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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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419
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_126, © Springer International Publishing Switzerland 2015
A 4-year-old boy was referred to our center for an evaluation
of cyanosis. Electrocardiography (ECG) showed a tall P wave,
and the axis of the frontal leads was superior.
Tricuspid Atresia
with Pulmonary Atresia
Case 126
Fig. 126.1 The modifi ed apical four-chamber view reveals the atresia
of the tricuspid valve ( arrow ). LA left atrium, RA right atrium
ab
Fig. 126.2 The atresia of the tricuspid valve is visualized in the apical
four-chamber view ( arrow ). Also, there is a large ventricular septal
defect ( curved arrow ) (VSD) in this view ( a , b ). The patient also had
the atresia of the main pulmonary artery and the pulmonary valve. The
right and left pulmonary arteries were about 11 mm. LA left atrium, LV
left ventricle, RA right atrium, RV right ventricle
420
Diagnosis
The patient was diagnosed with atresia of the tricuspid valve,
large muscular VSD, and atresia of the main pulmonary
artery and the pulmonary valve.
Comment
The patient was referred for a bidirectional Glenn shunt
(hemi-Fontan procedure).
Lesson
Tricuspid atresia is one of the rare congenital anomalies, the
diagnosis of which is aided by ECG. A tall P wave, in favor
of the right atrial enlargement, and the superior axis of the
frontal leads are the hallmarks of ECG diagnosis [ 2 , 142 ].
Case 126 Tricuspid Atresia with Pulmonary Atresia
421
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_127, © Springer International Publishing Switzerland 2015
An infant was referred to our center for an evaluation of
cyanosis.
Tricuspid Atresia with Inlet VSD
Case 127
Fig. 127.1 The parasternal long-axis view shows the continuity of the
mitral and aortic valves. LA left atrium, LV left ventricle, AO aorta
Fig. 127.2 The right ventricular infl ow view reveals the atresia of the
tricuspid valve ( arrow ) and an inlet ventricular septal defect (VSD). VSD
measures 10 mm. LA left atrium, RA right atrium, LV left ventricle
Electronic supplementary material The online version of this chapter
(doi:
10.1007/978-3-319-12934-1_127 ) contains supplementary material,
which is available to authorized users.
422
Diagnosis
The patient was diagnosed with tricuspid atresia, inlet VSD,
and elevated mean pulmonary arterial pressure.
Comment
The infant was referred for pulmonary arterial banding.
Lesson
1. An inlet VSD is usually accompanied by other cardiac
lesions.
2. Tricuspid atresia may be associated with an atretic or stenotic pulmonary valve or without it [ 142 ].
3. A bidirectional Glenn shunt is feasible when the mean
pulmonary arterial pressure is less than 15 mmHg [ 2 ].
ab
Fig. 127.4 The pulmonary artery is dilated with severe pulmonary
insuffi ciency ( arrow ) ( a ), and the mean pulmonary arterial pressure
is about 23 mmHg in the parasternal short-axis view. ( a , b ) Two-
dimensional echocardiography ( a ) and the continuous wave Doppler
study ( b ). PA pulmonary artery, RVOT right ventricular outfl ow tract,
AO aorta
ab
Fig. 127.3 The atresia of the tricuspid valve ( arrow ) and the inlet VSD
( curved arrow ) are also evident in the apical four-chamber view by two-
dimensional study ( a ) and the color Doppler fl ow study ( b ). No fl ow
passes from the tricuspid valve. The fl ow comes from the left ventricle
toward the right ventricle through the VSD ( curved arrow ) ( b ). LA left
atrium, LV left ventricle, RA right atrium, RV right ventricle
Case 127 Tricuspid Atresia with Inlet VSD
423
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_128, © Springer International Publishing Switzerland 2015
A 24-year-old man presented with palpitation.
Ebstein’s Anomaly with Severe
Tricuspid Regurgitation
Case 128
a
b
Fig. 128.1 The apical four-chamber view shows that the apical displacement of the septal leafl et of the tricuspid valve is about 17 mm without
signifi cant tethering ( a ). There is also severe tricuspid regurgitation ( 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_128 ) contains supplementary material,
which is available to authorized users.
