Journal of Neonatology Vol. 22, No.
3, July - September 2008
REVIEW ARTICLE
Fetal echocardiography
Sejal Shah, Sunita Maheshwari
Department of Pediatric Cardiology, Narayana Hrudayalaya, Bangalore
email: sejalshahsuresh@yahoo.com
Abstract
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Fetal echocardiography provides an insight not only
into the cardiac problems during antenatal period,
but also the non-cardiac issues which could make a
major impact on the management of the baby. It has
an important position in fetal medicine in the current
era when we are expecting major improvements in
the field of fetal cardiac interventions.
Introduction
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The fetal echocardiogram provides an insight into
the fetal cardiovascular system. The antepartum
obstetrical ultrasound has become a protocol and is
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commonly used for deciding the well-being of the fetus
and ruling out anomalies. With increase in the
awareness and detection rate of congenital anomalies,
the need for fetal echocardiography has also grown
over years. The fetal echocardiogram is unique as it
differs from both the antenatal ultrasound and the
pediatric echocardiogram.
Since the first experience of fetal echocardiography
(echo) by Winsberg in 1972 1, considerable advances
have been made in the ultrasound technology. This
has increased our ability to diagnose congenital heart
disease before birth and includes both structural and
functional assessment of the heart. This article provides
an overview of the applications of fetal
echocardiography in the current era.
Burden of congenital heart disease
Congenital heart disease is the most common
congenital anomaly found in the human2. The incidence
of congenital heart disease is generally estimated to be
3-12 in 1000 live births3,4, which is 6.5 times that of
chromosomal abnormalities and 4 times that of neural
tube defects5. Out of this, only 50% are minor defects.
The remainder have a significant disease requiring
intervention early in life 4, 6 and account for 30% of
perinatal deaths and 50% of lethal malformations.
Overall, congenital heart disease has high morbidity and
mortality, especially when not corrected in time. Hence,
detection of congenital heart disease during antenatal
period helps us in using a “team-approach” for
managing the “mother and baby” duo, so that a positive
controlled outcome is obtained.
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Clinical situations where fetal echocardio-
graphy plays a role
The knowledge of the presence of a “heart defect”
during pregnancy allows time for thorough counseling
and allows the family to make an informed decision
regarding continuation or termination of pregnancy.
It also helps the medical team to prepare for postnatal
cardiac interventions including cardiac surgery by
preferably delivering the baby in a tertiary care center
and providing a team approach involving the
obstetrician, neonatologist, pediatric cardiologist and
pediatric cardiac surgeon. It helps keeping patients in
a better preoperative condition by preventing
situations like “ductal shock” by giving prostaglandins
7
. A comprehensive evaluation of the fetal
cardiovascular system may aid management decisions
like need for early delivery, modify the mode of delivery,
medical therapy especially for arrhythmias or for
possible invasive intrauterine interventions like balloon
valvuloplasty 8. To summarize, fetal echocardiography
ensures the best possible transition from the pre to
post natal state with an opportunity to provide
immediate care to the baby before hemodynamic
compromise sets in.
Role of fetal echo as a diagnostic versus
screening test
The relative indications for a detailed fetal
echocardiogram are enlisted in Table I. This includes
“high-risk” cases either defined by maternal or fetal
factors. If confined to traditional high-risk groups, only
20% of babies with CHD will be identified. Hence, the
value of a screening test is outlined by the fact that
most congenital anomalies are found among
newborns from pregnancies with no risk factors.
Fetal factors
The incidence of congenital heart disease in presence
of chromosomal abnormalities is generally thought to
be 30-50%9 whereas in presence of CHD, the risk for a
chromosomal abnormality is 5-13%.
In presence of an extracardiac abnormality, the risk
of having CHD is 25-45%, however the actual incidence
varies depending on the organ involved. It is 2-5%
with CNS malformations and 50% with renal
 Journal of Neonatology
anomalies10.
The presence of fetal arrhythmias increases the
incidence of fetal distress during labor and increases
mortality. Persistent fetal arrhythmias occur in 1-2 %
of all pregnancies11. Extrasystoles account for 85% of
fetal arrhythmias and are usually benign 11.
Most tachycardias in fetus are atrial in origin and
occur in fetuses without structural heart disease.
Cardiac tumors, Ebstein’s anomaly of the tricupid valve
and myocarditis need to be ruled out in presence of
tachyarrhythmias. Indications for treatment of fetal
