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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5774_Библиотеки_им_академика_М_И_Перельмана.pdf
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Q. Zhu et al.
resulting in hypoplasia of the RV.TA is often
accompanied by other cardiovascular malformations, accounting for 1.4% of congenital
heart disease.
TA is divided into muscular atresia, membranous atresia, and valvar atresia. Muscular atresia is the most common.
The VSD is the only entrance of the RV
blood.
– Ultrasonic manifestation
The four-chamber view and the short axis of
the great artery show the complete absence of
the tricuspid valve. Thickened tissue at the
atrioventricular annulus separates the RA from
the RV, without movement. The LV is enlarged,
while the RV is small (Fig.4.25).
TA with a VSD is more frequently found.
Sometimes, ASD or patent foramen ovale can
be detected after birth.
Color Doppler ultrasound shows no blood ow
through the tricuspid valve. Blood travels from
right to left at the atrial level and left to right at
the ventricular level.
– Tips
The detection rate of TA is high prenatally.
TA often accompanies other cardiac defects.
Prognosis: TA cases with normal great arteries
arrangement have a relatively good prognosis,
with a 15-year survival rate of 65–70%. The survival rate reduces in TA cases accompanied by
transposition of great arteries or other
malformations.
Fig. 4.24 Ebstein’s anomaly. (a) The fetal four-chamber view shows the inferiorly displaced anterior leaet of the tricuspid valve. (b) The fetal
four-chamber view shows tricuspid regurgitation
Fig. 4.25 Tricuspid atresia. (a) Fetal four-chamber view shows tricus-
pid atresia in diastole and right ventricular hypoplasia. (b) The fourchamber view of the same fetus shows nonobstructive blood ow of the
mitral valve in diastole and right ventricular hypoplasia. No blood ow
through the tricuspid valve is detected
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4 Ultrasonic Diagnosis ofFetal Heart
205
4. Conotruncal defects
• Tetralogy of Fallot
– Basic concepts
Tetralogy of Fallot (TOF) is the most common
cyanotic CHD, accounting for 10% of postnatal
CHD.
TOF consists of pulmonary stenosis (including
subvalvular type, valvular type, and supravalvular type), malalignment-type VSD, overriding aorta, and hypertrophy of the RV. The
hypertrophy of the RV is not obvious in the
fetal period.
The spatial position of the two major arteries is
normal in TOF cases. The pulmonary artery
wraps the aorta.
– Pathological anatomical classication
Atypical TOF consists of pulmonary valve stenosis (mild or moderate), VSD, aortic overriding (mild), right ventricular hypertrophy (mild).
Cyanosis is not obvious after birth.
Typical TOF: pulmonary stenosis, VSD, aortic
overriding, right ventricular hypertrophy (not
obvious in the fetal period). Cyanosis is obvious after birth.
Severe TOF (pseudo-truncus arteriosus): pulmonary atresia or severe infundibular hypoplasia, VSD, aortic overriding, right ventricular
hypertrophy (not obvious in the fetal period).
Some cases often have concomitant Patent ductus arteriosus and multiple aortopulmonary collateral arteries. Cyanosis is obvious after birth.
– Ultrasonic manifestation
The LVOT and the ve-chamber view show
VSD, overriding aorta with increased inner
diameter. But no obvious thickened right ventricular wall is seen in the fetal period
(Fig.4.26).
The long axis of RVOT, the short axis of the
great artery, and the three-vessel view show the
narrowed infundibular, narrowed pulmonary
annulus and valve, narrowed pulmonary artery
and branches, etc. The diameter of the aorta
increases, which is out of proportion to that of
the pulmonary artery. The inner diameter of the
pulmonary artery is narrowed. The ratio of PA/
AO is less than 1.
Color Doppler ultrasound shows that biventricular blood ows into the aorta. Turbulence or
ne blood ow can be seen at the stenosis of
the right infundibular and valve.
– Tips
The intrauterine detection rate is relatively
high.
