Objective To explore the distribution patterns and risk factors of abnomal aortic valve function and vascular complications in patients with congenital bicuspid aortic valve (BAV) malformation.

Methods Data from patients with BAV confirmed by echocardiography from January 2012 to December 2015 in our institute were retrospectively collected.

Results A total of 2 781 patients with BAV were included, accounting for 1.1% of the total number of patients examined by echocardiography. Among them, 70.2% were male, and the patients with moderate or higher aortic valve dysfunction accounted for 60.4%, and the patients with concomitant ascending aorta and aortic sinus dilatation accounted for 54.2% and 19.7%, respectively. The detection rate of simple aortic stenosis and mixed aortic valve dysfunction increased with age (P < 0.000 1), while detection rate of simple aortic regurgitation decreased with age (P < 0.000 1). Male gender, right-left cusp fusion type, and simple aortic regurgitation were independent risk factors for aortic root dilatation. Elder age, simple aortic stenosis, and mixed aortic valve dysfunction were independndent risk factors for ascending aortic dilatation.

Conclusions The prevalence of BAV is high and its clinical manifestations are diverse. Aortic valve dysfunction and ascending aorta dilatation are the most common, and their phenotypic differences have guiding significance for the treatment.