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tachycardia include sustained tachycardia or presence
of hydrops as there is a 20-50% risk of intrauterine
death 12.
Amongst bradyarrhythmias, complete heart block
has 40% incidence of associated congenital heart
disease, most often a complete atrioventricular septal
defect or corrected transposition of great arteries. It
is important to check for maternal antibody status
(anti-Rho and –SSA) in presence of fetal complete heart
block to counsel the mother for recurrence risk in
future pregnancies. Maternal steroids, ionotropes and
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fetal pacing have been tried with no clear beneficial
results13,14.
CHD may present with evidence of intrauterine
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growth retardation and may be a cause for non
immune fetal hydrops. Intrauterine congestive cardiac
failure or impending failure may manifest with fetal
distress. Fetal distress could also be secondary to
tachyarrhythmias, foramen ovale closure or
myocarditis.
Maternal factors
If parents have one previously affected child, the
occurrence rate of CHD in the present pregnancy is 1
in 52. If parents have two previously affected children,
the occurrence rate is 1 in 10 5. The occurrence risk in
offspring of patients with congenital heart disease is
16% 15, however, the actual risk will vary depending
on the type of CHD and the parent affected.
When non steroidal anti-inflammatory agents or B
agonist sympathomimetics are being used to treat
preterm labor, it can produce ductal constriction,
progressive right ventricular overload and hydrops
fetalis. This acute and transient change in the caliber
of fetal ductus arteriosus and the effect on fetal
circulation can be studied using fetal echo.
Recently, a 3-fold increase in the prevalence of
congenital heart disease over general population has
been reported in infants born via intracytoplasmic
sperm injection and in vitro fertilization techniques 16.
USG factors
Nuchal translucency (NT) in the fetus refers to the
normal subcutaneous fluid filled space between the
back of the neck and the skin. The finding of increased
nuchal translucency may indicate a need for testing
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Vol. 22, No.3, July - September 2008
for chromosomal aneuploidy. Increased nuchal
translucency when measured between 10 to 14 weeks
of gestation is associated with increased risk for
congenital heart diseases in presence of aneuploidy 17-
19
and also independent of the karyotype 20. FISH
analysis is indicated for micro deletion when congenital
heart disease is detected in the presence of increased
nuchal translucency.
Fetal echo in addition to a detailed sonographic
evaluation is recommended in cases of single umbilical
artery as associated cardiac anomalies are frequently
observed. Presence of second pertinent sonographic
abnormality in addition to single umbilical artery is an
indication for fetal karyotyping 21.
Presence of tricuspid regurgitation early in gestation
during fetal life could also be an indicator of congenital
heart disease like atrioventricular septal defect,
Ebstein’s anomaly of the tricuspid valve, pulmonary
atresia with intact ventricular septum, premature
ductal closure or arrhythmias. It is also considered as a
marker for chromosomal defects even in absence of
structural heart disease 22. However, a recent study
conducted by Messing et al states that mild tricuspid
regurgitation in early / mid gestation is a benign finding
and may reflect a physiological change 23.
An ideal time for fetal echocardiography
Though fetal echo can be performed at any time
through out pregnancy, an initial evaluation early in
pregnancy may help in terms of decision making. There
are certain limitations for adequate visualization of
cardiac structures very early in pregnancy. Hence, ideally
the first imaging should be performed by 18-20 weeks
of gestation. Conditions which are easily detected
during early gestation are those with a large septal
defect, atretic atrio ventricular valve, great arteries
“not crossing” indicating malpostion of great arteries
or single great artery.
At the same time, it is important to realize that
there are certain lesions which may evolve in utero or
progress through out gestation making it important
to perform the study late in gestation also.
Hypertrophic cardiomyopathy is usually evident near
term and never early in gestation. Progressive stenosis
of an apparently normal semilunar valve, development
of severe lesions like pulmonary atresia / intact
ventricular septum 24 or hypoplastic left heart syndrome
later in pregnancy must be kept in mind while doing
the initial study. Hence, normal fetal echo in second
trimester does not ensure absence of serious cardiac
lesions later in gestation. Interestingly, some lesions
like ventricular septal defect and atrial septal aneurysm
are known to resolve in utero.
Since 1990, several reports have described the use
of early fetal echocardiography for detection of
anomalies. McAuliffe FM has shown early
 Journal of Neonatology Vol. 22, No.3, July - September 2008

