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Downloaded from http://heart.bmj.com/ on May 9, 2017 - Published by group.bmj.com
ii12
Heart 2001;86(Suppl II):ii12–ii22
Fetal echocardiography: 20 years of progress
H M Gardiner
Considerable advances in ultrasound technology and close collaboration between the
specialties of paediatric cardiology and fetal
medicine have resulted in the increasing ability
to diagnose congenital heart disease before
birth over the last two decades. Fetal cardiology
diVers from paediatric cardiology not only in
the spectrum of diseases seen, but also in the
assessment of function because of the extended
feto-placental circulation. Importantly, outcomes for specific lesions may diVer as extracardiac abnormalities and chromosomal defects
may alter the prognosis of otherwise straightforward cardiac lesions such as tetralogy of
Fallot.1 Historically the examination of the fetal
heart and circulation developed separately,
obstetricians using Doppler to examine the
uteroplacental circulation to study the pathophysiology of pregnancy,2 3 while cardiologists
focused their attention on the morphological
aspects of cardiac development using M mode
and Doppler.4 5 In today’s practice a comprehensive evaluation of the fetal cardiovascular
system includes both structural and functional
assessment using new technologies to monitor
the progression of cardiac defects and to aid
management decisions such as the need for
early delivery, treatment of arrhythmia or for
interventions such as balloon valvuloplasty of
semilunar valve obstruction.
Scanning the fetal heart: specialised
equipment and developments
PROBES
Transvaginal scanning of the heart is favoured
in some centres as part of the first trimester
scan at 11–14 weeks, and impressive views can
be obtained because of the close proximity of
the probe to the fetal heart when the fetal lie is
favourable.6 Transabdominal fetal cardiac
scanning is more commonly performed. Diagnostic views can be obtained at 14 weeks using
modern probes with limits of resolution of
about 50 µm in the axial plane at 6 Mhz and
less than 100 µm in the lateral plane, at normal
obstetric scanning depths. A curvilinear probe
with a cardiac package incorporating fast frame
rates is ideal, although standard cardiac probes
may also be used which have the advantage of
continuous wave Doppler available to interrogate high velocity atrioventricular valve regurgitation or severe semilunar valve stenosis.
Colour flow mapping and Doppler may be
helpful adjuncts to early diagnosis but their use
should be limited in the first trimester to avoid
any possible damage to the developing fetus.
Special presets for examination of the fetal
heart should be used to ensure that the spatialpeak temporal average power output for colour
and pulsed Doppler mode is < 100 mW/cm2.
NEW IMPROVEMENTS IN IMAGING
Division of
Paediatrics, Obstetrics
and Gynaecology,
Faculty of Medicine,
Imperial College
School of Science,
Technology and
Medicine, Royal
Brompton and
Harefield Hospital,
Sydney Street, London
SW3 6NP, UK
H M Gardiner
Correspondence to:
Dr Gardiner
[email protected]
The underlying problem
Congenital heart disease aVects 6–8 per 1000
live births, at least half of which should be
detectable before birth because of their serious
nature. However, if examination of the fetal
heart is confined to traditional high risk groups
only about 20% of babies born with congenital
heart disease will be identified (table 1).
Therefore the focus must be to examine all
pregnancies for cardiac malformations. The
opportunity to do so is present in some centres
at 11–14 weeks when nuchal fold thickness is
measured to assess the risk of chromosomal
defects in the fetus. However, in the UK most
pregnant women are assessed for the first time
in detail by sonographers at the level 2 or
anomaly scan at about 20 weeks in their local
obstetric ultrasound department.
Table 1
Two dimensional imaging is still the most
commonly used imaging modality in fetal
echocardiography. Most probes in use have
very fast frame rates, achieved by parallel
processing where the transducer transmits one
line and receives two, resulting in a doubling of
previous rates. This is now standard practice
and most useful in fetal echocardiography at
the 5 or 4 MHz settings when high maternal
body mass index makes visualisation diYcult,
or in polyhydramnios where the fetus may be at
more than 20 cm depth. Transmitting at
2 MHz and receiving at 4 MHz greatly improves the resolution at depth and decreases
interference caused by side lobes. This occurs
at the expense of reduced frame rates, which
can make fetal valvar structures appear
“thicker” than they really are. A 4:1 ratio will
soon be available but spatial resolution will
Factors defining the high risk population
Fetal factors
Maternal factors
Ultrasound findings
Extracardiac anomalies
Abnormal chromosomes
Multiple fetal pregnancy
Fetal cardiac arrhythmia
Family history/Maternal CHD
Diabetes mellitus
Autoimmune antibodies
Drugs—for example, lithium,
anticonvulsants
Increased nuchal fold, suspicious four chamber scan
Non-diagnostic scan
“Soft” diagnostic markers—for example, echogenic
cardiac foci, short femurs, echogenic bowel,
symmetrical growth restriction
CHD, congenital heart disease
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Fetal echocardiography
Figure 1 Colour Doppler energy scan of a 13 week fetal heart showing: (A) normal four chamber view with symmetrical flow into right (RV) and left
(LV) ventricles; (B) normal aortic and (C) ductal arches that can easily be distinguished by the hooked and broad orientation they take as they arise from
their respective ventricular origins.
