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A Practical Approach to Fetal Heart Scanning

Lindsey D. Allan

Evaluation o f the fetal heart can be readily incorporated into the obstetric ultrasound examination
and n e e d not add more than a few minutes to the examination. Correct analysis of the four-chamber
view and both outflow tracts will exchule the majority o f serious congenital heart disease. Where
cardiac malformations are identified during pregnancy, parents should be referred to a pediatric
cardiologist with expertise and experience o f fetal heart scanning for further counseling. This allows
for management o f the pregnancy to be tailored to the parents wishes and the type o f malformation
Copyright 9 2000 by W.B. Saunders Company

ardiac malformations are common, affect- congenital heart disease being detected is for
C ing about 8 o f 1,000 pregnancies. However, something abnormal being recognised during
only about 3 of 1,000 are serious and readily the obstetric scan. For this reason, the c o n c e p t
detectable prenatally. It is important to detect o f "screening" the fetal heart in a simplified
serious forms of congenital heart disease in the fashion during routine obstetric scanning was
fetus as they are associated with a significant introduced. 1
morbidity and mortality in affected children. Cardiac assessment during an anatomical ob-
All forms of congenital heart disease nowadays stetric survey is r e c o m m e n d e d to include at least
can be treated, although at varying levels of a four-chamber view o f the heart, 2 and this sin-
risk and quality of outcome. A clear understand- gle view will detect about 60% of serious malfor-
ing in early pregnancy o f the prognosis for the mations or abnormalities in about 2 of 1,000
child allows parents to make informed decisions pregnancies scanned. Despite the fact that al-
concerning the management. In continuing most all pregnancies are scanned at some time
pregnancies, the o u t c o m e for the affected child during gestation and that the four-chamber view
can be improved by optimizing perinatal man-
should be part of every ultrasound assessment,
agement. T h e r e are some categories of preg-
many forms of congenital heart disease recogni-
nancy in which there is an increased risk of
sable in this view continue to be overlooked
congenital heart disease and these mothers
during obstetric e v a l u a t i o n / M a n y authors have
should be referred to a fetal echocardiographer
suggested a more detailed study be p e r f o r m e d
for detailed study. These include mothers with a
during obstetric evaluation to increase the de-
family history of congenital heart disease, mater-
tection o f congenital heart disease by including
nal diabetes, exposure to cardiac teratogens in
identification o f the great artery connections. 4
early pregnancy, and the detection of fetal ar-
rhythmias or extracardiac abnormalities. This Accurate evaluation of the great arteries connec-
last group include those with extracardiac mal- tions will detect up to 90% of serious cardiac
formations, especially those with nuchal edema, malformations.
or fetal hydrops. However, 90% of mothers who
give birth to infants with congenital heart dis-
ease have no high risk features noted in their
pregnancy. The only h o p e these patients have o f Practical Scanning

For the perinatologist the following aspects o f

From the Department of Pediatric Cardiology, New York Presbyterian
Hospital, New Yark, NY. cardiac structure or function should be estab-
Address reprint requests to Lindsey D. Allan, MD, FRCP, FACC, lished: (1) The heart lies on the left side of the
Babies Hospital 2N, 3959 Broadway, New York, NY 10032; e-mail: fetus, on the same side as the stomach; (2) T h e
four-chamber view is normal; (3) T h e ventriculo-
Copyright 9 2000 by W.B. Saunders Company
O146-0005/00/2405-0002510. 00/0 arterial connections are normal; and (4) T h e
doi: 10. 1053/sper. 2000.16551 arch and duct are normal.

