Bicuspid aortic valve with aortic stenosis and subvalvar aortic stenosis

Subvalvular aortic stenosis (SAS), also called subaortic stenosis, is a rare disorder seen in infants. In most cases, it involves the presence of a membrane that is typically muscular just below the aortic valve, which causes a fixed obstruction to blood flow across the left ventricular outflow tract. The course of subvalvular aortic stenosis is gradual and patients rarely present with isolated symptoms. SAS may co-occur with a ventricular septal defect, patent ductus arteriosus, coarctation of the aorta, bicuspid aortic valve, abnormal left ventricular papillary muscle, and/or atrioventricular septal defect, among others. In the majority of the patients, SAS is incidentally found when evaluating patients for other congenital heart defects. This activity describes the evaluation, diagnosis, and management of subvalvular aortic stenosis and highlights the role of team-based interprofessional care for affected patients.