necessarily be compromised because the transmitting beam will cover four lines. More
advanced techniques such as three dimensional
rendering and spatial compounding may be of
use in the future, but currently are used in
more general imaging settings and as research
tools. Spatial compounding creates a frame
consisting of multiple lines of data from various
locations on the transducer. The resulting
image improves spatial resolution (again at the
expense of frame rates) and reduces clutter
with elimination of ultrasound artefacts. The
major problem thus far in the application of
this in fetal echo is the loss of frame rates multiple sampling entails. For example, a typical
spatial compounding setting requires seven
views temporally averaged over seven times the
normal frame rate. For a fundamental rate of
49 Hz (49 frames/second), a seven view
compounded image will contain frames taken
over the last 7/49th of a second while the image
is being updated every 1/49th second. This
results in frame rates too slow for practical use
so far in imaging the fetal heart.
COLOUR DOPPLER IMAGING
Colour flow mapping and particularly power
Doppler are helpful in imaging flow in the very
small heart. The advantages of colour flow
mapping are that it allows assessment of valve
opening and of symmetry in ventricular filling
(fig 1A) in cases where visualisation using the
grey scale alone, even with harmonic imaging,
is diYcult. It may highlight areas of turbulence
or of important atrioventricular valve regurgitation that provide important clues to major
structural defects during first trimester scanning. Power Doppler has certain advantages in
early pregnancy as it fills the vessels well,
particularly when there is a change in direction
of flow that might lead to “black spots” in conventional colour velocity flow maps (figs
1B,C).
Pitfalls in the use of colour Doppler are its
tendency to “bleed” through the cross sectional
images creating misleading appearances, such
as perimembranous ventricular septal defects,
and falsely increasing the size of the great
arteries.
Fetal cardiac function
An assessment of fetal cardiac function can be
made using traditional M mode to provide
information on wall thickness and ventricular
shortening fraction, but it is somewhat crude.
Fetal long axis function may provide additional
insight into endocardial function, most usefully
in the detection of early ischaemic changes
before the development of sonographically
detectable endocardial fibroelastosis (EFE).
Long axis recordings show the importance of
atrial function in the fetal heart (fig 2A) and
Doppler tissue imaging (DTI) provides a
transmyocardial tissue velocity profile and permits assessment of radial and longitudinal
strain rate (figs 2B,C), but the timing of cardiac
events cannot be verified with a concurrent
ECG recording and currently depends on surrogates such as mitral valve closure signals. Left
ventricular DTI has shown increased duration
of early diastole and reduced endocardial
velocities in children with obstructive lesions
(such as aortic stenosis) before obvious abnormalities of function are apparent.7 In the future
these methods may be helpful in determining
the timing of interventions such as either early
Figure 2 (A) Long axis function of the fetal heart shows the excursion of the atrioventricular junction during the cardiac cycle and the importance of
atrial contraction “(a)” in fetal function. (B) Doppler tissue imaging in M mode may reveal more about endocardial function than conventional M mode
traces (C) as it allows calculation of transmyocardial tissue velocity profile and longitudinal strain rates. Surrogate gating of the cardiac cycle utilises the
mitral valve closing artefact (MV).
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Gardiner
delivery of the fetus, or of in utero interventions
such as fetal aortic balloon valvuloplasty,
before irreversible damage to the endocardium
occurs.
Congestive cardiac failure is seen in the fetus
during tachyarrhythmias caused by the rise in
systemic venous pressure and reduced ventricular filling time, but may also be seen
secondary to viral myocarditis and restrictions
to flow—for example, at the oval foramen level.
