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Objectives: To determine the clinical morphology of congenital bicuspid aortic valves (BAV) and its relationship with congenital cardiac malformations in children.Methods: 252 patients (age<18 years old) with BAV who were identified by transthoracic echocardiography were included. All patients were divided into two groups, group 1 was ≤3 y, and group 2 was >3 y. To estimate the frequency of BAV associated with other cardiac malformations in all patients and to analyze BAV and the frequency of major congenitally cardiac malformations by age and sex roughly.Results: The mean age of the patients was 3y (1 day-17.9 years), males 188 (67.4%), females 64 (32.5%). R-L phenotype (absence of the inter coronary commissure) was the most common(71.4%), both in patients with isolated BAV (58.9%) and in those with additional pathology. L-N phenotype was rarely (1.6%), and 2 of 4 patients with this pattern had no other structural abnormality. The majority of patients with coarctation (82.4 %) and left heart obstructive lesions (80%) had R-L phenotype, non-left heart defects (79.8 %) had R-L phenotype. The difference between the numbers of aortic stenosis(AS) and aortic regurgitation(AR) of BAV patients was not remarkable. AR was observed in 20.2% of BAV patients. In R-L phenotype,most patients associated with AS(80.6%, P<0.05). Compared with the other two BAV types, patients with R-N phenotype were more likely to have at least moderate AR(78.4%, P<0.05) or AS and AR (74%, P<0.05). In male group: intracardiac shunt, coarctation and congenital mitral valve disease were common than female in <3y group. In group2: Intracardiac shunt and congenital mitral valve disease were most common in male. BAV in conjunction with aortic coarctation was associated with a lower prevalence of valve stenosis and regurgitation. In group 1, inracardiac shun, coarctation and congenital mitral valve disease were common in male than female. In group 2, intracardiac shunt and congenital mitral valve disease were most common in male group than female, but the prevalence of coarctation was not too high. Most aortic coarctation tended to be younger boys.Conclusions: Different morphologies of BAV were associated with different cardiac abnormalities, especially left-heart obstructive lesions. The AS was most often observed in patients with R-L phenotype. Most of AR and/or both of AS and AR could be observed in patients with R-N phenotype. Boys with BAV had higher risk of aortic coarctation and congenital mitral valve abnormalities than girls in ≤3 years group, but this difference decreased between sex in >3 years group.