echocardiography before 16 weeks to be feasible with

a sensitivity of 70% and has recommended it in high
risk cases with increased NT and presence of
extracardiac lesions 25 . Bebbington M et al also
recommends early fetal echo in fetuses at risk, but
emphasizes on the need for an additional routine
screening in the second trimester.26 Evaluation of the
fetal heart prior to 16 weeks also enables one to check
for associated chromosomal and extracardiac
conditions early in gestation.
The greatest challenge in performing a fetal echo
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is the constant movement of the baby with the small

size and dynamic nature of fetal heart. However, as
lungs are not inflated and bones are not ossified early
in gestation, it may be possible to image heart in planes
otherwise not possible after birth. Later in gestation,
Figure 1. Four chamber view on 2D echocardiogram showing
the ossification of the spine and ribs may make
both the atria and both the ventricles. Note that all the four
examination difficult. Maternal obesity and oligo /
chambers are good sized. (RV=right ventricle, LV=left
polyhydramnios may produce technical limitations
ventricle). The interventricular septum appears intact.
making it difficult to gather adequate information
during fetal echo.
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• 6 weeks = Motion of fetal heart observed

• 16 weeks = Structural analysis possible
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• 18-24 weeks = Heart easily imaged

• 3rd trimester = visualisation difficult due to
calcified ribs/spine

Steps while performing comprehensive fetal

echocardiography

A normal fetal heart occupies about 1/3rd of the

thorax. The axis of the heart is 45 o to sternal spine
axis.
1. Determination of right and left side of the baby Figure 2. Four chamber view showing the foramen ovale
2. Determination of the visceral and atrial situs with flap into the left atrium (LA = left atrium)
3. Determination of position of the heart in the
chest and position of the apex of the heart
4. Visualization of 4 chambers (Figure 1)
5. Demonstration of foramen ovale with flap
moving in left atrium (Figure 2)
6. Visualization of drainage of the systemic veins
and atleast two pulmonary veins
7. Identification of morphological right and left
ventricles and assuring atrio ventricular
concordance
8. Visualization of atrio ventricular valves
9. Visualization of crux of the heart
10. Demonstration of interventricular septum
11. Visualization of left and right outflow tracts and
assuring ventriculoarterial concordance (Figure
3 & 4)
12. Make sure great arteries are crossing each other
13. Ensure pulmonary artery / aorta ratio is
Figure 3. Long axis view showing the left ventricle with the
acceptable (mean value 1.09, range 0.75 – 1.43)
inflow and outflow.

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 Journal of Neonatology Vol. 22, No.3, July - September 2008
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Figure 4. Right ventricular outflow tract is seen. Note that left Figure 5. Bifurcation of branch pulmonary arteries seen.
ventricle is on the same side of the stomach bubble. (RPA = right pulmonary artery, LPA = left pulmonary artery)

14. See for bifurcation of main pulmonary artery

into branches (Figure 5) The obstetric sonographer’s cardiac screen is also
15. Demonstration of ductal and aortic arches advisable to be an “extended” cardiac screen which is
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(Figure 6) composed of the four chamber view, the left and right
16. Arrhythmia evaluation by Doppler / M mode of ventricular outflow tract and the main pulmonary
atrial and ventricular activity artery with its branches. Achiron et al found an increase
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17. Dopplers of ductus arteriosus, ductus venosus, in the sensitivity from 48% with the four chamber view
umbilical arteries near the fetal end and placental alone to 78% with the extended screen in low risk
end, right and left uterine arteries pregnancies between 18 and 24 weeks of gestation.27

Table 1. Indications for fetal echocardiography

High risk population
Fetal factors Maternal factors USG factors
Extracardiac anomalies Family history of CHD Increased NT
Chromosomal abnormalities Maternal diseases Single umbilical artery
Multiple fetuses Diabetes Mellitus Suspicious 4 chamber scan
Cardiac arryhythmias in fetus Phenylketonuria Non diagnostic scan
Abnormal fetal growth Collagen disorders "Soft diagnostic markers"
Fetal distress Teratogen exposures Echogenic cardiac foci
Alcohol Short femur
Anticonvulsants Echogenic bowel
Lithium Symmetric growth restriction
Progesterones
Exposure to PG synthetase inhibitors
Ibuprofen
Salicylic acid
Indomethacin
Maternal infection
Rubella
Familial inherited disorders
Ellis Van Crevald syndrome
Marfan syndrome
Noonan's syndrome
IVF
(CHD= congenital heart disease, NT = nuchal translucency, USG= ultrasonography, PG= prostraglandin, IVF=
In vitro fertilization)

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 Journal of Neonatology Vol. 22, No.3, July - September 2008

documented 28 while performing the study using

current commercial instrumentation, a threshold for
bioeffects has not been determined. Hence France et
al has advised a responsible approach by controlling
the output level and exposure time during the
procedure.

