Anatomical and clinical predictors of valve dysfunction and aortic dilation in bicuspid aortic valve disease

Abstract

ObjectiveBicuspid aortic valve (BAV) is associated with early valvular dysfunction and proximal aorta dilation with high heterogeneity. This study aimed to assess the determinants of these complications.MethodsEight hundred and fifty-two consecutive adults diagnosed of BAV referred from cardiac outpatient clinics to eight echocardiographic laboratories of tertiary hospitals were prospectively recruited. Exclusion criteria were aortic coarctation, other congenital disorders or intervention. BAV morphotype, significant valve dysfunction and aorta dilation (≥2 Z-score) at sinuses and ascending aorta were established.ResultsThree BAV morphotypes were identified: right–left coronary cusp fusion (RL) in 72.9%, right–non-coronary (RN) in 24.1% and left–non-coronary (LN) in 3.0%. BAV without raphe was observed in 18.3%. Multivariate analysis showed aortic regurgitation (23%) to be related to male sex (OR: 2.80, p<0.0001) and valve prolapse (OR: 5.16, p<0.0001), and aortic stenosis (22%) to BAV-RN (OR: 2.09, p<0.001), the presence of raphe (OR: 2.75, p<0.001), age (OR: 1.03; p<0.001), dyslipidaemia (OR: 1.77, p<0.01) and smoking (OR: 1.63, p<0.05). Ascending aorta was dilated in 76% without differences among morphotypes and associated with significant valvular dysfunction. By contrast, aortic root was dilated in 34% and related to male sex and aortic regurgitation but was less frequent in aortic stenosis and BAV-RN.ConclusionsNormofunctional valves are more prevalent in BAV without raphe. Aortic stenosis is more frequent in BAV-RN and associated with some cardiovascular risk factors, whereas aortic regurgitation (AR) is associated with male sex and sigmoid prolapse. Although ascending aorta is the most commonly dilated segment, aortic root dilation is present in one-third of patients and associated with AR. Remarkably, BAV-RL increases the risk for dilation of the proximal aorta, whereas BAV-RN spares this area.

Bicuspid aortic valve morphotype: are we closer to solving the mystery?

“…Additionally, the authors observed no differences in ascending aorta diameter between the BAV phenotypes,1 which is also consistent with our results 2. In our meta-analysis, we demonstrated that the aortic root diameter in BAV type I was significantly larger (on average of 1.66 mm/m 2 indexed and 2.0 mm non-indexed diameter of aorta at the level of sinus of Valsalva) in comparison to BAV type II.…”

“…Several previous studies reported significant disparities in the relationship between aorta dilation and BAV characteristics. However, the excellent study of Evangelista et al allows us to feel closer to solving the mystery of BAV morphology and its impact on aortic diameter 1. Their multicentre study on the impressive and the largest ever population of 852 patients with BAV, using prespecified protocol and core lab review of all images to define BAV morphology and function along with aortic dilation, covered multiple aspects (valve dysfunction and proximal aorta dilation) of heterogeneous BAV population.…”

Familial clustering of bicuspid aortic valve and its relationship with aortic dilation in first-degree relatives

“…Exclusion criteria were a non-definitive diagnosis of BAV or the presence of another connective tissue disorder. From 852 patients included in our previous study,18 256 agreed to undergo family screening. Anamnesis, three-generation pedigree, physical examination and transthoracic echocardiography were performed in each index case.…”

Heartbeat: phenotypic heterogeneity of bicuspid aortic valve disease

“…In this issue of Heart , Evangelista and colleagues1 sought to identify phenotypic predictors of valve dysfunction and aortic root dilation in a series of 802 consecutive adults diagnosed with BAV at eight tertiary care hospitals. As in previous studies, BAV leaflet morphology characterised by fusion of the right and left (RL) coronary cusps was most common (73%), followed by fusion of the right and non-coronary cusps (24%).…”