Associated findings may include an increased
cardiothoracic ratio, severe atrioventricular
valvar regurgitation, reversal of flow in the systemic veins at end diastole, and the development of eVusions or of fetal hydrops. A “heart
failure score” has been developed using this
profile and methods such as the Tei index,
which is afterload independent, may prove useful in serial assessment of the fetus with
hydrops.8 9
It is clear from these methods that fetal cardiac function is not assessed from measures of
intracardiac function alone but by Doppler
assessment of the fetal circulation, which
provides a more complete evaluation of the
eVects of cardiac preload and afterload on fetal
wellbeing. The salient features of the fetal
circulation that diVerentiate it from the postnatal cardiovascular system and make Doppler
such a useful tool are the arterial and venous
ducts, the oval foramen, and the aortic isthmus,
all of which close or diVer functionally after
birth. While the fetal ventricular arrangement
is in parallel, the arterial and venous ducts and
the oval foramen permit mixing of flows
between the right and left sides of the
circulation, thus allowing flow returning from
the placental circuit (with the highest oxygen
content) to reach the left heart (through the
venous duct and oval foramen) and therefore
perfuse the fetal brain. With increased pulmonary venous return after birth, left atrial
pressure rises and the oval foramen becomes
functionally closed, or if it remains patent flow
reverses to become a left to right shunt. In
addition, flow through the aortic isthmus
increases with a concomitant increase in size
An understanding of the role of these structures in the fetus helps with functional
assessment and in serial assessment of the fetus
with structural heart defects.
DEVELOPMENTAL CHANGES IN DOPPLER
WAVEFORMS
Doppler patterns that may be indicators of fetal
compromise in later pregnancy are a normal
early finding. An appreciation of this is important if first trimester assessment of fetal cardiac
function is performed.10 Flow in the early fetal
descending aorta and in the umbilical arteries
shows absent flow in diastole (fig 3A). This is
abnormal in later gestation as mature arterial
flow patterns show low velocity continuous
prograde flow throughout diastole (fig 3B).
The development of this pattern depends on a
successful twofold fall in systemic impedance
after 12 weeks gestation, secondary to placental
angiogenesis.11 Failure of adaptation leads to
increased left ventricular end diastolic pressure
and reversal of flow in the descending aorta
may be observed (fig 3C), with a reduction in
Figure 3 (A) Early Doppler flow in a 12 week gestation fetal aorta showing absence of diastolic flow (B) with continuous flow throughout the cardiac
cycle seen in the arteries (UA) and veins (UV) of the umbilical cord in the second trimester. (C) Absent or reversal of flow in diastole (AREDF) is a
pathological sign at this gestation indicating increased placental resistance. (D) Umbilical venous pulsations are an ominous sign and are accompanied by
absent end diastolic flow in this sick fetus. During early gestation flow towards the heart occurs mostly during ventricular systole (“S” wave) with a greatly
reduced velocity in early diastole (“D” wave) and reversal of flow into the vein during atrial contraction (“a” wave) (E). Doppler flow through the mitral
valve of a 15 week fetus illustrating the normal reversal of the E and A components of filling that characterise the fetal circulation (F).
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Fetal echocardiography
ventricular compliance and myocardial contractility12 often associated with poor intrauterine fetal growth.
VENTRICULO-VASCULAR COUPLING
Intracardiac Doppler is closely associated with
flow in the systemic veins throughout gestation
as evidenced during tachyarrhythmia. However, the early venous circulation is characterised by pronounced reversal of flow in the caval
veins coincident with atrial contraction. Inferior caval vein (IVC) reverse flow at 12 weeks
accounts for 25–30% of the total flow of the
IVC and is sixfold more than that seen at later
gestation (fig 3E).13 Atrial function is pivotal in
the early fetal circulation with monophasic patterns of ventricular filling at eight week’s gestation being replaced by diVerentiation into
dominant A and smaller E waves by nine weeks
and clear diVerentiation into mitral and tricuspid waveforms by 10–11 weeks (fig 3F). At all
stages during gestation the atrial wave is dominant, but the E:A ratio approaches unity at
term. Flow through the tricuspid valve is
greater than through the mitral throughout
gestation demonstrating right ventricular predominance. Umbilical venous pulsations are
also normal before 14 weeks of gestation and
are thought to be caused by transmission of
pressure waves (cardiac pulsations) along the
venous duct (fig 3D). However, flow in the
venous duct always remains prograde towards
the heart and is not characterised by reversal
during atrial contraction except in fetal hypoxaemia.14 Maturity leads to a major increase in
diastolic inflow in both the arterial and venous
components of the circulation and an increase
in cardiac compliance and relaxation rate.15
Congenital heart disease: making a
diagnosis
Despite the proven eYcacy of training sonographers to recognise cardiac abnormality in the
fetus16 17 only about a quarter of babies in the
UK born with a cardiac malformation are
diagnosed before birth.18 The detection of a
fetus who will have a duct dependent circulation after birth or who has simple transposition
of the great arteries with a restrictive oval
foramen may be life saving and has been shown
to confer benefit with improved neonatal
outcome.19 20
Understanding the role of the fetal arterial
and venous ducts and the aortic isthmus in the
circulation, and how functional abnormalities
secondary to structural cardiac defects manifest, may improve detection and alert the
examiner to deterioration.
