Introduction: The bicuspid aortic valve (BAV) is the most common congenital cardiac anomaly. The abnormal bicuspid morphology of the aortic valve leads to valvular dysfunction and aortopathy. However, the clinical presentations of BAV are quite heterogeneous with variable valvular dysfunction and abnormalities. We aim to study the correlations between degrees of aortic stenosis and insufficiency, ascending aorta dilation and associated anomalies in different BAV phenotypes in our local population.
Method: In this cross-sectional study, patients who were referred for echocardiography for any reason and were diagnosed with BAV accidentally, as well as those who were candidates for cardiac surgery due to complications of BAV and referred for echocardiography before surgery, were included. The BAV phenotype was defined as anterior-posterior leaflet orientation (BAV-AP) or right-left leaflet orientation (BAV-RL). Aortic stenosis and insufficiency were assessed. Aortic dimensions were measured at the aortic annulus, sinuses of Valsalva, sinotubular junction (STJ) and ascending aorta. Other cardiac anomalies were also recorded.
Results: A total of 141 patients (mean age 34±12.8 ranging from 11 to 74 years) with BAV were enrolled. The male to female ratio was 3:1. The prevalence of BAV-AP and BAV-RL was 56.7% and 43.3%, respectively. Comparing BAV-AP and BAV-RL, no differences in age or in the prevalence of male sex were identified. The pattern of valvular dysfunction was not statistically different between the  two BAV phenotypes, with moderate-to-severe AI being the most common finding (60% in BAV-AP vs. 57.4% in BAV-AP; p= 0.982). Aortic diameter was larger with BAV-AP than BAV-RL at the sinuses (3.49±0.656 cm vs. 3.27±0.507 cm; p= 0.039). Additionally, ascending aorta dilation using the cut off according to sex, age, and body surface area was more common in patients with BAV-AP (60.8% vs. 43.1% in BAVRL; p= 0.043). The most common cardiac anomalies in all patients were patent foramen ovale (8.5%) and aortic coarctation(7.1%) .
Conclusion: This study shows that moderate-to-severe AI is the most common valvular dysfunction in BAV patients, which is one of the independent risk factors for cardiac events in these patients; however, the pattern of valvular dysfunction is not different according to BAV phenotype. BAV-AP is associated with larger sinuses of Valsalva and more prevalent ascending aorta dilation, suggesting the possibility of different hemodynamic derangements or etiological entities between the two types of BAV.