Transesophageal echocardiographic assessment of type 0 and type 1 bicuspid aortic valve: clinical implications
A Bakalli, B Mustafa, X Krasniqi, R Lila, N Rexha, K Bince, A Jashari, A Berisha, G Bytyci, G GojaniAbstract
Introduction
Bicuspid aortic valve (BAV) is the most common congenital heart defect, with an incidence of 1–2%. Over one-third of patients develop serious complications during their lifetime. According to the Sievers classification, BAV is categorized into types 0, 1, and 2, based on the number of raphe, which are located below the functional commissures and do not reach the level of the coronary ostia.
Objective
To characterize BAV phenotypes using transesophageal echocardiography (TEE) and to analyze complications according to phenotype.
Methods
We conducted a retrospective study using the TEE database from January 2023 to December 2025, identifying 32 patients with BAV. Patients were divided into two groups according to phenotype: type 0 (group 1, n = 15) and type 1 (group 2, n = 17). Clinical and echocardiographic parameters were compared between the groups.
Results
Among the 32 patients, 20 (62.5%) were male and 12 (37.5%) female (p=0.047), with a mean age of 41.9 ± 14.6 years. Regarding BAV type, 15 patients (46.9%) had type 0 and 17 patients (53.1%) had type 1; no patients were classified as type 2. As shown in the table, the mean transaortic gradient and aortic regurgitation grade were significantly higher in the type 0 group. Aortic dimensions, particularly the aortic bulb, were generally larger in the type 0 group, although this difference did not reach statistical significance. In figure 1 the upper two TEE images (2D and 3D) illustrate a type 0 bicuspid aortic valve in a 17-year-old female patient, while the lower two images (2D and 3D) depict a type 1 bicuspid aortic valve with a raphe between the non-coronary and right coronary cusps in a 43-year-old male patient.
Conclusions
Identification of BAV phenotypes is crucial for accurate diagnosis, particularly in the presence of raphe, which can complicate assessment. TEE is particularly helpful in this regard, as raphes can be mistaken for true cusps on transthoracic echocardiography, potentially leading to missed diagnosis. However, type 0 BAV is associated with higher transaortic gradients, more severe aortic regurgitation, and larger aortic bulb dimensions, reflecting more pronounced hemodynamic impact. Phenotypic classification is therefore essential for clinical evaluation and long-term follow-up of these patients.Table 1For image description, please refer to the figure legend and surrounding text.Figure 1For image description, please refer to the figure legend and surrounding text.