324 Seminars in Perinatology, Vol 24, No 5 (October), 2000: pp 324-330

A Practical Approach to Fetal Heart Scanning 325

The Heart Lies on the Left Side o f the in a systematic fashion, which includes an as-
Fetus (on the Same Side as the Stomach) sessment o f h e a r t size, position, structure, a n d
Normally, when the u l t r a s o u n d b e a m is swept
Size. Normally, the heart occupies about one
cranially f r o m a transverse section o f the ab-
third of the thorax. If there is d o u b t about the
d o m e n to the apex o f the heart, it can be seen
that b o t h the s t o m a c h a n d the h e a r t lie o n the heart size on a visual assessment, the area or the
same side of the fetus (Fig 1). This will almost circumference of the heart can be measured and
always m e a n that they are b o t h o n the left side c o m p a r e d to n o r m a l values. 7
b u t if they are discrepant, the side o f each Position. Normally, the midline of the tho-
must be d e t e r m i n e d . Also, a l t h o u g h rarely, rax passes t h r o u g h the left atrium, f o r a m e n
they can b o t h be n o r m a l but b o t h lie o n the ovale, the right atrium, a n d the a n t e r i o r cor-
right (situs inversus). T h e t e c h n i q u e o f Cordes ner o f the right ventricle, such that most of the
et al 5 for d e t e r m i n i n g "sidedness" appears to h e a r t lies in the left chest (Fig 2). The inter-
be reliable. T h e first step is to orientate the ventricular s e p t u m forms an angle o f a b o u t
t r a n s d u c e r in the long-axis o f the fetus with 40 ~ with the midline, s An a b n o r m a l angle o f
the h e a d to the right o f the screen. T h e trans- the s e p t u m can indicate a cardiac malforma-
d u c e r is then t u r n e d t h r o u g h 90 ~ in a clock- tion or a space-occupying lesion within the
wise direction. If the spine is posterior, the left chest. 9
side o f the fetus will be o n the right o f the Structure. To o r i e n t in the f o u r - c h a m b e r
screen. Conversely, if the spine is anterior, the view, it is useful to relate the h e a r t to the
left side o f the fetus wilt be o n the left o f the spine. O p p o s i t e the spine is the a n t e r i o r
screen. chest wall or s t e r n u m a n d below this is the
right ventricle. I m m e d i a t e l y a n t e r i o r and to
The Four-Chamber View is Normal the left o f the spine is the d e s c e n d i n g aorta
A f o u r - c h a m b e r view o f the fetal h e a r t is ob- a n d anterior to that is the left atrium. T h e
tained in a horizontal cross-section of the tho- right atrium a n d left ventricle can t h e n be
rax j u s t above the d i a p h r a g m . A n o r m a l four- deduced.
c h a m b e r view excludes m a n y forms o f In the four-chamber view, the following as-
c o n g e n i t a l h e a r t disease. It must be evaluated pects of structure should be seen:

Figure 1. (A) The stomach in the abdomen in the usual position. (B) Sweeping cranially, the four-chamber
view in seen in the thorax just above the diaphragm, with the apex on the same side of the fetus as the
326 Lindsey D. Allan

