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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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274
No gradient could be found through this membrane; however, the gradient via this membrane was 20 mmHg in catheterism and the right ventricular ejection fraction was about
40–45 %.
Diagnosis
The patient was diagnosed with congenitally corrected transposition of the great arteries with subaortic stenosis.
Comment
The patient was referred for surgery, i.e., tricuspid valve
replacement with the preservation of the subvalvular apparatus and the resection of that membrane.
Lesson
Subvalvular aortic stenosis is an associated anomaly with
the congenitally corrected transposition of the great
arteries. It may be due to muscular infundibulum or a
membrane [ 113 ].
Sometimes there are tissue tags below the atrioventricular
valves which cause obstruction in the ventricular outfl ow
tract [ 114 ].
ab
Fig. 83.3 Transesophageal echocardiography (TEE) (long-axis view)
shows severe tricuspid regurgitation (systemic atrioventricular valve
regurgitation) and systolic turbulency, commencing below the aortic
valve (arrow) ( a ). The membrane below the aortic valve is attached to
the septal leafl et of the tricuspid valve (arrow) according to two-
dimensional TEE ( b ). LA left atrium, RV right ventricle
Case 83 Congenitally Corrected Transposition of the Great Arteries with Subaortic Stenosis
275
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_84, © Springer International Publishing Switzerland 2015
A 40-year-old asymptomatic woman was referred to our
echocardiography laboratory because of dextrocardia and a
systolic ejection murmur at the upper right sternal border.
Electrocardiography showed a negative P in leads I and
AVL. Chest X-ray revealed that the stomach and the heart
were located in their right positions.
Congenitally Corrected
Transposition of the Great Arteries,
Dextrocardia, and Subvalvular
Pulmonary Stenosis
Case 84
a
b
Fig. 84.1 Thesubcostal four-chamber view shows that the left atrium,
which receives the pulmonary veins ( arrows ) ( a ), connects to the right
ventricle and that the left atrioventricular valve is located more apically
( arrowhead ) ( b ). The apex of the heart is positioned on the right side of
the chest, i.e., dextrocardia ( curved arrow ). The left atrium is located on
the left side of the right atrium. LA left atrium, RV right ventricle, RA
rigght atrium, LV left ventricle
Electronic supplementary material The online version of this
chapter (doi:
10.1007/978-3-319-12934-1_84 ) contains supplementary
material, which is available to authorized users.
276
a
cd
b
Fig. 84.2 The subcostal four-chamber view reveals that the left
ventricle, which receives the fl ow of the right atrium, connects to a great
artery, which is divided ( arrow ) and indeed is the pulmonary artery
( a , b ). There is systolic turbulency in this artery, in favor of pulmonary
stenosis ( curved arrow ) ( b and c ). This turbulency begins below the
pulmonary valve ( curved arrow ) and is a subvalvular type ( c ). The peak
and mean gradients across the pulmonary valve are 73 and 42 mmHg,
respectively, by the continuous wave Doppler study ( d ). RA right
atrium, LV left ventricle, PA pulmonary artery
Fig. 84.3 There is moderate systemic atrioventricular valve regurgita-
tion ( arrow ). A conus can be detected between the tricuspid valve and
the aortic valve ( curved arrow ). RV right ventricle, AO aorta
Fig. 84.4 Moderate systemic atrioventricular (tricuspid) valve regur-
gitation is seen on transesophageal echocardiography (TEE) (0°)
( arrow ). LA left atrium, RV right ventricle
Case 84 Congenitally Corrected Transposition of the Great Arteries, Dextrocardia, and Subvalvular Pulmonary Stenosis
277
Diagnosis
The patient was diagnosed with visceral situs inversus,
cardiac situs solitus, dextrocardia, corrected transposition
of the great arteries, severe valvular and subvalvular pulmonary stenosis, and mild systemic ventricular dysfunction
(rightventricular ejection fraction of about 45 %).
Comment
The patient was asymptomatic, so medical treatment and
follow-up were recommended.
Lesson
The cardiac situs often follows the visceral situs [ 2 ]. The visceral situs is diagnosed by the abdominal organs (like the liver)
or the aorta and the inferior vena cava. The cardiac situs is characterized by the pulmonary veins and the left atrium. There are
rare cases like this case that cardiac situs does not correlate
with abdominal situs. The atrioventricular valves always follow the ventricles: the tricuspid valve is always attached to the
right ventricle and the mitral valve is always attached to the left
ventricle. The right ventricle is defi ned by the tricuspid valve,
which has a more apical insertion, coarse trabeculation, moderator band, and papillary muscle attachment to the septum,
which is never seen in the left ventricle. (The papillary muscles
in the right ventricle have septophylia, whereas they have septophobia in the left ventricle.) The great artery is defi ned by
bifurcation: the great artery which is bifurcated is the pulmonary artery. The conus is below the pulmonary valve in the normal heart. If the conus below the pulmonary valve is absorbed
and the conus below the aortic valve persists, It will be the
transposition of the great arteries (either complete or corrected).
If the conus below both great arteries persists, it will be defi ned
as a double-outlet right ventricle [ 115 ].
Fig. 84.5 The left atrial appendage ( arrow ) connects to the left atrium
and has the mirror-image pattern of a normal heart. LA left atrium, LAA
left atrial appendage
Case 84 Congenitally Corrected Transposition of the Great Arteries, Dextrocardia, and Subvalvular Pulmonary Stenosis
279
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_85, © Springer International Publishing Switzerland 2015
A 36-year-old woman was referred to our echocardiography
laboratory before surgical consultation. She was under
work-up for infertility. She had no dyspnea or other cardiac
symptoms. Physical examination showed a systolic ejection
murmur with maximal intensity at the pulmonic area.