424
Fig. 128.2 The three leafl ets of the tricuspid valve can be seen on
transgastric echocardiography (21°). S septal leafl et, P posterior leafl et,
A anterior leafl et, LV left ventricle
Diagnosis
The patient had Ebstein’s anomaly with severe tricuspid regurgitation and mild apical displacement of the septal leafl et of the
tricuspid valve. He was also diagnosed with mild right ventricular dilation and normal systolic function. Apart from palpitation,
there were no signs and symptoms of right-sided failure.
Comment
The patient was referred for Holter monitoring, medical
treatment, and follow-up.
Lesson
1. Supraventricular arrhythmia is a common fi nding in
Ebstein’s anomaly. If this arrhythmia is not controlled by
medical therapy or ablation, it becomes a relative indication for surgery [ 2 ].
2. In the transgastric view with a slightly increased degree,
the three leafl ets of the tricuspid valve can be visualized.
128 Ebstein’s Anomaly with Severe Tricuspid Regurgitation
425
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_129, © Springer International Publishing Switzerland 2015
A young pregnant woman, who was asymptomatic with a
very good functional class, was referred to us for further
evaluation. Physical examination revealed a holosystolic
murmur at the lower left sternal border, which increased with
inspiration, and the wide splitting of S1. The O
2
saturation
was 93 % in room air. She was referred for an evaluation of
the cardiac cause of the systolic murmur and consultation for
the continuation of pregnancy. Her body surface area was
1.6 m
. Echocardiography revealed a normal left ventricular
size and systolic function and the upper limit of the normal
right ventricular size with a normal right ventricular systolic
function.
Ebstein’s Anomaly
with Moderate Tricuspid
Regurgitation, Mild Septal
Displacement Ratio, and Grade 1
Right Atrial Cavity Ratio
Case 129
Fig. 129.1 The apical four-chamber view shows that the apical
displacement of the septal leafl et of the tricuspid valve is
approximately 18 mm (11.2 mm/m
2
), which is suggestive of
Ebstein’s anomaly. 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_129 ) contains supplementary material,
which is available to authorized users.
426
a
b
Fig. 129.3 This is a moderate tricuspid regurgitation with a gradient of about 36 mmHg. ( a , b ) Apical four-chamber view of the tricuspid valve
by the color-fl ow and continuous wave studies
a
c
b
Fig. 129.2 The apical four-chamber view demonstrates mild right
atrial dilation (right atrial area of 24.5 cm
2
) ( a ), right atrial cavity ratio
of 0.43 (24.5/16.8 + 26.2 + 14.2 = 24.5/57.9 = 0.43) ( a , b ) of grade 1, and
a septal displacement ratio smaller than 0.45 (96−78/96 = 18 %) ( c ), all
of which are in favor of the mild type of the Ebstein’s deformity of the
tricuspid valve
Case 129 Ebstein’s Anomaly with Moderate Tricuspid Regurgitation
427
Diagnosis
The patient was diagnosed with a mild form of Ebstein’s
anomaly with moderate tricuspid regurgitation.
Comment
Because of the patient’s good functional class, absence of
cyanosis, and normal right ventricular systolic function,
continuation of pregnancy was recommended [ 7 ]. Although
there is the risk of right ventricular failure, arrhythmia, and
paradoxical emboli, the probability of the involvement of
offspring is 6 %. Ebstein’s anomaly is more probable if the
mother uses benzodiazepines and lithium carbonate during
pregnancy [ 7 ].
Case 129 Ebstein’s Anomaly with Moderate Tricuspid Regurgitation
429
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_130, © Springer International Publishing Switzerland 2015
A 22-year-old woman with a history of dyspnea on exertion
(functional class I) was referred to us. Physical examination
showed a holosystolic murmur (grade II/VI) at the left sternal
border. Echocardiography revealed mild left ventricular systolic dysfunction (left ventricular ejection fraction of 50 %)
with moderate right ventricular dilation.
Ebstein’s Anomaly with Severe
Tricuspid Regurgitation
Case 130
Fig. 130.1 The apical four-chamber view demonstrates that
the apical displacement of the septal leafl et of the tricuspid valve
is approximately 20 mm. 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_130 ) contains supplementary material,
which is available to authorized users.
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