However, with s o m e o f the m o s t r e c e n t ultra-

s o u n d e q u i p m e n t , trivial tricuspid regurgita-
tion can be seen in a n o r m a l heart. H o w e v e r a
cause for tricuspid r e g u r g i t a t i o n s h o u l d be
e x c l u d e d b e f o r e d e n o t i n g this "physiological,"
as it is n o t as c o m m o n in the fetus as it is in
p o s t n a t a l life. n
In the normal heart, the atrial s e p t u m meets
the ventricular septum at the site of insertion o f
the 2 atrioventricular valves, f o r m i n g a "cross" at
the crux or center of the heart (Figs 1 a n d 2).
Because the septal leaflet o f the tricuspid valve is
inserted slightly lower in the ventricular s e p t u m
than the septal leaflet of the mitral valve, this
gives the a p p e a r a n c e o f "off-setting" or a cross
that is not quite straight. This is an i m p o r t a n t
n o r m a l finding that is lost in s o m e cardiac mal-
O n examination of the atrial septum, the fo-
Figure 2. The interventricular septum forms an an- r a m e n ovale can be seen to occupy the middle
gle of about 40 ~ to the midline of the thorax. The third o f the septum, although the size of the
right ventricle lies below the sternum. The left atrium
is anterior to the descending aorta, which is, in turn, f o r a m e n appears larger if it is i m a g e d in apical
anterior to the spine. projections. T h e flap valve is p u s h e d o p e n by a
j e t of b l o o d f r o m the ductus venosus via the
inferior vena cava a n d lies in the cavity o f the left
1. Two equally sized atria atrium during most of the cardiac cycle. It has,
2. Two equally sized ventricles however, a biphasic m o t i o n drifting toward the
3. Two equally o p e n i n g atrioventricular valves, atrial s e p t u m towards the e n d of systole a n d
no valvar regurgitation closing for a b o u t 20% o f the cardiac cycle dur-
4. An intact "crux" of the heart, with differential ing atrial contraction. 12 A right to left s h u n t
insertion, or "off-setting" of the atrioventric- t h r o u g h the f o r a m e n can be d o c u m e n t e d
ular valves. t h r o u g h o u t most of the cardiac cycle, although a
5. T h e p u l m o n a r y veins enter the back of the brief j e t of left to right flow can occur during
left atrium atrial systole. T h e flap valve can sometimes be
6. T h e f o r a m e n ovale defect occupies the mid- r e d u n d a n t and impinge on the left atrial wall in
dle third of the atrial septum the n o r m a l fetus.
7. T h e ventricular s e p t u m is intact Finally, it is i m p o r t a n t to focus on the ven-
tricular s e p t u m in o r d e r to e x c l u d e a ventric-
In the last 10 weeks of pregnancy, there can be ular septal defect. T h e s e p t u m s h o u l d b e
mild, or close to t e r m even fairly marked, dis- i m a g e d f r o m the a p e x to the c r u x in the four-
crepancy in the sizes of the ventricles with right c h a m b e r view, thus i m a g i n g p a r t o f the mus-
heart dilatation, even w h e n the heart is normal. cular a n d inlet s e p t u m a n d t h e n the b e a m
However, pathological causes of right h e a r t di- swept u p to the aortic outflow, which i m a g e s
latation should be excluded, such as coarctation m o s t o f the rest o f the m u s c u l a r s e p t u m a n d
of the aorta a~ or totally anomalous p u l m o n a r y the p e r i m e m b r a n o u s a n d o u t l e t parts o f the
venous drainage, before attributing any discrep- s e p t u m . It should be i m a g e d in b o t h apical
ancy to gestational age. p r o j e c t i o n a n d lateral p r o j e c t i o n s (Fig 3). I f
T h e a t r i o v e n t r i c u l a r valves should be e x a m - t h e r e is a real v e n t r i c u l a r septal defect, c o l o r
i n e d by c o l o r flow m a p p i n g to e n s u r e that the flow will be seen to b r e a c h the s e p t u m in a
orifices are o f e q u a l size a n d that b o t h ventri- lateral view w h e n the s e p t u m is p e r p e n d i c u l a r
cles fill equally in diastole. In addition, t h e r e to the b e a m . However, small defects, especially
s h o u l d be n o r e g u r g i t a t i o n f r o m e i t h e r valve. in the p e r i m e m b r a n o u s region or m u l t i p l e de-
A Practical Approach to Fetal Heart Scanning 327

