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109

Pictorial

Congenital
Diagnosis
Douglas

Cardiac

L. Brown,1

W. Chapman

Donald

Anomalies:

S. Emerson,1

Mark

S. Cartier,1

The

and Normal

fetal

heart

Findings

should

be evaluated

routinely

during

obstetric
sonographic
examinations.
The basic view for
imaging
the fetal heart is the four-chamber
view. This view,
however,
may not show all anomalies,
and a segmental
approach
allows
a more thorough
evaluation
[1 , 2]. The
four-chamber
view (Fig. iA) is obtainable
in most fetuses
older than 1 6 weeks.
The great arteries
and their connections also must be evaluated,
and although
several
views

are useful,

Richard

Sonographic

E. Felker,1

Thomas

G. DiSessa,2

and

Smith1

Many abnormalities
of the fetal heart can now be diagnosed
by routine
obstetrical
sonography.
This pictorial
essay illustrates
the normal
fetal heart and some of the
more common
fetal cardiac
structural
anomalies.
Diagnoses that often can be made prenatally
include ventricular
septal defect, endocardial
cushion
defect, Ebstein anomaly,
tetralogy
of Fallot, hypoplastic
left heart syndrome,
complete transposition
of the great arteries,
and double-outlet
right ventricle.
Coarctation
of the aorta is a challenging
diagnosis
but can sometimes
be suggested.
Lesions
that
are often difficult
to detect,
even with an optimal
sonographic examination,
include ostium secundum
atrial septal
defects,
small ventricular
septal defects,
semilunar
valve
stenoses,
and total anomalous
pulmonary
venous
return.

Technique

Prenatal

Essay

we find the long-axis

showing
the left ventricular
aortic arch (Fig. 1 C) is best

outflow
imaged

view

is most

helpful

tract (Fig. 1 B). The

in a longitudinal
axis.

Received
December
21 , 1 988; accepted
after revision
February
1 Department
of Radiology,
university
of Tennessee,
Memphis,
2 Department
of Pediatric
Cardiology,
University
of Tennessee,
AJR 153:109-114,

in

July 1989 0361-803x/89/1531-0109

The right ventricular

outflow
tract can be evaluated
in the
short-axis
plane (Fig. 1 D).
Important
observations
are that (1 ) the great arteries are
at nearly right angles to each other, with the aorta initially
crossing
from left to right and the pulmonary
artery from
right to left; (2) the pulmonary
valve is anterior,
cranial, and
to the left of the aortic valve; (3) the pulmonary
artery
is
minimally
larger than the aorta; and (4) the pulmonary
artery
arises from the anterior
ventricle.

Structural

Abnormalities

Ventricular

Septal

Defect

Ventricular
septal
defect
is the most common
cardiac
anomaly.
It may be isolated
or part of a more complex
abnormality.
There
is little or no hemodynamic
consequence
in utero. The sonographic
diagnosis
is based on
imaging the discontinuity
in the ventricular
septum
(Fig. 2).
This abnormality
can be both over- and underdiagnosed.
Figure
3 shows a potential
pitfall in overdiagnosis.
A small
defect,
particularly
when less than 1 -2 mm, may escape
detection.
Such a small isolated
defect
is unlikely
to be
important

clinically.

Endocardial
Endocardial
septal
defect,

Cushion
cushion
results

Defect
defect,
also called atrioventricular
from persistence
of the primitive

24, 1989.
800 Madison
Ave. , Memphis,
TN 381 63. Address
reprint
Memphis,
800 Madison
Ave. , Memphis, TN 38163.

C American Roentgen

Ray Society

requests

to D. L. Brown.

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Fig. 1.-Normal
fetal heart.
A, Four-chamber
view is obtained
with
essentially
a transverse
Image of fetal
thorax. Normally, right and left atria are approximately same size as right and left yentricles.
Foramen ovale is seen with septum
primum bulging into left atrium. Morphologic

left ventricle has a pointed apex, whereas

right ventricle contains moderator band and
its apex

is blunted.

