Assessment of Bicuspid Aortic Valve in Children


DOI: 10.2478/jim-2025-0001

ABSTRACT
Background: Bicuspid aortic valve (BAV) is the most common congenital heart defect and is frequently associated with aortic dilation (aortopathy) and other cardiovascular malformations. In pediatric patients, the relationship between BAV morphology and associated valvular and structural anomalies remains an important area of investigation. Aim: To assess the characteristics of BAV in the pediatric population and to analyze its association with other cardiac lesions, including valvulopathies. Methods: This retrospective study included 62 children diagnosed with BAV. We evaluated BAV morphology and analyzed associated valvulopathies and congenital heart defects using clinical and echocardiographic data. Results: BAV was more
common in men, accounting for 80.6% of cases. Type 1 BAV was the predominant phenotype. Aortic dilation was present in 69.3% of patients, aortic regurgitation (AR) in 64.5%, and aortic stenosis (AS) in 48.3%. Other congenital anomalies, such as atrial septal defect, ventricular septal defect, and mitral valve malformations, were observed in 59.5% of cases. No significant associations were found between gender and valvulopathies or between valve morphology and coarctation of the aorta (CoA). However, the R/L phenotype was strongly associated with AR, while the R/N phenotype was more frequently associated with AS. Conclusion: Aortopathy was identified in two-thirds of patients, primarily involving the sinotubular junction and ascending aorta. CoA was significantly associated with a lower prevalence of AS, while AR was notably more frequent in patients with aortopathy.