T h e Ventriculo-Arterial C o n n e c t i o n s
are N o r m a l

The great artery connections can be assessed in

a variety o f different views, b o t h transverse and
longitudinal. The transverse views are usually
easier to obtain and involve sweeping cranially
from the four c h a m b e r view to show the aorta
arising in the center of the heart just above
the two atrioventricular valves (Fig 4). It is im-
portant to note that the posterior leaflet of the
aortic valve is in continuity with the anterior
leaflet of the mitral valve and that the anterior
wall of the aorta is continuous with the ventric-
ular septum. Slight transducer angulation so
that the beam cuts between the right shoulder
and left hip often "opens out" the left ventricu-
lar outflow tract optimally. The aorta sweeps out
to the right of the thorax before turning left-
wards to form the aortic arch. The p u l m o n a r y
Figure 3. There is equal color flow into both ventri- artery arises anterior and cranial to the aorta, is
cles through the atrioventricular valves. Here the seen in a transverse section just above the aortic
beam is almost at right angles to the septmn. No color origin, and crossing over it. The pulmonary ar-
is seen breaching the septum indicating it is intact. tery, and its c o n n e c t i o n to the arterial duct, lie
in an almost straight antero-posterior line,
fects in the m u s c u l a r septum, can be over- slightly to the left o f the midline. In the trans-
l o o k e d particularly in early p r e g n a n c y when verse section (Fig 5), this view is known as the
they may be below the resolution of the ultra- "three vessel view" as it shows the p u l m o n a r y
s o u n d machine. artery in a long axis projection, with the aorta

Figure 4. (A) A diagramatic representation of the transducer beam is shown as it is swept though tile heart from
the four chamber view successively to the aortic (AoV) and pulmonary valves. The most superior slice shows the
aorta forming the arch just above the pulmonary artery and duct. 4oh, four chamber; 3V view, three vessel view.
(B) Moving cranially from the four-chamber view allows the aortic origin from the left ventricle to be imaged.
Angling slightly between the apex and the right shoulder helps to "open out" this view.
328 Lindsey D. Allan

Figure 5. Just above the aortic origin, the pulmonary

connection to the duct is shown in the three vessel Figure 7. The transverse arch crossing the midline of
view. Normally the pulmonary artery is the largest, the thorax in front of the spine and meet with the
most anterior and leftwards of the three vessels, with duct (D), which lies to the left and below the arch.
the'superior vena cava (SVC) the smallest, most pos-
terior and rightwards. The ascending aorta lies be-
tween these two vessels. I n t h e l o n g i t u d i n a l sections, t h e r i g h t v e n t r i c -
u l a r o u t f l o w tract c a n b e s e e n a r c h i n g o v e r t h e
left v e n t r i c l e or, j u s t to t h e r i g h t o f this view,
a n d s u p e r i o r v e n a cava lying to t h e r i g h t o f it. ~3
a r c h i n g over t h e a o r t a c u t in short-axis (Fig 6).
T h e p u l m o n a r y a r t e r y is t h e largest, m o s t a n t e -
W h a t e v e r views o f t h e g r e a t a r t e r i e s a r e s e e n , t h e
r i o r a n d leftwards o f t h e t h r e e vessels with t h e
p o i n t s to b e n o t e d i n c l u d e : (1) T h e n o r m a l
s u p e r i o r v e n a cava t h e smallest, m o s t p o s t e r i o r
cross-over o f t h e g r e a t arteries. (2) T h e n o r m a l
a n d r i g h t wards. T h e a o r t a is in b e t w e e n b o t h in
size r e l a t i o n s h i p o f t h e 2 vessels. (3) N o r m a l
size a n d p o s i t i o n .

Figure 6. The arterial duct (D) in a long axis view of Figure 8. The characteristic "hook" shape of the aor-
the fetus. The aorta (Ao) lies in the center of this scan tic arch with the head and neck vessels arising from
plane. the superior aspect of the arch.
A Practical Approach to Fetal Heart Scanning 329