Atrioventricular

valves

are at essentially the same level, although

tricuspid valve joins septum slightly more
toward apex of heart than does mitral valve.
B, Normal left ventricular outflow tract is
shown in long-axis view of LV, which is obtamed
by slight cranial angulatlon of transducer from four-chamber view and slight rotation of transducer (if fetal spine is posterior, rotate transducer clockwise on maternal
abdomen).
Continuity
between
ventricular
septum and anterior wall of aorta Is shown.
C, Longitudinal view of aortic arch with relatively narrow candy cane curve. Aorta is identified by head and neck arteries (straight
arrows)
originating
from it.
D, Normal right ventricular outflow tract is shown by slightly more cranial angulation and rotation of transducer
counterclockwise
(if fetal spine is
posterior).
PA is seen arising from RV. Note characteristic
branching of PA into right pulmonary artery and ductus arteriosus
in this projection. Left
pulmonary artery Is located Inferior to this plane.
E, Longitudinal view of ductus arteriosus arch with relatively
wide walking stick curve.

Fig. 2.-Ventricular
septal defect (30 weeks).
Four-chamber view shows defect (arrow) in yentricular septum
nearcrux
of heart.
may be observable
in four-chamber

four-chamber
Sweeping

Although
defect
view, a single

view shows only part of septum.

from apex

to base

of heart

In short-axis

projection is helpful in evaluating entire septum.

Fig. 3.-Pseudo-ventricular
A, Apical four-chamber

just below atrioventricular

lateral
gradual

septal
view (sound

defect
beam

(VSD) (20 weeks).

parallel
to septum)

valves. This artifactual

shows defect (arrow)

In septum
defect is thought to be due to limitations of

resolution
and relative
thinness
of this portion
of septum.
It can
fading of septum
instead
of a more abrupt termination
of septum

be recognized
by a
with a true VSD and

by absence of a defect when Imaging perpendicular to septum.

B, Four-chamber
view from slightly more lateral position shows Intact ventricular

septum.

AJR:153,

SONOGRAPHY

July 1989

OF CONGENITAL

CARDIAC

ANOMALIES

111

canal. It is associated
with Down syndrome
of patients with endocardial
cushion defect have
21) and with left and right atrial isomerism. Corn-

atrioventricular

Used in Figures

Key to Abbreviations

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(30-40%
trisomy
plete

AO

aorta

AscA

ascending

thoracic

aorta

DA

ductus

DscA
IA

descending

arteriosus

thoracic

innominate

artery

L
LA
LCA

left
left atrium
left common

LSA

left subclavian

LV
MV

left ventricle
mitral valve

PA

pulmonary

A
RA

right
right

atrium

RPA

right

pulmonary

RV

right

ventricle

S
SP
TV
VS
VSD

spine
septum primum
tricuspid valve
ventricular septum
ventricular septal defect

aorta

heart

septum

carotid

artery

artery

Fig. 4.-Endocardial
ventricular

cushion defect.
view (28 weeks)
shows
at crux of heart. Atrial and

sspta do not meet, nor do atrioven-

tricular valve Insertions.

B, Another
patient
(35 weeks),
four-chambar view. Note absence
of lower part of atrial
and higher part of ventricular
septum.
Normal

slight differential

Insortlon

valves
Is lost (cf. Fig.
common
atrioventricular

of atrloventricular

IA),
and there
valve (arrOw).

Is a

Fig. 5.-Normal
coronary
sinus (28 weeks).
A, This may be seen when transducer
Is
angled
Inferiorly
from typical
four-chamber
view. Coronary
sinus (arrow)
Is In atrioventricular groove
on left, crossing
to right to enter
right atrium.
It should not be mistaken
for an
isolated
ostlum primum atrial septal defect.
B, Slightly
different
transducer
angulatlon
shows charact#{149}rlstlc location
and tubular
nature of coronary
sinus (arrow).

4). The

valve

regurgitation

can

normal

differential

insertion

of the

Anomaly
anomaly

various

degrees,

tricuspid

valve

be dysplastic.
leading

is due to downward
displacement,
to
of the septal and posterior leaflets of the

into

the right

ventricle.

The

valve

is usually

The tricuspid

to right

atrial

enlargement,

which

valve

also

may

insufficient,

is evident

sono-

graphically
(Fig. 6). The resulting
right ventricular
cavity
may be small, as may the pulmonary
artery. Tricuspid
regurgitation
may be shown by Doppler examination.
Tetralogy
Tetralogy
deviation

A, Four-chamber
defect (Iarg. arrow)

(Fig.