The arterial duct directs 80% of the right
ventricular output to the descending aorta. Its
dimensions in the fetus are greater than those
of the aortic arch and flow in it is continuous
towards the descending aorta (figure 4A, B) at
high systolic (1.2–1.4 m/s) and continuous low
velocity diastolic flow. In congenital heart
disease with either right or left outflow tract
obstruction the arterial duct plays an important role in allowing blood to flow retrogradely
about the narrowed arch where there is left
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sided heart disease (for example, aortic stenosis, hypoplastic left heart syndrome) or down
the pulmonary trunk (fig 4C) in cases of severe
pulmonary stenosis or atresia when it may take
an abnormal course—the curly pig’s tail duct
(fig 4D). Both important aortic and pulmonary
stenosis may be less severe in early pregnancy
and the first signs of progression may be
reversal of flow in the arterial duct and
worsening atrioventricular valve regurgitation
(fig 4E).
The venous duct determines the flow of oxygenated blood to the left side of the circulation.
It is a small conical structure, measuring
1–2 mm at its narrowest portion, connecting
the intrahepatic part of the umbilical vein to
the inferior caval vein, closing after birth. Doppler signals from this vein are the highest in the
fetal venous circulation (40–80 cm/s) and prograde throughout systole and diastole). With
fetal hypoxia the venous duct dilates and allows
transmission of the atrial “a” wave, thus
producing an altered Doppler pattern.14 This
has been reported in fetuses with structural
cardiac defects in the first trimester and is
thought to reflect transiently altered fetal
haemodynamics associated with congenital
heart defects.
The fetal aortic isthmus is small and receives
only about 30% of the left ventricular output
(10% of the combined ventricular output). It
occupies a watershed position and flow volume
or direction within it is dramatically altered by
increasing umbilical resistance, or by decreasing cerebral resistance. Stepwise increases in
systemic resistance result in a reduction and
then negligible flow within the isthmus,
eVectively dividing the circulation into two
parallel arterial circuits such that the left
ventricle perfuses the ascending aorta, coronaries, and cerebral circulation and the right ventricle only the pulmonary flow and descending
aorta.21 With increasing downstream impedance there is reversal of flow about the aortic
arch, easily detected on colour flow mapping.
Because of the large arterial duct, detection of
a discrete coarctation of the aorta may be diYcult antenatally (and indeed may not be
evident until the duct closes after birth).
Therefore diagnosis often depends on surrogate measures such as an enlarged right ventricle22 or if tubular hypoplasia of the aortic arch
is present by an abnormal “three vessel view”.
PITFALLS OF EARLY SCANNING
The heart is formed and septation completed
by 50 embryonic days, and early imaging of the
heart is possible from transvaginal views at
about 11–12 weeks (63–70 days). Transvaginal
scanning in a large series of almost 40 000 early
pregnancies in Israel has detected cardiac
abnormalities in almost 0.5%.23 Just over a
quarter represented isolated abnormalities
with 40% associated with chromosomal abnormalities. The majority (82%) was found in
those formerly assessed as low risk pregnancies
(table 1) but one third of the total had
increased nuchal translucency indicating the
potential importance of this in screening in the
future. In one UK study, diagnostic images
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Gardiner
were obtained using transabdominal echocardiography in 226/229 fetuses (98.7%)
between 12–15 weeks, with abnormalities of
the cardiac connections detected in 13 fetuses
(5.7%) on the initial scan and another four
cases detected later in gestation or postnatally
(mostly ventricular septal defects).24 Conditions that are easier to detect in early gestation
are those with an absent connection or large
septal defect, those without a great arterial
crossover, or an apparently single great artery.
