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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5761_Библиотеки_им_академика_М_И_Перельмана
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332
Diagnosis
The patient was diagnosed with a bicuspid aortic valve,
severe aortic regurgitation, moderate aortic stenosis, dilation
of the ascending aorta (51 mm), and severe mitral valve
regurgitation secondary to the rupture of the chordae of the
posterior mitral leafl et owing to the prolapse of the mitral
valve.
Comment
The patient was referred for the Bentall operation and mitral
valve replacement or repair following work-up for infective
endocarditis.
Lesson
The bicuspid aortic valve is sometimes associated with the
mitral valve prolapse [ 2 ].
ab
Fig. 103.5 There is severe mitral valve regurgitation on TEE (long-
axis view) ( arrow ) ( a ). TEE (0°) shows a mobile mass on the atrial side
of the posterior mitral leafl et, most probably owing to the rupture of the
chordae of the posterior mitral leafl et or vegetation ( arrow ) ( b ). LA left
atrium, RA right atrium, LV left ventricle, RV right ventricle, AO aorta
Fig. 103.4 The ascending aorta is dilated (up to 51 mm) on TEE
(long-axis view). LA left atrium, RA right atrium, AO aorta
Fig. 103.3 The aortic valve is bicuspid on TEE (short-axis view). LA
left atrium, RA right atrium, AO aorta. 1 fi rst aortic leafl et, 2 second
aortic leafl e t
Case 103 Bicuspid Aortic Valve and Rupture of the Chordae of the Posterior Mitral Leafl et
333
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_104, © Springer International Publishing Switzerland 2015
A young man with a history of palpitation and two episodes
of syncopal attacks and a systolic murmur (grade IV/VI) in
the aortic area was referred to our echocardiography laboratory for an evaluation of the cardiac cause of the syncopal
attacks. Echocardiography revealed normal left and right
ventricular sizes and systolic functions as well as concentric
left ventricular hypertrophy.
Supravalvular Aortic
Stenosis – Hourglass Type
Case 104
Fig. 104.1 The parasternal long-axis view illustrates the narrowing of
the sinotubular junction ( arrow ) and the hourglass appearance of the
aorta. LA left atrium, LV left ventricle, RV right ventricle, AO aorta
Electronic supplementary material The online version of this chapter
(doi:
10.1007/978-3-319-12934-1_104 ) contains supplementary material,
which is available to authorized users.
334
Fig. 104.2 The sinotubular
junction is about 17 mm in size,
compared with the annulus,
which is 20 mm, in the
parasternal long-axis view. The
ST junction/annulus is 17/20, and
the normal ratio of the ST
junction/annulus is greater than
or equal to 1 [ 2 ]. AO aorta
Fig. 104.3 The dilation of the ascending aorta is up to 42 mm on trans-
esophageal echocardiography (TEE) (long-axis view), with a mild
withdrawal of the probe. AO aorta
Case 104 Supravalvular Aortic Stenosis – Hourglass Type
335
c
a b
Fig. 104.4 The narrowing of the sinotubular junction ( arrow ) and the
hourglass appearance of the ascending aorta are suggestive of supravalvular aortic stenosis ( a ). The doming of the aortic valve ( arrows ) ( b ) is
seen in this view, which is in favor of accompanying valvular aortic
stenosis. ( a , b ) Transesophageal echocardiography (120°). LA left atrium,
LV left ventricle, RV right ventricle, AO aorta, STJ sinotubular junction.
This is a schematic illustration of the valvular and supravalvular aortic
stenosis in this view ( c ) IVS interventricular septum, MV mitral valve
ab
Fig. 104.5 The asymmetrical closure of the aortic valve ( arrow )
( a ) and the fi shmouth appearance of the aortic opening in systolic time
( arrow ) ( b ) are suggestive of a bicuspid aortic valve. The raphe of the
bicuspid aortic valve ( double arrows ) should be diagnosed from the
third leafl et of the tricuspid aortic valve. ( a , b ) Transesophageal echo-
cardiography (120° and short-axis view)
Case 104 Supravalvular Aortic Stenosis – Hourglass Type
336
Fig. 104.6 The aortic valve area via direct planimetry is approxi-
mately 2.1 cm
2
on TEE (short-axis view)
Fig. 104.7 The continuous wave Doppler study of the aortic valve in
the apical fi ve-chamber view shows that the peak and mean transaortic
gradients are 97 and 66 mmHg, respectively
Fig. 104.8 TEE (120°) reveals
that the systolic turbulency
originates from the aortic valve
and sinotubular junction,
denoting that there is valvular
and supravalvular aortic stenosis
Case 104 Supravalvular Aortic Stenosis – Hourglass Type
337
Diagnosis
The patient was diagnosed with mild valvular and severe
supravalvular aortic stenosis with severe obstruction, as well
as a bicuspid aortic valve, mild aortic regurgitation, and dilation of the ascending aorta (up to 42 mm); this is the more
common form of supravalvular aortic stenosis (hourglass
form with dilation of the ascending aorta) [ 87 ].