Ebstein

artery

or atrioventricular

atrioventricular
valves may be lost, and a common
atrioventricular
valve may be present. One should not mistake
the coronary sinus for an ostium primum atrial septal defect
(Fig. 5).
Ebstein

artery

block

lead to congestive
heart failure and hydrops.
Sonographically, one observes
a defect in the inferior portion of the
atrial septum and the superior
portion of the ventricular

of Fallot
of Fallot

to be caused by anterior
septum, which causes pulmo-

is believed

of the infundibular

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112

BROWN

ET AL.

not be apparent,
pulmonary
artery

deviation
of the infundibular
septum,
which causes pulmonary infundibular
obstruction,
a malaligned
ventricular
septal defect,
and an overriding
aorta. Right ventricular
hypertrophy
is not thought
to be present
in the fetus. The right
ventricular
outflow
tract may be mildly stenotic
to atretic.
Anomalies
of the pulmonary
valve also may be present.
If
the pulmonary
valve is absent,
there may be aneurysmal
dilatation
of the pulmonary
artery and its more peripheral
branches.
Tetralogy
of Fallot does not usually cause hemodynamic
compromise
in the fetus. The sonographic
diagnosis is based on observing
the aorta overriding
the yentricular
septal
defect
(Fig. 7). Recognition
of the aorta
overriding
the ventricular
septum
should prompt
examination

of

the

pulmonary

artery,

as it is not diagnostic

AJA:153,

Coarctation

particularly
early
has been observed

July 1989

in pregnancy;
a small
later in gestation
[1].

of the Aorta

Coarctation
is due to a discrete shelflike lesion, usually
opposite the entrance of the ductus arteriosus.
A bicuspid
aortic valve occurs
in up to 70-85%
percent
of patients,
although
this may be difficult
to determine
prenatally.
Coarctation
is common
in Turner syndrome.
It is not likely
to cause significant
hemodynamic
disturbance
in the fetus.
The sonographic
diagnosis
is challenging
but can be sus-

pected

of

when

ventricular

size is disproportionate,

with the

left ventricle
smaller
than the right ventricle
(Fig. 8). A
subjective
impression
of ventricular
disproportion
is made
from the real-time
image; measurements
can be obtained
for confirmation
[4]. The pulmonary
artery may be enlarged

tetralogy.
If the pulmonary
artery arises normally
from the
right ventricle,
tetralogy
of Fallot is the diagnosis.
If the
pulmonary
artery arises from the aorta, truncus
arteriosus
is present,
and if there is no pulmonary
artery, the diagnosis
is pulmonary
atresia.
Infundibular
pulmonary
stenosis
may

compared
specific

with the aorta. These findings,

however,

are not

for coarctation.

Fig. 6.-Ebstein
anomaly
(34 weeks).
Transverse image of fetal thorax shows right
atrial

enlargement

and

ment of tricuspid

Fig. 7.-Tetralogy
Long-axis
view
tricular
septum

produce

downward

of Fallot

reports

I,._1

(35

weeks).

shows aorta overriding

yenand a VSD. It Is possible
to

a false

Image

of sortie

Origin of this artifact is uncertain.

is seen, it should
be confirmed

different

displace-

valve leaflet.

angulatlon

overriding.
If overriding
with slightly

of transducer.

that If long-axis

view

Allan [1]

is obtained

such

that posterior

wall of aorta is continuous

with

anterior

of mitral

sep-

cusp

valve,

ventricular

tum should be continuous with aorta. (Reprinted with permission from Benson et al.
[3].)

Fig. 8.-Coarctatlon
of aorta (25 weeks).
A, Four-chamber
view shows slight decrease
in LV size relative to RV size. Arrows

indicate free walls of ventricles.

cific finding Is only suggestive
B, Longitudinal view of sortie
narrowing
(arrow)
in isthmlc

narrowing
to image

from coarctatlon
directly.

When

This nonspeof coarctation.

arch suggests
regIon.

Actual

usually is difficult

seen,

this narrowing

must be interpreted cautiously, as it can be

simulated at times in normal fetuses.