An important false negative finding at this early
stage is when progressive stenosis of an apparently normal semilunar valve occurs and may
result in severe conditions such as pulmonary
atresia/intact ventricular septum or hypoplastic
left heart syndrome later in pregnancy. Thus, it
is important to perform sequential scans to
confirm that growth is normal, or to clarify the
findings and progression of any malformation
identified in early gestation.
While echocardiographic screening hinges
on incorporating five transverse views of the
fetal abdomen and chest into the routine
obstetric scan (fig 5), images of the fetal heart
are more varied than those possible after birth
and resemble cuts obtained using magnetic
resonance imaging (fig 6). Limited scans, such
as the four chamber view alone, are insuYcient
and although this view may detect absent connections such as tricuspid atresia (fig 7A), large
septal defects (fig 7B), ventricular hypoplasia
associated with pulmonary atresia and intact
ventricular septum (fig 7C), and hypoplastic
left heart syndrome (fig 7D), major cardiac
abnormalities such as tetralogy of Fallot or
transposition of the great arteries that may have
normal four chamber views (figure 8A, C) are
likely to be missed unless the scan is extended
to include the outflow tracts, equivalent to the
“five chamber view” (fig 8B, D) and views to
assess the pulmonary trunk.25 Disproportion or
abnormal flow on colour Doppler at the “three
vessel view” is of tremendous importance in the
fetal cardiac examination (fig 5F). There is no
postnatal equivalent to this view that shows the
transverse aortic and ductal arches and superior caval vein (or persistent left SVC) (fig 5G).
Figure 4 (A) The pulmonary trunk extends into the arterial duct to
form a ductal arch (D) that is of similar size to the transverse aortic
arch (Ao), with flow in the same direction as shown on colour flow
mapping. (B) Doppler velocity in the arterial duct is of high velocity
and continuous. (C) Pulmonary atresia manifests in reversal of flow
from the transverse aortic arch retrogradely to the atretic valve and
may result in a curly “pig’s tail” duct (D). Tricuspid regurgitation of
high velocity and long duration impairs ventricular filling (E).
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Fetal echocardiography
ii17
Figure 5 (A) A full examination of the fetal heart may
be obtained by five transverse sections through the
abdomen and chest of the fetus. The first section shows
abdominal situs (B) with the aorta (Ao) to the left of the
spine and the inferior caval vein (IVC) anterior and to
the right. The normal fetal stomach (St) and heart lie on
the left side. The second section (C) illustrates the four
chambers of the heart with the left atrium (LA) in front
of the spine and the right ventricle (RV) just below the
sternum. The third cut (D) shows the aorta arising
centrally in the heart from the left ventricle (LV) and the
fourth the pulmonary trunk (PV) arising from the
anteriorly placed right ventricle and crossing to the fetal
left over the ascending aorta (E). The fifth section shows
the anteriorly positioned ductal arch (D) and the
transverse aortic arch (Ao) to be of equal size traversing
back to the fetal spine (F). A normal variant “three
vessel” view is shown with a right sided aortic arch and
persistent left superior caval vein (LSVC). The trachea
(T) can be seen lying between the aortic (Ao) and ductal
(D) arches (G).
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Gardiner
Figure 6 Additional views are often required to make a full diagnosis in cases of abnormality. (A) Coronal view of the
inferior bridging leaflet of the common valve in an unbalanced atrioventricular septal defect. (B) Sagittal views of the superior
(SVC) and inferior caval (IVC) veins draining to the right atrium (RA), and (C) of the aortic arch. (D) Coarctation of the
aorta may manifest as early ventricular disproportion with persistent enlargement of the right ventricle (RV).
Figure 7 Major abnormalities such as absent right connection (tricuspid atresia (A)), large septal defects (tetralogy of
Fallot (B)), ventricular hypertrophy or hypoplasia (pulmonary atresia with intact ventricular septum, (C)) and
hypoplastic left heart syndrome (D) may be diagnosed from these five screening views.
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Fetal echocardiography
ii19
Abnormality of this may be the first clue of
important semilunar valve or aortic arch
malformation, as the diagnosis of coarctation
of the aorta may be diYcult because of the
large patent arterial duct. The combination of
disproportion at the four chamber view and
three vessel view is suYcient to be forewarned
and to arrange delivery of a fetus near to a cardiac centre for early postnatal assessment and
possible surgery.22
Coronal views of the fetal heart are particularly useful for looking at the atrioventricular
valves and in delineating the features of a common valve in atrioventricular septal defect
(AVSD), particularly in the setting of ventricular imbalance (fig 6A). These views are similar
to postnatal subcostal views. Saggital views of
the fetus directed parallel to the fetal spine
profile the systemic venous connections, the
right ventricular outflow tract and ductal arch,
and the aortic arch and its branches (fig 6B, C).