Electrocardiography showed right-axis deviation and right
ventricular hypertrophy (tall R in lead V1).
Valvular Pulmonary Stenosis
and Atrial Septal Defect
Case 85
Fig. 85.1 The parasternal long-axis view reveals right ventricular
hypertrophy ( arrow ). LA left atrium, LV left ventricle, AO aorta
a
b
Fig. 85.2 There is systolic turbulency in the pulmonary artery in the
parasternal short-axis view ( arrow ) ( a ). The peak gradient across the
pulmonary valve is about 93 mmHg, and the mean gradient is about
56 mmHg in this view ( b ). AO aorta, PA pulmonary artery
Electronic supplementary material The online version of this
chapter (doi:
10.1007/978-3-319-12934-1_85 ) contains supplementary
material, which is available to authorized users.
280
Diagnosis
The patient was diagnosed with severe valvular pulmonary
stenosis and a small ASD (ostium secundum type).
Comment
The patient was referred for the valvuloplasty of the pulmonary valve and follow-up for her small ASD.
Lesson
1. The patient’s ASD was not at the site of the foramen
ovale; it was, as such, not a patent foramen ovale.
2. A peak gradient greater than 60–70 mmHg across the pul-
monary valve is considered as severe pulmonary stenosis
[ 2 , 7 , 116 ].
3. The balloon valvuloplasty of the pulmonary valve is per-
formed with a balloon 20–30 % larger than the pulmonary
annulus, but the risk of pulmonary insuffi ciency is
increased with the use of a larger balloon [ 116 ].
ab
Fig. 85.4 Transesophageal echocardiography (bicaval view) depicts a small atrial septal defect (ASD) (ostium secundum type) by the color
Doppler study ( arrow ) ( a ). The defect measures 3 mm and is near the inferior vena cava ( b ). LA left atrium, RA right atrium, IVC inferior vena cava
Fig. 85.3 The right ventricle is not dilated in the apical four-chamber
view. LA left atrium, LV left ventricle, RA right atrium, RV right
ventricle
Case 85 Valvular Pulmonary Stenosis and Atrial Septal Defect
281
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_86, © Springer International Publishing Switzerland 2015
A 69-year-old man presented with dyspnea on exertion
(functional class II). Physical examination revealed an
ejection systolic murmur at the pulmonic area. Electrocardiography showed right-axis deviation and a prominent R
in the right precordial leads.
Severe Valvular Pulmonary
Stenosis and Patent `
Foramen Ovale
Case 86
Fig. 86.1 The parasternal long-axis view shows the hypertrophy of the
septum and the right ventricular free wall ( arrow ). LA left atrium, LV
left ventricle, RV right ventricle, AO aorta
a
b
Fig. 86.2 The peak gradient across the pulmonary valve is about
97 mmHg, mean gradient is about 55 mmHg ( a ), and subvalvular gradi-
ent is about 17 mmHg ( b ) by the continuous wave fl ow Doppler study
in the parasternal short-axis view
Electronic supplementary material The online version of this
chapter (doi:
10.1007/978-3-319-12934-1_86 ) contains supplementary
material, which is available to authorized users.
282
ab
c
Fig. 86.3 Transesophageal echocardiography (TEE) (long-axis view)
shows that there is a patent foramen ovale (PFO) with a bidirectional
fl ow: the color blue in favor of a left-to-right shunt ( arrow ) ( a ) and the
color red in favor of a right-to-left shunt ( arrow ) ( b ). The distance
between the two layers is about 7 mm ( c ). LA left atrium, RA right
atrium
ab
Fig. 86.4 The bidirectional fl ow of the PFO is also evident on TEE (short-axis view) ( arrow ) ( a, b ). LA left atrium, RA right atrium, AO aorta
Case 86 Severe Valvular Pulmonary Stenosis and Patent Foramen Ovale
283
Diagnosis
The patient was diagnosed with severe valvular pulmonary
stenosis with a PFO.
Comment
The patient was referred for the valvuloplasty of the pulmonary valve.
Lesson
In valvular pulmonary stenosis due to a high right atrial pressure, there will be a shunt across the PFO from the right
atrium to the left atrium [ 116 ].
Fig. 86.6 There is poststenotic dilation of the pulmonary artery (up to
66 mm) on TEE (short-axis view). LA left atrium, RA right atrium, PA
pulmonary artery
ab
Fig. 86.5 The severe doming and thickening of the pulmonary valve ( arrow ) is evident on TEE (short-axis view) ( a ). The pulmonary annulus is
about 21 mm ( b ). LA left atrium, RA right atrium, RVOT right ventricular outfl ow tract, PA pulmonary artery, AO aorta
Case 86 Severe Valvular Pulmonary Stenosis and Patent Foramen Ovale
285
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_87, © Springer International Publishing Switzerland 2015
A 15-year-old girl presented with dyspnea on exertion
(functional class II) and cyanosis. Physical examination
revealed an ejection-type murmur at the pulmonic area.
The electrocardiogram showed right-axis deviation and right
ventricular hypertrophy.
Severe Valvular and Subvalvular
Pulmonary Stenosis
Case 87
Fig. 87.1 The parasternal long-axis view shows severe right ventricu-
lar free wall hypertrophy ( arrow ). 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_87 ) contains supplementary
material, which is available to authorized users.
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