Conclusion

It is important to treat the fetus and not only the

heart problem after doing fetal echo. Associated
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chromosomal and extracardiac anomalies need to be

considered while making the final decision. It is
imperative to provide a true picture to the expectant
parents about the overall prognosis and then counsel
them. It is crucial then to coordinate with the
Figure 6. Aortic arch with arch vessels visualized obstetrician and the neonatologist for proper pre and
postnatal management. Serial studies may be needed
Limitations of fetal echo: Impact of fetal to note a change in the disease or watch for
circulation development of hydrops or monitor the efficacy of
medical therapy, all of which could alter the final
Fetal echocardiography is different from pediatric management strategies and time / mode of delivery.
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echocardiography as the spectrum of diseases are Though fetal echocardiography is an investigational

different due to the feto-placental circulation. modality, its role is not limited to only detection of
1. The right ventricle takes care of 2/3 rd of the cardiac defects prior to birth. In addition to giving
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heart’s work. Hence, the right ventricle is slightly insight into the pathophysiology of fetal circulation,
larger, thicker and contributes to an increased it gives an improved understanding of additional
amount of flow into the pulmonary artery anomalies. Adequate counselling at this stage helps
relative to aorta. Allan et al found that both improve the psychological state of the parents and
ventricles are fairly equal in size in early gestation improve their coping skills in facing the birth of the
with right ventricle becoming slightly more child with a “heart” problem. With the improvement
dominant in later gestation 5. Hence, the left in the ultrasound technology and close collaboration
side of the heart may appear slightly smaller than across different specialities, we can expect major
the right side. How small is “abnormal” needs improvements in the field of fetal cardiology including
to be defined. fetal cardiac interventions in situations like semilunar
2. As the pulmonary blood flow is reduced, the valve stenosis or atresia and transposition of great
pulmonary venous return is also reduced. Hence, arteries or mitral atresia with restriction of foramen
lesions like partial anomalous pulmonary venous ovale.
return and mitral stenosis may be missed on fetal
echo. References
3. Flow across the ascending aorta is only 10% of
the cardiac output. Hence, isthmic narrowing 1. Winsberg F. Echocardiography of the fetal and newborn
(coarctation) may not be fully appreciated. heart. Invest Radio 1972;3:152.
4. Assessment of the ventricles for suitability for 2. Hoffman JI, Kaplan S. The incidence of congenital heart
future biventricular repair may be difficult. disease. J Am Coll Cardiol 2002;39:1890-1900.
5. Mild valvar stenosis may be missed 3. Hoffman JIE. Incidence of congenital heart disease: I. Post
6. Small ventricular septal defects may not be seen natal incidence. Pediatr Cardio 1995;16:103-113.
due to equalisation of both ventricular 4. Hoffman JI, Christianson R. Congenital heart disease in a
cohort of 19,502 births with long-term follow-up. Am J
pressures.
Cardiol. 1978;42:641-647.
7. Impossible to predict if secundum atrial septal 5. Allan L, Sharland G, Milburn A, Lockhart SM, Groves AM,
defect and patent ductus arteriosus will persist Anderson RH et al. Prospective diagnosis of 1,006
after birth consecutive cases of congenital heart disease on the fetus.
8. Coronary anomalies are difficult to diagnose J Am Coll Cardiol 1994;23:1452.
6. Report of the New England Regional Cardiac Program.
Consideration of bioeffects while performing fetal Pediatrics 1980; 65: 375-461.
echo 7. Satomi G, Yasukochi S, Shimizu T, Takigiku K, Ishii T. Has
fetal echocardiography improved the progosis of congenital
Though no permanent injury has ever been heart disease? Comparision of patients with hypoplastic