Progression of cardiac lesions: lessons
and pitfalls
Major structural defects are relatively easy to
detect early in pregnancy and counselling is
straightforward—for example, an absent connection usually determines that the only surgical option will be univentricular palliation for
the child (a Fontan type circulation). However,
defects that worsen during the second and
third trimester, such as pulmonary atresia with
intact septum, may only show subtle morphological abnormalities at first—for example, tricuspid regurgitation and mild right ventricular
hypertrophy—and the severity may be underestimated as the right ventricle fails to grow
during pregnancy. Pulmonary stenosis has
been shown to progress during pregnancy26 and
postnatal evaluation of even mild fetal pulmonary stenosis is important. The assessment of
adequacy of ventricular size for suitability for a
functionally biventricular repair is diYcult
before delivery. The presence of a left superior
caval vein draining to an enlarged coronary
sinus in a normal heart may cause significant
disproportion in early pregnancy, which is
insignificant by term. In contrast, the heart
with multiple ventricular septal defects and a
slightly small aortic root may not be suitable for
a biventricular repair after birth. Hypertrophic
cardiomyopathy is usually only evident near
term, or in infancy, and has not been described
earlier in gestation. Therefore, where there is a
family history, follow up arrangements should
be set in place, despite the presence of a normal
early fetal assessment, which should not be
regarded as “reassuring”. Abnormalities of
Doppler flow, such as reversal of flow in the
pulmonary veins with increasing left atrial
pressure, may provide early clues to developing
or worsening cardiac disease in the setting of
hypoplastic left heart syndrome or of a restrictive oval foramen in transposition of the great
arteries—an early warning that the neonate
may require early balloon atrial septostomy to
prevent pulmonary haemorrhage and neonatal
demise (fig 9A–D).
Figure 8 Important conditions such as transposition of the great arteries and tetralogy of Fallot may be missed on a four
chamber view (A, C) and require visualisation of the great arteries to make the diagnosis (B, D).
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Gardiner
ii20
Figure 9 (A) Normal flow in pulmonary veins is triphasic with systolic (S), early diastolic (D), and late diastolic (d) peaks flowing forward into the left
atrium throughout the cardiac cycle as shown here along with systolic flow in a branch pulmonary artery. With increasing left atrial pressure there is firstly
reversed flow with atrial contraction “a” (B), reduction (C), and then absence of flow in late diastole (D). In contrast to other systemic veins, flow in the
venous duct is prograde throughout the cardiac cycle, even in early gestation (E) and reversal of flow is an ominous sign (F).
Fetal treatment
ARRHYTHMIAS
Complete heart block is thought to occur in
about 1:20 000 newborns. Tachycardias are
more commonly seen during infancy with a
prevalence of about 1:3000, although both
bradycardia and tachycardia may be recognised
more commonly in a fetal population.
The successful treatment of fetal tachycardia
may be lifesaving as untreated these fetuses
may become hydropic and die (fig 10A). Most
tachycardias are atrial in origin and occur in
fetuses without associated structural abnormalities. Indications for treatment are sustained tachycardia or fetal hydrops because
there is a risk of intrauterine death, estimated
between 20–50%.27 The diagnosis is made
using M mode or Doppler tracing, and simultaneous Doppler interrogation of the aorta and
SVC provides useful information as the
reversed “a” wave of the venous trace represents atrial activity and its relation with the
aortic flow allows assessment of the timing of
atrial and ventricular activity and calculation of
V-A intervals; thus, fetal tachycardias can be
divided into long (automatic atrial tachycardia
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and persistent junctional reciprocating tachycardia) and short V-A (atrioventricular reentrant tachycardias) intervals which guides
therapeutic management (fig 10B, C).28 Doppler tissue imaging at the atrioventricular junction has also been used to calculate the A-V
and V-A time intervals and looks promising.
Newer developments such as fetal ECG and
magnetocardiography also show promise but
M mode and Doppler remain the most practical methods to date.
If there is no hydrops high dose digoxin may
be given orally to the mother (transplacental
route) with the addition of flecainide or
verapamil if there is no cardioversion. Direct
fetal treatment by umbilical vein puncture
under ultrasound guidance (fig 10D) may be
more successful in the presence of hydrops.