Double outlet of right ventricle should be considered when the rate of aorta straddling is
greater than 50%.
The conical structure between the semilunar
valve and the atrioventricular valve of the
double- outlet right ventricle cases is usually
visible after birth, which is usually invisible in
the fetus.
In TOF, the spatial position of the two major
arteries is normal, while is arranged in parallel
in the double-outlet right ventricle cases.
Fig. 4.26 Tetralogy of Fallot. (a) The long-axis view of left ventricle shows VSD and the aorta straddling across the ventricular septum. (b) The
short axis of the great artery shows the pulmonary artery with a decreased inner diameter and an enlarged aorta. The ratio of PA/AO is less than 1
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There is no signicant right ventricular hypertrophy in the fetal period.
The prognosis is poor in cases of severe pulmonary artery stenosis or combined with extracardiac malformations (including chromosomal
abnormalities).
• Double-outlet right ventricle
– Concepts
Double-outlet right ventricle (DORV) is
dened as all or most of two major arteries
arise from the RV, accounting for 5% of the
postnatal CHD. On the contrary, it is called
double-outlet left ventricle if all or most of two
major arteries arise from the LV, which is one
of cyanotic CHD.
VSD is the only outlet of the left ventricle.
The conical structure under the semilunar valve
is visible after birth, which is usually invisible
in the fetus.
– Ultrasonic manifestation
The long axis of the LVOT and the short axis of
the great arteries show that the two great arteries arise from the RV in parallel (Fig.4.27).
VSD is visible in DORV cases.
Color Doppler ultrasonography shows two parallel color blood ows from the RV and blood
shunt at the ventricular level.
– Tips
The diagnosis of TOF should be considered
when the rate of aorta straddling is greater than
50%, accompanied by pulmonary artery stenosis. The spatial position of the two major arteries is abnormal in DORV cases.
When the rate of pulmonary artery straddling is
less than 50%, transposition of the great arter-
ies should be considered. Use the segmental
analysis to determine the position of atrium, the
position of ventricles and great arteries, the
connection between atrium and ventricle, the
relationship between ventricle and great arteries. According to the above analysis, identify
whether the abnormity is DORV or transposition of major arteries.
The subaortic conical structure is not obvious
in fetus.
The prognosis is poor.
• Truncus arteriosus
– Concepts
Truncus arteriosus is characterized by a single
great artery arising from the base of the heart,
which supplies the systemic circulations, pulmonary circulations, and coronary arteries,
accounting for 2% of the postnatal CHD.
There is only one set of semilunar valves in the
truncus arteriosus, which can consist of two
valves, three valves, four valves and more.
There are four types of truncus arteriosus. Type
I: the pulmonary artery, arising from the posterolateral ascending portion of the common
truncal artery, then divides into the left and right
branches. Type II: the left and right pulmonary
arteries arise directly from the posterior or lateral truncus. Type III: one pulmonary artery
directly arises from the truncus, while the other
artery is absent (usually left pulmonary artery).
Type IV: the pulmonary artery arises from the
descending thoracic aorta or is absent.
– Ultrasonic manifestation
The large truncal artery overriding the ventricular septum is distinguished in the long axis of
Fig. 4.27 Double-outlet right ventricle. (a) A nonstandard view of fetal heart shows that the pulmonary artery and the aorta arise from the RV. (b)
A nonstandard view of the fetal heart shows that the color Doppler ow imaging of the two major arteries arises from the RV
4 Ultrasonic Diagnosis ofFetal Heart
207
the LVOT. The inner diameter of the truncal
artery is signicantly increased, forming a single outlet of both ventricles. The interruption
between ventricular septum and the anterior
truncal artery is visible. The anterior wall of the
RV connects the anterior wall of the truncal
artery (Fig.4.28).
The short axis of the great artery shows “single-
• Transposition of great arteries
loop sign” and a set of semilunar valves, without normal RVOT and pulmonary artery.
