Congenital @entity886 are the cause of @entity472 in pediatric and younger adult populations. Despite being very rare, unicuspid aortic valves account for approximately 5% of isolated aortic valve replacements. @entity1 with @entity472 , present with the same symptomatology independent of leaflet morphology. However, the presence of bicuspid and unicuspid @entity472 is associated with a higher incidence of aortopathy, especially in @entity2689 @entity1 . @entity2689 , an X monosomy, is associated with @entity886 , aortopathy, and @entity101 . These risk factors lead to a higher incidence of @entity1580 in this population. @entity1 with @entity2689 and @entity472 that present for aortic valve replacement should therefore undergo extensive aortic imaging prior to surgery. Transthoracic echocardiography is the diagnostic tool of choice for valvular pathology, yet it can misdiagnose unicuspid aortic valves as @entity4503 due to certain similarities on imaging. Transesophageal echocardiography is a better tool for distinguishing between the two @entity340 , although diagnostic errors can still occur. We present a case of a 50-year-old female with history of @entity2689 and @entity4503 presenting for aortic valve replacement and ascending aorta replacement. Intraoperative transesophageal echocardiography revealed a stenotic unicommissural unicuspid aortic valve with an eccentric orifice, which was missed on preoperative imaging. This case highlights the importance of intraoperative transesophageal echocardiography in confirming preoperative findings, diagnosing further cardiac pathology, and ensuring adequate surgical repair.

Unicuspid Aortic Stenosis in a XXXX with Turner Syndrome: A Case Report.

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients'] @entity4503 :: ('MESH:C562388', 'Disease') :: ['bicuspid aortic stenosis', 'bicuspid valves'] @entity340 :: ('MESH:D006349', 'Disease') :: ['valvular abnormalities'] @entity101 :: ('MESH:D006973', 'Disease') :: ['hypertension'] @entity886 :: ('MESH:C563178', 'Disease') :: ['aortic valve anomalies'] @entity2689 :: ('MESH:D014424', 'Disease') :: ['Turner syndrome'] @entity472 :: ('MESH:D001024', 'Disease') :: ['premature aortic stenosis', 'aortic stenosis'] @entity1580 :: ('MESH:C562834', 'Disease') :: ['aortic dissection']
@entity1 :: (9606,Species) :: ['Patient']
