Valvular and Subvalvular

Aortic Stenosis with Bicuspid Case 97

Aortic Valve

A young woman presented with a history of atypical chest ventricular sizes and functions and mild concentric left
pain and a systolic murmur (grade III/VI) in the aortic area ventricular hypertrophy.
without ejection clicks. She had normal left and right

a b

Fig. 97.1 Two linear densities protrude from the left side of the
interventricular septal basal wall and the base of the anterior mitral
valve leaflet toward the left ventricular outflow tract, in favor of
subvalvular aortic stenosis (circumferential membranous type).
The arrows in (a, b) show this membrane, which is attached to the
interventricular septum and is about 4 mm in size (c). (a) Apical
five-chamber view, (b) parasternal long-axis view by four-dimensional
reconstruction, and (c) parasternal long-axis view by two-dimensional
echocardiography. AV aortic valve, LA left atrium, LV left ventricle

Electronic supplementary material The online version of this chapter

(doi:10.1007/978-3-319-12934-1_97) contains supplementary material,
which is available to authorized users.

H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease, 313

DOI 10.1007/978-3-319-12934-1_97, © Springer International Publishing Switzerland 2015
314 Case 97 Valvular and Subvalvular Aortic Stenosis with Bicuspid Aortic Valve

Fig. 97.2 The color-flow Doppler study in the parasternal long-axis b

view shows systolic turbulency, which originates from the subvalvular
area (two arrows). LV left ventricle, AO aorta

Fig. 97.4 A thick aortic valve (a) and the fishmouth appearance of the
aortic valve opening, suggestive of a bicuspid aortic valve, can be seen
here. A raphe (arrow) is visible between the right and left coronary
cusps. (a, b) Short-axis views. LA left atrium, RA right atrium, RCC
right coronary cusp, LCC left coronary cusp, AO aortic valve, RV right
ventricle
Fig. 97.3 The peak and mean gradients across the left ventricular out-
flow tract are 102 and 71 mmHg, respectively, by the continuous wave
study (apical five-chamber view)

Fig. 97.5 The color-flow Doppler study (parasternal long-axis view) in

diastole shows mild aortic insufficiency (arrow) despite a bicuspid aor-
tic valve and severe subvalvular aortic stenosis. LA left atrium, LV left
ventricle, AO aorta
Case 97 Valvular and Subvalvular Aortic Stenosis with Bicuspid Aortic Valve
Diagnosis
The patient was diagnosed with valvular and subvalvular
aortic stenosis (membranous type) with a severe left ventric-
ular outflow tract gradient, bicuspid aortic valve, and mild
aortic insufficiency.
Comment
Since the patient had valvular and subvalvular aortic steno-
sis, TEE was recommended for a direct planimetry of the
315
aortic valve area and investigation for the need for aortic
valve replacement before surgery.
Lesson
When there is valvular and subvalvular aortic stenosis, it is
crucial to determine the degree of valvular aortic stenosis.
TEE by direct planimetry can help to measure the aortic
valve area.