individual patient. Valves with fusion of right-left coronary cusps are more likely to develop AI whilst valves with fusion of right and non-coronary cusps are more likely to develop stenosis. Furthermore, aortopathy is influenced by different BAV phenotypes, probably due to the eccentricity of the outflow jet through the BAV.
A flowchart showing the process for patient selection is shown in Figure 1. Of the adult patients with a BAV (n=149) the mean age was 48 years old. Of the paediatric patients with BAV (n=51), the mean age was 15 years old. The demographics of patients with BAV phenotype and aortic valve disease and aortopathy is shown in Table 1.
Figure 1. Flowchart of inclusion and exclusion criteria used in formation of paediatric and adult study cohorts (CMR cardiac MRI, AAo ascending aorta, RL BAV with right-left cusp fusion, RN BAV with right-non coronary cusp fusion)
Table 1. Patient demographics in BAV and normal aortic valve cohorts
This study showed that patients with BAV exhibit significant sinus asymmetry characterised by preferential non-fused sinus dominance. Eccentricity is seen in both adults and children – it isn’t associated with age suggesting possible genetic pre-disposition. In this small study, there was no statistically significant difference in degree of eccentricity of the aortic root in either adults or children with RL or RN cusp fusion. Larger observational studies are required.