The bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality. Though most often isolated, BAV may be associated with other cardiovascular malformations. BAV-related aortopathy is the most common, sharing genetic alterations and phenotypic heterogeneity characteristics. Sometimes silent for a lifetime, BAV may manifest as aortic valve dysfunction, aortic aneurysm, or more emergent situations, such as endocarditis or aortic dissection. Its embryological origin and the characterization of the genes involved, as well as the histopathological and hemodynamic aspects of its natural history, are becoming increasingly clear. In addition, emerging evidence of rhythm disorders associated with BAV has been identified. A new international nomenclature and classification has been introduced to interpret all the advances made in recent years for the comprehension of this condition. In the guidelines, more attention has been paid to the diagnosis of BAV and related aortopathy, together with surveillance, and family screening. Surgical treatment remains the gold standard, especially in young low-risk patients, and valve repair techniques have been shown to be effective and durable. Finally, the new era of transcatheter techniques is also being applied to dysfunctional BAV, allowing the treatment of patients at high surgical risk, with increasingly promising results, and the possibility of expanding indications through the introduction of more advanced devices. This review aims to comprehensively describe the BAV conundrum, focusing on anatomy, pathophysiology, genetics, diagnosis of BAV-related disorders, and the different treatment options available in the transcatheter era.

The aim of this retrospective study is to show medium-long-term results in terms of cardiac death and aortic events in patients undergoing reduction ascending aortoplasty between 1997 and 2009 in our hospital. The Fine and Grey model for competing risk analysis was performed for time to cardiac death, with non-cardiac death as the competing risk, and time to recurrence of both re-dilation (aortic diameter > 45 mm) and re-operation with overall death as the competing risk. Paired t-test was used to evaluate the change in aortic diameter from the post-operative values to follow-up. The population included 142 patients. The mean pre-operative aortic diameter and the diameter at follow-up were respectively 46.5 ± 5.11 mm vs. 41.4 ± 5.55 mm (p-value < 0.001). At a mean follow-up of 11.6 ± 4.15 years, 11 patients (7.7%) required re-operation on the ascending aorta. At 16 years, the CIF of aortic-related events was 29.4 ± 7.2%; the freedom from cardiac death was 89.2 ± 3.7%. Ten patients (7%) died from cardiac causes but no one was aortic-related. The Fine and Grey analysis did not identify any significant predictors. This procedure is safe but might be justified only in high-risk patients or in those with advanced age/short life expectancy.

A retrospective study on the 10-year outcomes of transcatheter edge-to-edge repair using the MitraClip device for severe mitral regurgitation in high-risk patients.