Comment
Surgery was recommended for the patient. The use of the Y
patch, resection with end-to-end anastomosis, and the Ross
procedure are the main techniques employed with valvuloplasty [ 2 , 118 , 119 ].
Fig. 104.10 This is a schematic illustration of the hourglass form of
supravalvular aortic stenosis with stenosis of the sinotubular junction
and dilation of the ascending aorta DAO descending aorta
Fig. 104.9 TEE (120°) depicts a
faint diastolic fl ow ( arrow ) across
the aortic valve, in favor of mild
aortic insuffi ciency
Case 104 Supravalvular Aortic Stenosis – Hourglass Type
339
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_105, © Springer International Publishing Switzerland 2015
A 13-year-old boy presented with frequent episodes of
syncopal attacks. Physical examination showed a systolic
ejection murmur (grade IV/VI) in the aortic area with radiation into the jugular notch and along the carotid vessels. An
ejection click was not heard, and the blood pressure was
120/80 mmHg in the left arm and 140/80 mmHg in the right
arm. The patient had regular but unequal upper extremity
pulses with prominence in the right upper extremity. He was
referred to us for an evaluation of the cardiac cause of the
syncopal attacks. Transthoracic echocardiography revealed a
relatively small left ventricular cavity and a normal systolic
function as well as concentric left ventricular hypertrophy.
Supravalvular Aortic
Stenosis – Tubular Type
Case 105
ab
Fig. 105.1 The apical four-chamber view shows systolic turbulency ( arrow ) in the left ventricular cavity ( a ) and a 45-mmHg intraventricular
gradient (dynamic type) via the continuous wave study ( b ). LA left atrium, LV left ventricle, RA right atrium, RV right ventricle
Electronic supplementary material The online version of this chapter
(doi:
10.1007/978-3-319-12934-1_105 ) contains supplementary material,
which is available to authorized users.
340
Fig. 105.2 The continuous wave study of the aorta shows a peak
gradient of about 100 mmHg
ab
Fig. 105.3 The suprasternal view reveals systolic turbulency, which
begins at the site of the sinotubular ( ST ) junction and is in favor of
supravalvular aortic stenosis via the color-fl ow Doppler study ( a ), and a
signifi cant narrowing of the ST junction by two-dimensional echocardiography ( b ). AA ascending aorta, STJ sinotubular junction
Fig. 105.4 The subcostal view demonstrates an underdeveloped
descending aorta, about 11 mm in size. The patient underwent transesophageal echocardiography (TEE) for a further evaluation of the aortic
valve and the subvalvular and supravalvular components of the aorta. IVC
inferior vena cava, DAO descending aorta
Fig. 105.5 TEE (short-axis view) shows a tricuspid aortic valve and
dilation of the left main ( arrow ). LA left atrium, RA right atrium,
AV aortic valve
Case 105 Supravalvular Aortic Stenosis – Tubular Type
341
Diagnosis
The patient was diagnosed with the tubular type of supravalvular aortic stenosis with severe obstruction and dynamic
left ventricular outfl ow tract obstruction [ 87 ].
Comment
There was no membrane in the subvalvular area of the aortic
valve; consequently, the 45-mmHg gradient in the left ventricular outfl ow tract was most probably due to the small left
ventricular cavity and left ventricular hypertrophy. Given
the patient’s signifi cant supravalvular aortic stenosis and
several episodes of syncopal attacks, surgery was recommended for supravalvular aortic stenosis (with subaortic
resection).
ab
Fig. 105.7 TEE (99°) reveals a signifi cant narrowing of the ST junction ( arrows ) ( a ) and an ST junction diameter of about 13 mm ( b ). LA left
atrium, AOV aortic valve, STJ sinotubular junction, AO aorta
Fig. 105.6 TEE (short-axis view) depicts a normal aortic valve area of
about 3.6 cm
2
. Dilation of left main is also evident in this fi gure ( arrow ).
AOV aortic valve
Fig. 105.8 The underdeveloped descending aorta is approximately
12 mm in diameter on TEE (0°). DAO descending aorta
Case 105 Supravalvular Aortic Stenosis – Tubular Type
342
Lesson
1. The diagnosis of supravalvular aortic stenosis is made
when there is a gradient across the aorta. In our patient,
however, the aortic valve opening seemed normal or inconsistent with the degree of stenosis. Moreover, the ratio of
the ST junction to the aortic annulus was less than 1 [ 2 ].
2. In patients with supravalvular aortic stenosis, the coronary arteries are subjected to a high pressure and
dilated. Nevertheless, the long-term effect of this
pressure overload is unclear, although the coronary
artery perfusion is mostly a diastolic phenomenon
[ 87 , 118 ].
3. In rare tubular form, ascending, arch, and descending
aorta are hypoplastic [ 87 ].
Supravalvular aortic stenosis is associated with the elastin
gene abnormality on chromosome 7 [ 87 ].
Case 105 Supravalvular Aortic Stenosis – Tubular Type
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