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Stefano Nistri, Betti Giusti, Guglielmina Pepe, Filippo Cademartiri, Another piece in the puzzle of bicuspid aortic valve syndrome, European Heart Journal - Cardiovascular Imaging, Volume 17, Issue 11, November 2016, Pages 1248–1249, https://doi.org/10.1093/ehjci/jew169
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Bicuspid aortic valve (BAV) is a syndrome whose natural history is determined by valvular impairment (congenital or acquired) and/or increased prevalence of aortic abnormalities ranging from reduced aortic elasticity to aortic dilatation/aneurysm [thoracic aortic aneurysm (TAA)] and dissection. Familial clustering has been documented in up to 20–30% of patients with isolated BAV, supporting the notion of a relevant genetic background in BAV and highlighting the need for screening first-degree relatives of BAV patients.1
Schnell et al.2 describe the 4D flow cardiovascular magnetic resonance (CMR) findings in 24 BAV relatives (mostly first-degree relatives) with trileaflet aortic valves and normal aortic size, in comparison with those obtained in 15 age-matched healthy individuals. A higher prevalence of cubic and gothic shaped aortas was found in BAV relatives, which were also associated with a significantly higher incidence of vortices in the ascending aortas of BAV relatives than in controls.
This small study generates new intriguing perspectives. To date, there are two main pathogenetic hypotheses of BAV-related aortopathy, i.e. the haemodynamic and the genetic ones.3 Flow abnormalities have been attributed to the inherently dysfunctioning BAV shape, and the critical blood flow pattern in the ascending aorta of BAV patients is considered to be responsible for the development and progression of aneurysm. Schnell et al. show that abnormal flow in the ascending aorta may not be solely due to abnormal valvular shape or to aortic dilatation, suggesting a relationship between aortic shape and vortices in BAV relatives with trileaflet aortic valve and normally sized thoracic aorta.
