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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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407
Diagnosis
The patient was diagnosed with dextrocardia, situs inversus,
ventricular inversion, transposition of the great arteries, aorta in
anterior location to the pulmonary artery, subpulmonic VSD,
valvular and subvalvular pulmonary stenosis, moderately
severe atrioventricular valve regurgitation, and a mobile mass
on the pulmonary valve (suggestive of infective endocarditis).
Comment
Work-up for infective endocarditis, followed by catheterism
and surgery, was recommended for the patient.
Lesson
In this complex congenital lesion, the most important issue
is that desaturated blood from the right atrium enters the
left ventricle and then pulmonary artery. Indeed, there is a
ventricular inversion (corrected transposition of the great
arteries) in association with VSD and valvular and subvalvular pulmonary stenosis. These associated anomalies produce cyanosis. As regards surgery, one possible method is
to close the VSD and to use a conduit between the morphological left ventricle and the pulmonary artery.
Nevertheless, this strategy is associated with the possibility of the persistence of the symptoms and signs of heart
failure due to systemic atrioventricular valve regurgitation
and systemic ventricular failure. (The morphological right
ventricle is the systemic ventricle.) Another possible strategy for surgery is double switch (a venous switch such as
Mustard or Senning) or ventricular switch (Rastelli operation) [ 113 ].
Fig. 122.15 There is a membrane below the pulmonary valve, which
produces severe subvalvular pulmonary stenosis. LA left atrium, RV
right ventricle, LV left ventricle, PA pulmonary artery
Fig. 122.16 There is a mobile mass on the pulmonary valve, which is
in favor of vegetation on TEE (0°). LA left atrium, AO aorta, PV pul-
monary valve
Case 122 Situs Inversus, Dextrocardia, Corrected Transposition of Great Arteries
409
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_123, © Springer International Publishing Switzerland 2015
A 16-year-old female referred to our center for the evalua-
tion of cyanosis. She was a known case of congenital heart
disease from childhood. She had easy fatigability and dyspnea on exertion (functional class II). Physical examination
revealed an ejection systolic murmur at the left sternal border
and apex with maximal intensity in the pulmonic area.
Electrocardiography showed normal sinus rhythm and normal axis deviation with no evidence of right ventricular
hypertrophy. Chest X-ray demonstrated an increased cardiothoracic ratio and dilated pulmonary artery branches.
Single Ventricle, Malposition
of Great Arteries, and Valvular
and Subvalvular Pulmonary
Stenosis
Case 123
ab
Fig. 123.1 A single ventricle and the absence of the interventricular septum are evident in the parasternal long-axis infl ow view ( a ) and the apical
four-chamber view ( b ). LA left atrium, RA right atrium, SV single ventricle
Electronic supplementary material The online version of this chapter
(doi:
10.1007/978-3-319-12934-1_123 ) contains supplementary material,
which is available to authorized users.
410
a
b
Fig. 123.2 There is a subvalvular thickening ( arrow ) below the great
artery ( a ) which is related to the left atrium ( a ). The great artery is
located posteriorly and is divided ( arrow ) ( b ), and it is, therefore, the
pulmonary artery. ( a ) Parasternal long-axis view and ( b ) parasternal
short-axis view. LA left atrium, PA pulmonary artery, SV single
ventricle
a
c
b
d
Fig. 123.3 There is turbulency ( arrow ) in the pulmonary artery in the
parasternal long-axis ( a ) and parasternal short-axis ( b ) views. LA left
atrium, PA pulmonary artery, SV single ventricle. The peak gradient
across the pulmonary valve is 60 mmHg ( c ), and the subpulmonic
component of this gradient is about 37 mmHg. The size of the right and
left pulmonary arteries is within the normal limits ( d ). RPA right pul-
monary artery, LPA left pulmonary artery. PA pulmonary artery, LA left
atrium, SV single ventricle
Case 123 Single Ventricle, Malposition of Great Arteries, and Valvular and Subvalvular Pulmonary Stenosis
411
a
c
b
Fig. 123.4 The parasternal long-axis infl ow view shows that there is
moderate tricuspid regurgitation ( arrow ) ( a ). The vena contracta of the
tricuspid regurgitation is about 4 mm ( b ). The surface of the tricuspid
regurgitation is about 6.6 cm
2
( c ). LA left atrium, RA right atrium,
SV single ventricle
Fig. 123.5 There is a single ventricle in the parasternal short-axis
view. SV single ventricle
Case 123 Single Ventricle, Malposition of Great Arteries, and Valvular and Subvalvular Pulmonary Stenosis
412
Diagnosis
The patient had a single ventricle with two atrioventricular
valves, malposition of the great arteries, and valvular and
subvalvular pulmonic stenosis.
Comment
The patient was referred for cardiac catheterization. The surgical strategy for the single ventricle is to use the Fontan
operation. First, a bidirectional Glenn (hemi-Fontan) is per-
formed. Shortly thereafter, an extracardiac conduit is utilized
to perform complete Fontan operation.
