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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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232
a
c
b
Fig. 73.5 The thickening and doming of the pulmonary valve ( arrow )
( a ), subpulmonic narrowing of the right ventricular outfl ow tract ( b ),
and aliasing fl ow in the subpulmonic area ( c ) are suggestive of valvular
and subvalvular pulmonary stenosis by short-axis view. LA left atrium,
AO aorta, PA pulmonary artery
Fig. 73.6 The peak gradient across the pulmonary valve is approxi-
mately 62 mmHg in the short-axis view
Fig. 73.7 The left pulmonary artery is about 13 mm and the right pul-
monary artery is approximately 18 mm in diameter. The Nakata index
is 227 mm
2
/m 2 . The Nakata index: right pulmonary artery surface
(0.785 × 18 × 18) + left pulmonary artery surface (0.785 × 13 × 13)/body
surface area (1.7) = 227
Case 73 Tetralogy of Fallot with an Additional VSD, Good Nakata Index, and Abnormal Course of the Coronary Artery
233
There is no main aortopulmonary collateral artery in the
suprasternal long-axis view, and the pulmonary artery annulus diameter is about 19 mm.
Diagnosis
The patient was diagnosed with tetralogy of Fallot, in conjunction with an additional VSD, good Nakata index, and
abnormal course of the coronary arteries.
Comment
The patient was referred for angiography because of the
abnormal course of the coronary artery on echocardiography.
Because the Nakata index was greater than 200 mm/m 2 , it
seemed that this patient was a good candidate for surgery
(total correction).
Lesson
1. The Nakata index is a crucial index for decision-making
about the type of operation in the tetralogy of Fallot. If the
index is greater than 150 mm 2 /m 2 , the patient can be referred
for total correction. If the index is less than 100 mm 2 /m 2 , the
patient should be a candidate for a modifi ed Blalock–
Taussig shunt so as to confer time and chance to the pulmo-
nary vascular bed to develop. A Nakata index between 100
and 150 mm 2 /m 2 is a borderline case [ 102 , 103 ].
2. If a patient with the tetralogy of Fallot cannot be weaned
off the pump during surgery, there are two possible diag-
noses: (a) an undiagnosed additional VSD and (b) an
abnormal course of the coronary artery. If the pressures of
both ventricles are almost equal, the fl ow through the
VSD may not be visualized and an exact echocardio-
graphic examination via two-dimensional echocardiogra-
phy is mandatory.
3. If the pulmonary annulus is too small (≤2 standard deviation from the normal or Z score), a transannular patch
should be done and so the pulmonary valve will be
damaged.
4. The major dangerous point of an abnormal course of the
coronary artery occurs when it is associated with a
small pulmonary annulus, necessitating a transannular
patch. During a transannular patch, the risk of rupture
of the coronary artery is too high when its course is
abnormal.
5. If there is any suspicion of the abnormal course of the
coronary artery on echocardiography, coronary angiography is mandatory.
6. Branch pulmonary stenosis is a common fi nding, especially in the left pulmonary artery [ 103 ]. The probable
explanation is that when there is a narrowing of the pulmonary vascular bed during the fetal circulation, the fl ow
passes from the ductus arteriosus to the pulmonary trunk
and some cells immigrate toward the pulmonary artery
and branches and produce stenosis. Regarding the leftward orientation of the duct, the left pulmonary artery is
more prone to stenosis, as was the case in our patient.
This is the antithesis of the mechanism that is explained
for coarctation when there is a left-sided reduction in the
fl ow in the fetus and there is an augmented fl ow from the
ductus arteriosus toward the descending aorta. In such
circumstances, the possibility of the coarctation of the
aorta is increased.
7. The abnormalities of the coronary arteries occur in 5 % of
the cases of tetralogy of Fallot. Usually, the left anterior
descending artery originates from the right coronary
artery and courses anteriorly to cross the infundibulum, as
was the case in our patient [ 2 ].
Case 73 Tetralogy of Fallot with an Additional VSD, Good Nakata Index, and Abnormal Course of the Coronary Artery
235
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_74, © Springer International Publishing Switzerland 2015
A 23-year-old man presented with dyspnea on exertion
(functional class III), hemoptysis, and cyanosis. Physical
examination revealed severe cyanosis (O 2 saturation of
76 % in air room) as well as a systolic ejection murmur in
the pulmonic area at the left sternal border. He had undergone surgery at childhood for a shunt. The electrocardiogram showed right-axis deviation and right ventricular
hypertrophy.
Tetralogy of Fallot, Small Pulmonary
Annulus, and Low Nakata Index
Case 74
a
b
Fig. 74.1 The parasternal long-axis view demonstrates the overriding
of the aorta and the hypertrophy of the right ventricle as well as a
21-mm ventricular septal defect (VSD) (malalignment type) ( a ) and a
bidirectional fl ow crosswise ( arrow ) ( b ). LA left atrium, LV left ventri-
cle, RV right ventricle, AO aorta
Electronic supplementary material The online version of this
chapter (doi:
10.1007/978-3-319-12934-1_74 ) contains supplementary
material, which is available to authorized users.