There are two vessels in three-vessel view, one
is the truncal artery, and the other is the superior vena cava. Sometimes only one enlarged
artery is visible.
Color Doppler ultrasound shows that the blood
from the left and right ventricular converges
and ows into the great artery during the systolic period. Subvalvular regurgitation can be
detected (Fig.4.28).
– Tips
a
b
Only one great artery is visible in several views.
The absence of RVOT indicates truncus
arteriosus.
Truncus arteriosus is diagnosed if four or more
semilunar valves are observed in the great
artery.
Truncus arteriosus has a poor prognosis.
– Concepts
Transposition of great arteries (TGA) is a cyanotic CHD, which is often associated with
other cardiac anomalies, accounting for 7–9%
of the postnatal CHD.
TGA is divided into two types: Dextro-TGA
(d-TGA) and congenitally corrected TGA
(CCTGA). In both types, ventricular arterial
discordance is visible.
d-TGA is characterized by ventricular arterial
discordance with atrioventricular concordance,
no matter the atrium is in situs solitus or situs
c
Fig. 4.28 Truncus arteriosus. (a) A nonstandard view of the fetal heart
shows that a large artery arises from both ventricles. The anterior wall
of the RV connects the anterior wall of truncal artery. (b) A nonstandard
view of the fetal heart shows that the color ow of a large artery arises
from both ventricles; (c) Three-vessel view shows the truncal artery and
the superior vena cava
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inversus. The pulmonary artery arises from the
LV, and the LV connects with the LA.The aorta
arises from the RV, and the RV connects with
the RA.
CCTGA is characterized by both ventricular
arterial discordance and atrioventricular discordance, regardless of the orientation of the
atrium, the ventricles, and the great arteries.
The pulmonary artery arises from the LV, and
the LV connects with the RA.The aorta arises
from the RV, and the RV connects with the
LA. There are no hemodynamic changes in
oxygenation, although the orientation of the
blood ow through atrioventricular and great
arteries is abnormal.
– Ultrasonic manifestation
The short axis of the great artery shows the
double-ring sign of two great arteries, with the
absence of the pulmonary artery crossing the
aorta.
In the long axis of LVOT and RVOT, two large
arteries are parallel to each other. The pulmonary artery connects with the LV, and the aorta
connects with the RV. Identify the pulmonary
artery according to the fact that the great artery
divides into left and right branches. The arcuate
large artery connecting with the brachiocephalic artery is identied as the aorta.
The four-chamber view shows atrioventricular
concordance in d-TGA and atrioventricular
discordance in CCTGA (Fig.4.29). Identify the
LA and RA according to the direction of opening of foramen ovale valve and the connection
of the pulmonary vein, vena cava, and atrium.
Determine the LV and RV according to the site
of attachment of the atrioventricular valve.
– Tips
The heart structure is in a state of disorder in
TGA cases. Use the segmental analysis to tell
the following aspects sequentially: the position
of atrium, ventricle, and great arteries, the connection between atrium and ventricle, the relationship between ventricle and great arteries.
If the rate of pulmonary artery straddling is
greater than 50%, a special type of DORV
should be considered, known as Taussig–Bing
syndrome.
The spatial relationship of the two great arteries
is abnormal.
The prognosis is poor.
Other congenital heart malformations.
• Single atrium
– Concepts
The single atrium is characterized by a common atrial cavity without atrial septum.
The single atrium is a rare cyanotic CHD.
– Ultrasonic manifestation
No atrial septum is visible in the four-chamber
view and the short axis of the aorta. (Fig.4.30).
Color Doppler ultrasound shows that the blood
of vena cava and pulmonary vein mix in the
common atrium.
– Tips
The intrauterine detection rate of the single
atrium is high. Please pay attention to other
accompanied cardiac malformations.
The prognosis of the single atrium is poor.
