Abstract
57-years old man presented with exertional dyspnea. An early systolic
murmur was heard over the aortic areas 2D and 3D Echocardiography
revealed unicuspid , unicommissural aortic valve (UAV) with a
characteristic “teardrop” lateral orifice (Figure A) and moderate
valve stenosis (3D planimetered aortic valve area (AVA) is 1.1cm2)
(Figure B) Continuous wave Doppler across aortic valve (AV) showed high
peak and mean systolic gradients of 85 and 60mmHg respectively.(Figure
C). 2D /3D Transesophageal Echocardiography (TOE) revealed a subaortic
ridge attached to the posterior annulus (Arrow) (Figure D) Further
En-face viewing of the aortic valve from the left ventricular outflow
tract (LVOT) perspective showed a shelf-like ridge extending from the
commissure to the cusp (Arrow) (Figure E) Zoomed mode of the aortic-
LVOT junction confirmed the presence of the subaortic ridge seen
attached to the posterior aortic annulus near the commissural opening
(Figure F) The patient was referred for surgical consultation ..
Unicupid aortic valve (UAV) is a rare congenital anomaly that has.2
subtypes ; unicomissural and acommissural subtypes. Both can present
with variable degrees of the aortic stenosis (AS) and/or aortic valve
regurgitation (AR).UAV has more early, accelerated and severe valvular
degeneration in addition to smaller orifice in comparison with bicuspid
and tricuspid aortic valve. Echocardiography is the gold standard for
diagnosis and evaluation of the AV morphology and function and the
associated disorders such as ventricular septal defect , aortopathy and
subaortic obstruction.. Surgical aortic valve replacement (AVR) and
repair of the associated anomalies are the most common treatment
modality .