236
Fig. 74.4 The suprasternal long-axis view demonstrates no main aor-
topulmonary collateral artery. DAO descending aorta
a
b
c
Fig. 74.2 The parasternal short-axis view demonstrates severe valvu-
lar and subvalvular pulmonary stenosis (peak gradient of 80 mmHg)
( a ). The pulmonary valve annulus is 12 mm ( b ), main pulmonary artery
is 11 mm, right pulmonary artery is 8 mm, and left pulmonary artery is
9 mm ( c ). RVOT right ventricular outfl ow tract, PA pulmonary artery,
AO aorta
Fig. 74.3 The apical four-chamber view shows the VSD ( arrow ).
There is no additional VSD, and there is a moderate right ventricular
dilation. LA left atrium, LV left ventricle, RA right atrium, RV right
ventricle
Case 74 Tetralogy of Fallot, Small Pulmonary Annulus, and Low Nakata Index
237
Diagnosis
The patient was diagnosed with tetralogy of Fallot with a
small pulmonary annulus and a low Nakata index (46 mm 2 /
m
2
). There was no main aortopulmonary collateral artery and
no evidence of shunt of previous operation.
Comment
A Blalock–Taussig shunt was recommended [ 102 , 103 ].
Case 74 Tetralogy of Fallot, Small Pulmonary Annulus, and Low Nakata Index
239
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_75, © Springer International Publishing Switzerland 2015
A 17-year-old female presented with dyspnea on exertion
(functional class II) since childhood. Physical examination
showed cyanosis and clubbing. Oxygen saturation was 80 %
in air room. The electrocardiogram revealed right-axis deviation and a tall R in lead V1.
Tetralogy of Fallot and
Pulmonary Atresia
Case 75
a
b
Fig. 75.1 The parasternal long-axis view shows the overriding of the
aorta, malalignment of the septum, hypertrophy of the right ventricular
free wall ( curved arrow ), and a perimembranous ventricular septal
defect ( VSD ) ( arrow ) ( a ). This is a schematic illustration of these
lesions ( b ). AO aorta, RV right ventricle, LV left ventricle
Electronic supplementary material The online version of this
chapter (doi:
10.1007/978-3-319-12934-1_75 ) contains supplementary
material, which is available to authorized users.
240
a
b
c
Fig. 75.2 The atresia of the pulmonary valve and the main pulmonary
artery is visualized in the parasternal short-axis view by two-dimensional
echocardiography ( a ) and the color-fl ow study ( arrows ) ( b ). This is a
schematic depiction of the atresia of the pulmonary valve and the main
pulmonary artery ( c ). RA right atrium, AO aorta, RVOT right ventricular
outfl ow tract, PA pulmonary artery
a
b
Fig. 75.3 The apical four-chamber view demonstrates the VSD ( arrow ) ( a ). A schematic illustration of the VSD is presented ( b ). LA left atrium,
LV left ventricle, RA right atrium, RV right ventricle
Case 75 Tetralogy of Fallot and Pulmonary Atresia
241
d
a
b
c
Fig. 75.4 The longitudinal view of the arch shows the descending
aorta as well as the main aortopulmonary collateral artery, which arises
from it ( arrow ) ( a ). Also, an antegrade deviation of the probe reveals
the left pulmonary artery, right pulmonary artery, and main aortopulmonary collateral artery toward them by two-dimensional echocardiogra-
phy ( b ) and the color-fl ow study ( c ). This is a schematic presentation of
the main aortopulmonary collateral artery, right pulmonary artery, left
pulmonary artery, and descending aorta ( d ). LPA left pulmonary artery,
RPA right pulmonary artery, DAO descending aorta, MAPCA major
aortopulmonary collateral arteries
Case 75 Tetralogy of Fallot and Pulmonary Atresia
242
a
b
c
Fig. 75.5 The suprasternal long-axis view shows the systolodiastolic
fl ow from the descending aorta through the main aortopulmonary collateral artery toward the left and right pulmonary arteries: systolic ( a )
and diastolic ( b ). Also, continuous wave Doppler demonstrates the sys-
tolic and diastolic components of the main aortopulmonary collateral
artery fl ow ( c ). The reversal fl ow in the descending aorta is a marker for
MAPCA ( arrowhead ). DAO descending aorta
Fig. 75.6 The suprasternal long-axis view reveals the origin of the
main aortopulmonary collateral artery from the descending aorta (5 mm
in size). DAO descending aorta
Case 75 Tetralogy of Fallot and Pulmonary Atresia
243
a
b
c
Fig. 75.7 Transesophageal echocardiography (TEE) (long-axis view) shows the VSD and the overriding of the aorta ( arrow ) ( a ) as well as mild
aortic insuffi ciency ( b ). The VSD is 24 mm in size ( c ). LA left atrium, LV left ventricle, RV right ventricle, AO aorta
a
b
Fig. 75.8 The abnormal course of the left coronary artery (between the ascending aorta and the left atrium) is evident on TEE (short-axis view)
( arrows ) ( a , b ). LA left atrium, RA right atrium, AO aorta
Case 75 Tetralogy of Fallot and Pulmonary Atresia
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