unaliased color flow m a p p i n g across each arte- Comments

rial valve with no regurgitation. Normally, the
p u l m o n a r y artery is slightly larger than the aorta T h e accuracy of all ultrasound is d e p e n d e n t on
in size. If there is a suggestion of discrepancy in o p e r a t o r knowledge a n d e x p e r i e n c e and the im-
the great artery sizes, they should be m e a s u r e d age quality obtained. T h e image quality in turn
a n d c o m p a r e d to n o r m a l ranges. 6 Calculation of is d e p e n d e n t on the c o m b i n a t i o n of o p e r a t o r
the A o / P A ratio is often useful, as some degree skill and experience, m a c h i n e resolution, the
of outflow tract obstruction or an arch anomaly thickness o f the m a t e r n a l a b d o m e n , fetal posi-
may be present where this is abnormal. 1~ T h e tion and gestational age. By 18 weeks of gesta-
tion, the f o u r - c h a m b e r view a n d the two outflow
arterial valves must be interrogated by either
tracts can be i m a g e d transabdominally in t h e
pulsed or color Doppler, obtained as close as
vast majority of patients by an e x p e r i e n c e d
possible to parallel to the direction of b l o o d
s o n o g r a p h e r with m o d e r n equipment. In obese
flow. Color flow m a p p i n g is m u c h quicker a n d
patients, or in late pregnancy, a limited study
easier. If there is unaliased, n o n t u r b u l e n t flow
may have to be a c c e p t e d after a range of trans-
across the arterial origin this indicates b l o o d
ducer frequencies a n d aids such as h a r m o n i c
flow at a n o r m a l velocity. However, if there is imaging have b e e n tried. However, even where a
aliasing of the color signal, either because of the study is o p t i m u m in quality, there are confi-
d e p t h of the fetus or the transducer frequency, dence limits to fetal h e a r t scanning. Small ven-
or because of an abnormally high velocity, tricular septal defects will be overlooked and
pulsed D o p p l e r must be used to obtain the exact lesions such as aortic or p u l m o n a r y stenosis, car-
velocity across the valve in question. T h e velocity diomyopathies a n d cardiac tumors may evolve in
of flow should not exceed 1 m / s e c across nor- later pregnancy, t5 In addition, the atrial septum
mal arterial valves. and the arterial duct are always patent prenatally
and persistent patency after birth, which are
T h e Arch and Duct Are N o r m a l b o t h forms of congenital h e a r t disease, albeit
minor, c a n n o t be predicted f r o m the fetal study.
Also, the p o o r e r the image quality, the wider the
T h e arch and duct can be imaged in either
confidence limits. Although the technique of
transverse or longitudinal views but all views are
fetal h e a r t scanning appears c o m p l e x at first
n o t essential to image in every n o r m a l fetus. T h e
sight, b r o k e n down into the above c o m p o n e n t s
transverse aortic arch is seen immediately above
and in the hands of a motivated operator, a
the three vessel view as a curved vessel arising in
t h o r o u g h assessment can usually be accom-
the middle of the thorax and crossing the mid-
plished in minutes. 16 In a fetus in an ideal posi-
line in front of the spine and trachea. This con-
tion, sweeping f r o m the a b d o m e n to the inlet of
firms a normal left arch. By slight transducer the thorax and down again by using color flow
angulation in the n o r m a l fetus, the aortic arch mapping, provides comprehensive information
a n d duct can be imaged simultaneously. This providing p r o o f of normality. If the fetal posi-
allows the arch to be seen to the right o f the tion or imaging is difficult, as long as the essen-
duct. T h e p u l m o n a r y artery branches laterally tials listed above are seen, m a j o r anomalies will
into the right p u l m o n a r y artery a n d duct. T h e rarely be overlooked.
aortic arch and duct should be similar in size
and join just in front and to the left of the spine
(Fig 7). In the long axis views, the duct can be
seen just to the left of, and below, the aortic
arch. The aortic arch forms a tight "hook" shape 1. Allan LD, Crawford DC, Chita SK, et ah Prenatal screen-
a n d gives off 3 cranial branch~es (Fig 8). T h e ing for congenital heart disease. Br MedJ 292:1717-1719,
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show no sites of turbulence. M e a s u r e m e n t s of
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vessel or c h a m b e r sizes are useful w h e n they outcomes of prenatal diagnosis of congenital cardiac
a p p e a r a b n o r m a l but are not required routinely. malformations by fetal echocardiography in a well de-
330 Lindsey D. Allan

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