• Single ventricle
Fig. 4.29 Transposition of great arteries. (a, b) A nonstandard view of the long axis of the LV shows 2-D and color Doppler ow imaging shows
ventricular arterial discordance with atrioventricular concordance, indicating d-TGA
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4 Ultrasonic Diagnosis ofFetal Heart
Fig. 4.30 Single atrium. (a, b) 2-D shows the absence of atrial septum in the atrial cavity
209
– Concepts
The ventricular septum is completely absent or
extremely dysplasia.
Anatomic single ventricle: only one ventricular
chamber, with one or two sets of atrioventricular valves connected with the ventricle
abnormally.
“Functional” single ventricle: there are two
anatomic ventricles, and one of them is
extremely small.
It is a rare cyanotic CHD, accounting for 1% of
postnatal CHD.
It is often accompanied by ventricular arterial
discordance and abnormal spatial
relationships.
– Ultrasonic manifestation
No ventricular septum is found. The fourchamber view shows only one common ventricle, with the absence of ventricular septum,
communicating with the atrioventricular valves
(Fig.4.31).
Color Doppler ultrasonography shows the atrial
blood ows into the common ventricle from the
left and right atrioventricular valves.
– Tips
5. Cardiac tumors
• Concepts
The intrauterine detection rate is high. Single
ventricle is often accompanied by ventricular
arterial discordance, atrioventricular discordance, and other cardiac malformations.
The prognosis of single ventricle is extremely
poor.
• Anomalous pulmonary venous connection
– Concepts
When an anomalous pulmonary venous connection is present, none or some of the pulmonary veins return to the LA. The pulmonary
veins connect with the RA directly or indirectly
by other paths.
It can be divided into partial anomalous pulmonary venous connection (PAPVC), which is
common, and total anomalous pulmonary
venous connection (TAPVC). According to the
pulmonary vein connection, both two types can
be divided into intracardiac type, supracardiac
type, and infracardiac type.
– Ultrasonic manifestation
Four-chamber view and nonstandard view
shows none of the pulmonary veins drains into
the left atrium.
Usually, common vena cava can be detected in
the lateral and posterior walls of LA (Fig.4.32).
The left atrium shrinks.
– Tips
The antenatal sonographic diagnosis of anomalous pulmonary venous connection is difcult.
We should be cautious to improve the detection
rate.
Pay attention to other concomitant cardiac
anomalies.
– The fetal cardiac tumor is the abnormal mass in the
heart, usually arising from the ventricular wall.
The majority of cardiac tumors are benign tumors,
and rhabdomyomas are the most frequently
detected.
– Rhabdomyomas account for 60% of cardiac
tumors. It can be multiple or isolated. Larger
tumors may result in hemodynamic disorders,
leading to hydrops and fetal demise.
– Other rare cardiac tumors include teratoma,
broma, lipoma, hemangioma, and myxoma, etc.
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Q. Zhu et al.
a
b
c
Fig. 4.31 Single ventricle. (a) The fetal four-chamber view shows the
complete absence of ventricular septum—two sets of atrioventricular
valves open to the common ventricle. (b) The four-chamber view shows
• Ultrasonic manifestation
– In the four-chamber view, rhabdomyomas appear
as well-circumscribed, hyperechogenic masses.
– Rhabdomyomas, multiple or single, may protrude
into the heart cavity or locate in the ventricular
wall mostly in the ventricular septum.
Rhabdomyomas move with the systolic and diastolic cardiac movement (Fig.4.33).
– We should be cautious because of the various
sonographic features in different cardiac
tumors.
– Color Doppler ultrasonography shows the blood
ows between the tumor and the chamber’s wall.
In the cases with obstruction, accelerated blood
ow will be detected.
• Tips
– The intrauterine detection rate is high.
– It is difcult to make a pathological diagnosis of
cardiac tumors.
– Once space-occupying lesions are detected, close
observation should be followed. Some cardiac
a complete absence of the atrial septum and ventricular septum, indicating bilocular heart (single atrium and single ventricle). (c) Color blood
ow imaging of the bilocular heart of the same fetus
rhabdomyomas have been reported to shrink or
completely regressed later.