Similar methods are used to assess the fetus
with heart block. Echocardiographic assessment is essential in these fetuses to exclude
abnormalities of situs as about 40% will have
complex structural heart disease, most often
left atrial isomerism with a complete atrioventricular septal defect, or congenitally corrected
transposition (double discordancy). These
fetuses need sequential evaluation to anticipate
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Fetal echocardiography
ii21
Figure 10 (A) Increased cardiothoracic ratio in a fetus with atrial flutter and hydrops. (B) Simultaneous Doppler
tracings of the systolic components and atrial wave (a) of the superior caval vein and aorta (V) showing a non-conducted
atrial ectopic beat. M mode through an atrium and ventricle or the aortic root (C) allow comparison of the timing of atrial
and ventricular beats. (D) Direct injection of digoxin into the fetal hepatic vein is eVective when hydrops prevents eVective
chemoversion via oral administration to the mother.
heart failure and hydrops and those with the
lowest heart rates (atrial rate < 100 and
ventricular rate < 45) are more likely to die in
utero. Maternal steroids, ionotropes, and fetal
pacing have all been tried but there is no clearly
superior treatment.29 30 Maternal antibody status (anti-Rho and -SSA) should be checked
and if positive the family counselled that there
is a risk of recurrence in future pregnancies of
up to 30%.
INTERVENTIONAL VALVULOPLASTY
Percutaneous ultrasound guided techniques
aimed at opening the stenosed fetal aortic valve
before birth have resulted in technical success
in less than half of the cases (6/14) attempted
worldwide with only one long term survivor.31
The indications currently for fetal intervention
are limited and the risks and benefits of the
procedure weighed carefully. Work is in
progress in lamb models to improve this
method using fetoscopic approaches guided by
transoesophageal echocardiography, which improves the imaging when the balloon catheter is
inside the fetal heart, but this approach has not
yet been used in the human fetus.32
Conclusions
Fetal echocardiography includes more than the
detection of cardiac defects before birth. Using
Doppler ultrasound we have gained insights into
the pathophysiology of the fetal circulation during development and have the, albeit currently
limited, ability to eVect change in the fetal circulation before birth in the hope that the postnatal
course will be ameliorated. Improvements in
ultrasound imaging and close collaboration
across specialties have enabled the majority of
these improvements to take place.
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1 Allan LD, Sharland GK. Prognosis in fetal tetralogy of Fallot. Pediatr Cardiol 1992;13:1–4 .
2 Campbell S, GriYn DR, Pearce JM, et al. New Doppler
technique for assessing uteroplacental blood flow. Lancet
1983;iii:675–7.
3 Laurin J, Lingman G, Marsal K, et al. Fetal blood flow in
pregnancies complicated by intrauterine growth retardation. Obstet Gynecol 1987;69:895–902.
4 Allan LD, Joseph MC, Boyd EG, et al. M-mode echocardiography in the developing human fetus. Br Heart J 1982;47:
573–83.
5 Allan LD, Chita SK, Al-Ghazali WH, et al. Doppler
echocardiographic evaluation of the normal human fetal
heart. Br Heart J 1987;57:528–33.
6 Achiron R, Rotstein Z, Lipitz S, et al. First-trimester
diagnosis of fetal congenital heart disease by transvaginal
ultrasonography. Obstet Gynecol 1994;84:69–72.
7 Kiraly P, Kapusta L, Thijssen JM, et al. Tissue velocity and
strain rate imaging-a new diagnostic approach. Cardiol
Young 2001;suppl 11:25.
8 Tulzer G, Khowsathit P, Gudmundsson S, et al. Diastolic
function of the fetal heart during second and third
trimester:
a
prospective
longitudinal
Dopplerechocardiographic study. Eur J Pediatr 1994;153:151–4.
9 Tei C. New non-invasive index for combined systolic and
diastolic ventricular function. J Cardiol 1995;26:135–6.
10 Hecher K, Campbell S, Doyle P, et al. Assessment of fetal
compromise by Doppler ultrasound investigation of the
fetal circulation. Arterial, intracardiac, and venous blood
flow velocity studies. Circulation 1995;91:129–38.
11 Jauniaux E, Jurkovic D, Campbell S. In vivo investigation of
the anatomy and the physiology of the early human placental circulation. Ultrasound Obstet Gynecol 1991;1:435–45.
12 Rizzo G, Arduini D. Fetal cardiac function in intrauterine
growth retardation. Am J Obstet Gynecol 1991;165(4 Pt
1):876–82.