Lesson
For a bidirectional Glenn shunt, the mean pulmonary arterial
pressure should be less than 15 mmHg, and the pulmonary
vascular bed should have an adequate size (e.g., Nakata
index >100 m 2 /m 2 ). If Glenn is not possible, a Blalock–
Taussig shunt is recommended [ 102 , 141 ].
ab
Fig. 123.6 The apical four-chamber view shows moderate tricuspid regurgitation ( arrow ) ( a ). The tricuspid regurgitation gradient is about
113 mmHg ( b ). LA left atrium, RA right atrium, SV single ventricle
Case 123 Single Ventricle, Malposition of Great Arteries, and Valvular and Subvalvular Pulmonary Stenosis
413
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_124, © Springer International Publishing Switzerland 2015
A 20-year-old man with a history of a single ventricle and
Fontan operation was referred to our echocardiography laboratory for the evaluation of the extracardiac conduit function and Glenn shunt.
Fontan Operation in a Patient
with Single Ventricle
Case 124
ab
Fig. 124.2 The color fl ow Doppler study of the previous view shows a laminar fl ow in the conduit ( a ) and an acceleration fl ow with inspiration
within ( b ) indicative of the good function of the extracardiac conduit. RV right ventricle
Electronic supplementary material The online version of this chapter
(doi:
10.1007/978-3-319-12934-1_124 ) contains supplementary material,
which is available to authorized users.
Fig. 124.1 The subcostal modifi ed four-chamber view reveals a tunnel-
shaped density behind the right ventricle, suggestive of an extracardiac
Fontan conduit without thrombus formation in it. RV right ventricle
414
ab
Fig. 124.3 The suprasternal short-axis view depicts the connection of
the superior vena cava to the right pulmonary artery (Glenn shunt) and
a laminar fl ow within ( a ) as well as a continuous low pressure Doppler
fl ow in the Glenn shunt, in favor of the good function of this shunt.
RPA right pulmonary artery, SVC superior vena cava
Diagnosis
The patient, post-Fontan operation, had no obstruction in the
extracardiac conduit and good function of Glenn shunt.
Case 124 Fontan Operation in a Patient with Single Ventricle
415
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_125, © Springer International Publishing Switzerland 2015
A 13-year-old girl with a history of an extracardiac conduit
and a total cavopulmonary connection was referred to our
echocardiography laboratory for the evaluation of the extracardiac conduit and Glenn shunt.
Fontan Operation
Case 125
Fig. 125.1 The subcostal visceral view shows that the inferior vena
cava is interrupted ( arrow ) by the connection to the extracardiac con-
duit. IVC inferior vena cava
Electronic supplementary material The online version of this chapter
(doi:
10.1007/978-3-319-12934-1_125 ) contains supplementary material,
which is available to authorized users.
416
a
b
c
Fig. 125.2 The subcostal visceral view reveals that the inferior vena
cava is connected to the conduit ( c ). The arrow points to the suture line
( a ). The color-fl ow Doppler study demonstrates a laminar fl ow ( blue
color ) in the inferior vena cava and the extracardiac conduit ( b ). This
schematic illustration of the subcostal visceral view demonstrates
that the inferior vena cava is interrupted at the right atrium and is connected to the extracardiac conduit. RA right atrium, RV right ventricle,
IVC inferior vena cava, C conduit, HV hepatic vein
Fig. 125.3 The peak gradient across the extracardiac conduit is
3 mmHg
Case 125 Fontan Operation
417
Diagnosis
The patient, post-Fontan operation, had no obstruction in
the extracardiac conduit and a good function of the Glenn
shunt.
Fontan Operation
The Fontan operation was fi rst used for tricuspid atresia and
consists of a cavopulmonary connection. In the fi rst stage,
the fl ow of the superior vena cava is diverted toward the right
pulmonary artery near the bifurcation, so that the superior
vena cava drains about half of the cardiac output into the
pulmonary system (this amount is more than half of the cardiac output in children and less than half of the cardiac output in adults) [ 2 ]. In the second stage, the fl ow of the inferior
vena cava is diverted toward the pulmonary system via an
extracardiac conduit. One of the major complications of
these procedures is thrombosis or obstruction, which manifests as plethora and edema. By echocardiographic evaluation, a peak gradient below 3 mmHg with a nonturbulent
fl ow in the Glenn shunt or an extracardiac conduit is considered a normal value. Fontan operation is used for single ventricle or other complex cardiac lesions that are equivoval to
single ventricle [ 108 ].
Fig. 125.5 This illustration of a Fontan heart shows the stages of the
Fontan operation: ( 1 ) ligation of the main pulmonary artery, ( 2 ) ligation
of the superior vena cava, ( 3 ) connection of the superior vena cava to
the right pulmonary artery, ( 4 ) connection of the inferior vena cava to
the extracardiac conduit ( gray conduit ), and connection of the extracar-
diac conduit to the right pulmonary artery. Stages 1–3 are named Glenn
or hemi-Fontan. RV right ventricle, LV left ventricle, RA right atrium, LA
left atrium, SVC superior vena cava, PA pulmonary artery, AO aorta, IVC
inferior vena cava
a
b
Fig. 125.4 The suprasternal short-axis view depicts a laminar blue
color fl ow near the ascending aorta, which drains into the right pulmo-
nary artery: this indicates the connection between the superior vena
cava and the right pulmonary artery (Glenn shunt or hemi-Fontan) ( a )
and the continuous venous fl ow within with a 2.7-mmHg gradient ( b ).
SVC superior vena cava, AO aorta, RPA right pulmonary artery
Case 125 Fontan Operation
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