– Terminate the pregnancy if inow or outow tract
obstruction is obvious.
6. Summary
• Some CHDs are easy to be diagnosed by prenatal
ultrasonic examination as follows: large VSD, ostium
primum ASD, AVSD, single atrium, single ventricle,
mitral valve atresia, tricuspid valve atresia, severe
Ebstein’s anomaly, left or right cardiac hypoplasia
syndrome, etc.
• Some CHDs are easy to be missed by prenatal ultrasonic examination as follows: high VSD, VSD that less
than 3 mm, sinus venosus ASD, fenestrated ASD,
anomalous pulmonary venous connection, valve stenosis, aortic coarctation, coronary artery disease, doublechamber right ventricular, myocardial disease, etc.
• Some CHDs are difcult to be diagnosed by prenatal
ultrasonic examination as follows: conotruncal
defects, such as TOF, transposition of the great artery,
DORV, TA.
4 Ultrasonic Diagnosis ofFetal Heart
211
a
b
c
Fig. 4.32 Normal view of the pulmonary vein. (a) Three pulmonary
veins (arrows) return into the left atrium in the four-chamber view of
the normal fetal heart. (b) The fetal four-chamber view shows Color
ow imaging of pulmonary venous blood ow (arrow) return into the
• Some CHDs cannot be diagnosed by prenatal ultrasonic examination as follows: patent ductus arteriosus and patent foramen ovale.
7. Notice Items in Fetal Echocardiography.
There are some difculties and limitations in fetal echocardiography. To avoid unnecessary medical disputes,
we should pay attention to the following aspects:
• Standardize medical behaviors. Performing fetal
echocardiography is highly specialized, which
requires relevant clinical knowledge of cardiovascular disease, knowledge of basic cardiac anatomy, and
the ability to diagnose various heart diseases with
ultrasound.
• The limitations of ultrasonography should be under-
stood sufciently. During pregnancy, small fetal cardiovascular volume, variable fetal position, maternal
obesity, polyhydramnios, and oligohydramnios are
important factors that affect the fetal heart examina-
LA. (c) Color ow imaging of pulmonary vein blood ows to the LA
(arrows show pulmonary veins) in the four-chamber view in a supine
position
tion. Screening is challenging, which requires
patience and meticulousness to improve the diagnosis
of fetal cardiovascular disease.
• Due to the ultrasonic biological effect, shorten the
examination time as much as possible.
• You can ask the pregnant woman to lie on the left or
right side or to perform appropriate activities, if the
fetal position is not suitable or some structures are not
well displayed, to obtain a satisfactory image of the
fetal heart.
• For complex fetal CHD, only partial malformation
images can be obtained, and make a relevant qualitative diagnosis, which is difcult to further accurate
classication.
8. Development of fetal echocardiography
• New applications of fetal 2-D echocardiography
In the past decades, with the booming develop-
ment of minimally invasive surgery in neonates, the
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a
c
b
d
e
Fig. 4.33 Fetal cardiac tumor. (a) The four-chamber view shows
hyperechogenic masses in the RV and LV. (b) Color ow imaging of the
same view shows no obvious obstruction. (c) The interventricular septum is thickened and hyperechogenic in the four-chamber view. (d) The
same view shows poor blood ow of the cavity, indicating cardiac
obstruction. (e) In the four-chamber view, multiple hyperechogenic
masses are observed both in the RV and LV after birth
4 Ultrasonic Diagnosis ofFetal Heart
213
application of fetal minimally invasive technology is
increasing. 2-D sonography, as an advanced interventional guidance technology, plays a vital role in the
minimally invasive operation of fetal closed treatment. Some CHD in fetal period, such as valvular
stenosis or atresia, may have hemodynamic changes
which affect the development of atrium and ventricle.