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AJA:153,

SONOGRAPHY

July 1989

OF

CONGENITAL

CARDIAC

ANOMALIES

113

Fig. 9.-Hypoplastic
left heart syndrome
(19 weeks).
A, Four-chamber
view shows small LV and LA.
B, Longitudinal
view shows ascending
aorta (arrows)
to be very small compared
with pulmonary
artery.
C, Pulmonary artery (cursors and arrows).
In hypoplastic
left heart syndrome,
left atrium may be small or normal in size. Left ventricle
is usually
severely underdeveloped,
but there is a spectrum
of severity. Typically, mitral valve is hypoplastic
and sortie valve is an imperforate membrane.
Ascending
aorta and arch are most often hypoplastic.

Fig. 10.-Complete
transposition
of the great arteries
(34 weeks).
A, Rather than crossing perpendicular
to each other, aorta and PA exit heart in parallel.
B, Longitudinal
view shows
aorta arising
from more anterior
right ventricle.
Other views

showed PA arising from left ventricle.

Fig. 11.-Double-outlet
right ventricle
(27
weeks).
As In complete
transposition,
normal
crossing
of aorta and PA is absent,
and they
exit in parallel.
In this anomaly,
however,
both

great arteries

Hypoplastic

Left Heart

Syndrome

Complete

This syndrome
consists
of a small left ventricle
with aortic
atresia and mitral valve hypoplasia
or atresia.
Its pathogen-

esis

remains

unexplained.

There

is usually

no hemody-

namic compromise
in utero, as the right ventricle
supplies
both the pulmonary
and systemic
circulations.
Sonographically, one observes
a small left ventricular
cavity (Fig. 9).
This is often obvious
from the four-chamber
view. Normal
measurements
of ventricular
cavity
size are available
for
comparison
if needed [4]. If a small left ventricular
cavity is

recognized,

one should

valve, a small ascending

then look for atresia

aorta, and

of the aortic

a small mitral valve.

arise from right ventricle.

Transposition

In complete
transposition
of the great arteries,
the atrioventnicular
connections
are normal,
but the ventriculoarterial connections
are abnormal.
Failure of the aorticopulmonary septum
to follow its normal spiral course has been
proposed
as the cause.
With complete
transposition,
the
relative
positions
of the aorta and pulmonary
artery vary,
although
in most cases the aorta is anterior
and to the right
of the pulmonary
artery.
Usually there is no hemodynamic
consequence
in utero,
although
congestive
heart failure
can occur if pulmonary
stenosis
is present.
Sonographically, one observes
the absence
of the normal crossing
of

1 14

the

BROWN

aorta

vessels
The

and

the

pulmonary

exit the ventricles

aorta

arises

from

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Double-outlet
abnormalities.

Right

artery

Instead,

the

great

to each other (Fig. 10).

the anterior,

tricle, and the pulmonary

morphologic
left ventricle.
Double-Outlet

artery.

parallel

morphologic

right

yen-

arises from the posterior,

is one of the transposition

the pulmonary
artery
and

most of the aorta arise from the right ventricle.

A ventricular

septal defect is almost always present.

There is usually no
hemodynamic
consequence
to the fetus, unless obstructing lesions
such as pulmonary
or mitral stenosis
also are

present.

The sonographic

AJR:153,

both great arteries arising

ventricle (Fig. 1 1). Accurate
times,

as the

transposition

abnormality

or tetralogy

July 1989

predominantly
from the right
diagnosis
may be difficult at
may

appear

similar

to complete

of Fallot.

REFERENCES

Ventricle

right ventricle
It occurs
when

ET AL.

diagnosis

is based on observing

1 . Allan LD. Manual of fetal echocardiography.

Lancaster,
England:
MTP
Press Ltd., 1986:13-47, 108, 127-1 37
2. Romero A, Pilu G, Jeanty P, Ghidini A, Hobbins JC. Prenatal diagnosis of
congenital
anomalies.
Norwalk,
CT: Appleton
and Lange, 1988:
125-194
3. Benson
CB, Jones
TB, Lavery
MJ, Platt LD. Atlas
of obstetrical
ultrasound.
Philadelphia Lippincott, 1988:210
4. DeVore
GA. Fetal echocardiography-the
challenge
of the 1980s.
Semin US CT MR 1984;5:229-252