13 Reed KL, Appleton CP, Anderson CF, et al. Doppler studies of vena cava flows in human fetuses. Insights into
normal and abnormal cardiac physiology. Circulation 1990;
81:498–505.
14 Kiserud T. In a diVerent vein: the ductus venosus could
yield much valuable information. Ultrasound Obstet Gynecol
1999;9:369–72.
15 Hecher K, Campbell S, Snijders R, et al. Reference ranges
for fetal venous and arterioventricular blood flow parameters. Ultrasound Obstet Gynecol 1994;4:381–90.
16 Hunter S, Heads A, Wyllie J, et al. Prenatal diagnosis of congenital heart disease in the northern region of England:
benefits of a training programme for obstetric ultrasonographers. Heart 2000;84:294–8.
17 Sharland GK, Allan LD. Screening for congenital heart disease prenatally. Results of a 2.5 year study in the south east
Thames region. Br J Obstet Gynaecol 1992;99:220–5.
18 Bull C. Current and potential impact of fetal diagnosis on
prevalence and spectrum of serious congenital heart
disease at term in the UK. Lancet 1999;354(9186):1242–7.
19 Brackley KJ, Kilby MD, Wright JG, et al. Outcome after
prenatal diagnosis of hypoplastic left-heart syndrome: a
case series. Lancet 2000;356:1143–7.
Downloaded from http://heart.bmj.com/ on May 9, 2017 - Published by group.bmj.com
Gardiner
ii22
20 Bonnet D, Coltri A, Butera G, et al. Fetal detection of transposition of the great arteries reduces morbitidity and mortality in newborn infants. Fetal Diagn Ther 1998;13:148.
21 Bonnin P, Fouron J, Teyssier G, et al. Quantitative
assessment of circulatory changes in the fetal aortic
isthmus during progressive increase of resistance to umbilical blood flow. Circulation 1993;88:216–22.
22 Sharland GK, Chan KY, Allan LD. Coarctation of the aorta:
diYculties in prenatal diagnosis. Br Heart J 1994;71:70–5.
23 Bronshtein M. First and early second trimester detection of
fetal heart disease. In Conference Proceedings. Advances in
fetal and perinatal cardiology. Hornberger LK, Allan LD,
eds. World Congress in Paediatric Cardiology and Surgery,
Toronto, 2001:39.
24 Simpson JM, Jones, A. Accuracy and limitations of transabdominal fetal echocardiography at 12-15 weeks of gestation
in a population at high risk for congenital heart disease. Br
J Obstet Gynaecol 2000;107:1492–7.
25 Kirk JS, Riggs TW, Comstock CH, et al. Prenatal screening
for cardiac anomalies: the value of routine addition of the
aortic root to the four-chamber view. Obstet Gynecol 1994;
84:427–31.
26 Todros T, Presbitero P, Gaglioti P. Pulmonary stenosis with
intact ventricular septum: documentation of development
of the lesion echocardiographically during fetal life. Int J
Cardiol 1988;19:355–60.
27 Allan LD, Chita SK, Sharland GK, et al. Flecainide in the
treatment of fetal tachycardias. Br Heart J 1991;65:46–8.
28 Fouron J-C. Doppler and M-mode assessment of fetal tachyarrhythmia mechanisms. In Conference Proceedings.
Advances in fetal and perinatal cardiology. Hornberger LK,
Allan LD, eds. World Congress in Paediatric Cardiology
and Surgery, Toronto, 2001:47–50.
29 Groves AM, Allan LD, Rosenthal E. Outcome of isolated
congenital complete heart block diagnosed in utero. Heart
1996;75:190–4.
30 Walkinshaw SA, Welch CR, McCormack J, et al. In utero
pacing for fetal congenital heart block. Fetal Diagn Ther
1994;9:183–5.
31 Kohl T, Sharland GK, Allan LD, et al. World experience of
percutaneous ultrasound-guided balloon valvuloplasty in
human fetuses with severe aortic valve obstruction. Am J
Cardiol 2000;85:1230–3.
32 Kohl T, Szabo Z, Suda K, et al. Fetoscopic and open
transumbilical fetal cardiac catheterization in sheep. Potential approaches for human fetal cardiac intervention. Circulation 1997;95:1048–53.
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Fetal echocardiography: 20 years of progress
H M Gardiner
Heart 2001 86: ii12-ii22
doi: 10.1136/heart.86.suppl_2.ii12
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