Fetal development will be affected without early
intervention, and further operation for correction is
needed after birth. At present, large quantities of
researchers are exploring various inuencing factors
of fetal intervention therapy, in order to improve the
success rate of treatment.
• New ultrasonic techniques for fetal cardiac
examination
The increasingly widespread application of ultrasound requires the improvement of technology for
ultrasonic diagnosis. Therefore, with the development of computer and ultrasound application technology, a variety of new ultrasonic technologies have
developed, demonstrating an amazing prospect.
Among them, many of the latest technologies can be
applied in fetal echocardiography. For example, tissue Doppler technology is used to determine the location and classication of fetal arrhythmia, analyze
myocardial activity, and monitor the overall and local
functions of the heart. Harmonic imaging technology
is used to improve the image quality of the fetal heart,
especially that of obese pregnant women.
• The application of fetal 3-D echocardiography
Since the application of 2-D echocardiography in
fetal heart examination, the diagnostic accuracy of
prenatal CHD, especially some severe CHD, has
remained at a low level. The quality of the images is
limited by various objective factors, such as gestational age, fetal position, shadow of the ribs, and
maternal abdominal wall thickness. Moreover, qualied images depend on the operator’s skills in screening and extensive experience in diagnosing. In the
1970s, with the introduction of 3-D ultrasound in
adult transthoracic echocardiography, this technique
was also introduced into fetal echocardiography subsequently. Fetal 3-D echocardiography has undergone a process from static to dynamic, from delayed
to real time.
– Spatial-temporal image correlation (STIC)
STIC realizes two key points in 3-D dynamic
imaging technology. One is to collect lots of volume datasets at a certain time of the cardiac cycle,
and the other is the electrocardiogram (ECG) gating. STIC is a kind of delayed 3-D imaging technology with fast reconstruction speed and satised
reconstructed images. STIC has a powerful post-
analysis technology in ofine analysis software,
which has become the study focus in recent years.
The following are the applications of 3-D ultrasonography based on STIC for the structural and
functional evaluation of the fetal heart.
– Application of STIC and tomographic ultrasound
imaging (tomographic ultrasound imaging, TUI)
(TUI-STIC) in the segmental analysis of fetal
CHD
Segmental analysis is often used to describe
cardiovascular characteristics of the fetus with
CHD.The segmental analysis method includes the
analysis of all the views from the fetal abdominal
to the ductal arch. TUI-STIC benets to display
the structural lesions of complex CHD vividly,
such as visceral ectopic syndrome, complex
conotruncal anomalies, etc. TUI imaging mode
can be used to display several parallel cardiac
plans simultaneously after volume data reconstructed by STIC, which helps to understand fetal
cardiac structural abnormalities spatially.
D.Paladini etal. performed a TUI-STIC examination on 103 CHD fetuses conrmed by 2-D fetal
echocardiography. The research shows that all
cases can obtain precise segmental analysis results
using TUI-STIC.
– 3-D cardiac imaging of STIC with surface render-
ing (STIC-rendering)
Many planes cannot be displayed by standard
views of 2-D fetal echocardiography, such as the
lateral view of the atrial septum and ventricular
septum, the transverse view of the atrioventricular
annulus. STIC-Rendering can render the plane of
hole ventricular septum or atrial septal on the
screen, enabling observers to view the complete
septum from the atrium–atrium and ventricle–ventricle perspectively. The number and motion of
atrioventricular valves are vital to atrioventricular
connection. The shape of the semilunar valve
plays an important role in determining the ventricular arterial connection. However, regular 2-D
ultrasound, which can only observe the heart from
the long axis: the anterior and posterior view of the
atrioventricular valve ring and the plane of the
coronary-atrioventricular valve. Whereas, STICrendering can display the above views.
– STIC combined with 2-D B-ow imaging mode
(STIC-B ow) for the diagnosis of pulmonary vascular abnormalities
B-ow imaging is a new imaging technology of
coded digital ultrasound, which uses “coded excitation” to enhance signals from weak blood ow
and suppress signals from the static tissues.