158
 Journal of Neonatology
left heart syndrome with ad without prenatal diagnosis.
Pediatr Int 1999; 41:728-732.
8. Tworetzky W, Wilkins-Haug L, Jennings RW, van der Velde
ME, Marshall AC, Marx GR et al. Balloon dilation of severe
aortic stenosis in the fetus: potential for prevention of
hypoplastic left heart syndrome: candidate selection,
technique, and results of successful intervention. Circulation
2004;110:2125-2131.
9. Copel JA et al. The frequency of aneuploidy in prenatally
diagnosed congenital heart disease: an indication for fetal
karyotyping. Am J Obstet Gynecol 1988; 158: 409-413.
10. Copel JA, Gianluigi P, Kleinman CS. Congenital heart disease
http:\\www.IndianJournals.com
and extracardiac anomalies: Association and indication for
fetal echocardiography. Am J Obstet Gynecol 1986;154:
1121-1132.
11. Reed KL: Fetal arrhthmias: etiology, diagnosis,
pathophysiology and treatment. Semin Perinatol 1989;13:
294-304.
12. Allan LD, Chita SK, Sharland GK et al. Flecainide in the
treatment of fetal tachycardias. Br Heart J 1991;65: 46-48.
13. Groves AM, Allan LD, Rosenthal E. Outcome of isolated
congenital complete heart block diagnosed in utero. Heart
1996;75:190-194.
14. Walkinshaw SA, Welch CR, McCormack J et al. In utero
Downloaded From IP - 117.195.225.56 on dated 5-Feb-2010
pacing for fetal congenital heart block. Fetal Diagn Ther
1994; 9:183-185.
15. Whittemore B, Hobbins JC, Engle MA. Pregnancy and its
outcome in women with and without surgical treatment of
A product of Divan Enterprises
congenital heart disease. Am J Cardiol 1982; 50: 641-651.
16. Hansen M, Kurinczuk JJ, Bower C, Webb S. The risk of major
birth defects after intracytoplasmic sperm injection and in
vitro fertilization. N Engl J Med 2002; 346: 725-730.
17. Hyett J, Moscosco G, Nicolaides KH. Cardiac defects in 1st
trimester fetus with trisomy 18. Fetal Diagn Ther 1995;
10:381-386.
18. Zosmer N, Souter VL, Chan CS, Huggon IC, Nicolaides KH.
Early diagnosis of major cardiac defects in chromosomally
normal fetuses with increased nuchal translucency. Br J
Obstet Gynaecol 1999; 106: 829-833.
19. Bahado-Singh RO, Wapner R, Thom E, Zachary J, Platt L and
Mahoney MJ et al; First Trimester Maternal Serum
159
Vol. 22, No.3, July - September 2008
Biochemistry and Fetal Nuchal Translucency Screening Study
Group. Elevated first-trimester nuchal translucency
increases the risk of congenital heart defects. Am J Obstet
Gynecol 2005; 192:1357-1361.
20. Hyett J, Moscoso G, Pappanagiotou G, Perdu M, Nicolaides
KH. Abnormalities of the heart and great arteries in
chromosomally normal fetuses with increased nuchal
translucency thickness at 11-13 weeks of gestation.
Ultrasound Obstet Gynecol 1996;7: 245-250.
21. Geipel A, Germer U, Welp T, Schwinger E, Gembruch U.
Prenatal diagnosis of single umbilical artery: determination
of the absent side, associated anomalies, Doppler findings
and perinatal outcome. Ultrasound Obstet Gynecol
2000;15:114-117.
22. Huggon IC, Defigueiredo DB, Allan LD. Tricuspid
regurgitation in the diagnosis of chromosomal anomalies
in the fetus at 11-14 weeks of gestation. Heart 2003; 89:
1071-1073.
23. Messing B, Porat S, Imbar T, Valsky DV, Anteby EY, Yagel S.
Mild tricuspid regurgitation: a benign fetal finding at
various stages of pregnancy. Ultrasound Obstet Gynaecol
2005; 26: 606-610.
24. 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: 355-360.
25. McAuliffe FM, Trines J, Nield LE, Chitayat D, Jaeggi E,
Hornberger LK. Early fetal echocardiography-A reliable
prenatal diagnosis tool. Am J of Obstet and Gynecol 2005;
193: 1253-1259.
26. Bebbington M, Wilson RD, Johnson MP. Detection of
congenital heart disease in the first trimester of pregnancy.
Progress in Pediatric Cardiology 2006; 22: 3-8.
27. Achiron R, Glaser J, Gelernter I, Hegesh J, Yagel S. Extended
fetal echocardiographic examination for detecting cardiac
malformations in low risk pregnancies. Br Med J 1992;
304:671-674.
28. Abramowicz JS, Kossoff G, Marsal K, Ter Haar G. Literature
review by the ISUOG bioeffects and safety committee.
Ultrasound Obstet Gynecol 2002; 19: 318-319.