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ORIGINAL RESEARCH
Prenatal Diagnosis of Prevalence of the
Right Heart
Associated Anomalies and Outcome Predictors
Ruan Peng, MD, Hong-Ning Xie, PhD, Yun-Xiao Zhu, PhD, Li-Juan Li, MD, Liu Du, MD, Ju Zheng, PhD
Article includes CME test
Received July 11, 2013, Department of Ultrasonic
Medicine, Fetal Medical Center, First Affiliated
Hospital of Sun Yat-sen University, Guangzhou,
China. Revision requested August 20, 2013.
Revised manuscript accepted for publication
November 6, 2013.
This study was supported by research grant
81071166 from the National Scientific Foundation
Committee of China.
Address correspondence to Hong-Ning Xie,
PhD, Department of Ultrasonic Medicine, Fetal
Medical Center, First Affiliated Hospital of Sun
Yat-sen University, 58 Zhongshan Er Rd, 510080
Guangzhou, Guangdong, China.
E-mail: hongning_x@126.com
Abbreviations
LA, left atrium; LV, left ventricle; RA, right
atrium; RV, right ventricle; 2D, 2-dimensional
doi:10.7863/ultra.33.7.1155
©2014 by the American Institute of Ultrasound in Medicine | J Ultrasound Med 2014; 33:1155–1161 | 0278-4297 | www.aium.org
Objectives—To analyze the characteristics, associations, and outcomes of prevalence of
the right heart and to evaluate the use of sonography to predict the risk of a poor prog-
nosis in fetuses with this condition.
Methods—We conducted a retrospective cohort study of 182 fetuses with prevalence
of the right heart. At the initial evaluation, the left ventricle (LV) and right ventricle
(RV) sizes, left atrium and right atrium sizes, great artery diameters, appearance of
the aortic arch and ductus arteriosus arch, and flow direction across the foramen ovale
and aortic arch were documented. Malformations were documented in fetuses with
intracardiac anomalies, who were divided into groups with and without coexisting
extracardiac defects. The RV-LV diameter ratio and main pulmonary artery-to-aortic
root diameter ratio were calculated and compared. The following variables were ana-
lyzed: coexisting intracardiac anomalies, associated extracardiac anomalies, diagnosis
before 24 gestational weeks, fetal growth restriction, reversed flow across the foramen
ovale, reversed flow in the aortic arch, and RV-LV ratio greater than 2.0. Relationships
between these sonographic findings and the prognosis were evaluated by multivariable
logistic regression.
Results—Of the 182 fetuses, 1 (0.5%) had intrauterine death, and 25 (13.7%) had
neonatal death; 1 (0.5%) had selective reduction, and 106 (58.2%) underwent termina-
tion of pregnancy; 49 (26.9%) survived at this writing. The mean RV-LV ratio was 1.67.
Multivariable logistic regression revealed that only 2 sonographic parameters, coexisting
intracardiac anomalies (odds ratio, 17.75; 95% confidence interval, 4.18–75.26) and
diagnosis before 24 weeks (odds ratio, 17.26; 95% confidence interval, 1.80–165.39)
were significantly associated with a poor prognosis.
Conclusions—The sonographic parameters of coexisting intracardiac anomalies and
diagnosis before 24 gestational weeks are significant independent predictors of a poor
prognosis in fetuses with prevalence of the right heart. Combinations of these risk factors
may be useful in prenatal consultation.
Key Words—congenital heart disease; obstetric ultrasound; outcome; prediction;
prevalence of the right heart
I n healthy fetuses, the two ventricles are approximately sim-
ilar in diameter, with mild prevalence of the right one. Dif-
ferent studies have reported that prevalence of the right heart
increases with advancing gestational age.1,2 Prevalence of the right
heart may be completely normal or may be caused by extracardiac
anomalies, such as hydrops fetalis, a vein of Galen aneurysm,3,4 and
twin-twin transfusion syndrome. Moreover, prevalence of the right
heart is considered an important indirect sign of aortic coarctation5
and a total anomalous pulmonary venous connection,6 and it has
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Peng et al—Prenatal Diagnosis of Prevalence of the Right Heart
also been reported with several congenital heart diseases,
particularly an unbalanced atrioventricular septal defect7
and a persistent left superior vena cava. Several studies have
reported reference ranges for the diameters of the left
ventricle (LV) and right ventricle (RV), and that the diam-
eters have been shown to increase significantly as gesta-
tional age advances,1,2,8,9 whereas the RV-LV diameter ratio
only changes slightly during the gestational period.10–12
Unfortunately, it may be impossible to exclude congenital
cardiac abnormalities categorically, as the right heart struc-
tures may appear larger than the left in a healthy fetus during
late pregnancy.13
On the other hand, although the association between
aortic coarctation and prevalence of the right heart has
been described,10 the literature contains limited discussions
of sonographic prognostic factors,12,13 which could be used
to predict perinatal outcomes of fetuses with prevalence
of the right heart. Prognoses of this condition may be
influenced by coexisting intracardiac defects. In addition,
prevalence of the right heart is progressive prenatally, so the
prognosis may be influenced by the gestational weeks at
which it is suspected. Combining size-based cardiac param-
eters with the gestational age at diagnosis may improve the
accuracy of fetal echocardiography for predicting the out-
come of this condition. The objectives of this study were to
analyze the characteristics, associations, and outcomes of
prevalence of the right heart and to evaluate the prognostic
impact of these characteristics on survival.
Materials and Methods
This study was conducted at a single tertiary center for
prenatal care between January 2008 and June 2012.
Two-dimensional (2D) fetal echocardiography was per-
formed in all cases with a convex transabdominal 4–8-MHz
transducer and an ultrasound system equipped with pulsed,
continuous, and color Doppler capacities (Voluson 730;
GE Healthcare, Zipf, Austria). The fetuses were suspected
to have prevalence of the right heart on 2D echocardiog-
raphy, and then after the 2D examination, cardiac volume
data sets were acquired with an original plane from the apical
4-chamber view. The volume data sets were stored for
analysis. If echocardiographic reports were inconclusive,
images and spatiotemporal image correlation volume data
were reviewed again. Written informed consent was
obtained from all patients, and the study was approved by
the Ethical Committee of the institution. Each fetus was
considered only once in this study.
Fetal sonographic examinations included a detailed
extracardiac structural survey and complete echocar-
1156
diography. The diameters of the aortic root and main pul-
monary artery, maximum width of the LV and RV, and
maximum width of the left atrium (LA) and right atrium
(RA) were measured and compared with normal reference
ranges.1 We measured the maximum width of the LV and
RV on a transverse 4-chamber view of the fetal heart just
below the atrioventricular valve orifice and made meas-
urements at the end of diastole when the atrioventricular
valves were closed but before the onset of systole. In all
cases, measurements of the arteries were made when the
walls of the arteries were perpendicular to the ultrasound
beam. The internal diameters of the vessels were measured in
diastole when the arterial valves were closed. The diameters
of the aortic root were measured using only longitudinal
views, and the main pulmonary artery was measured in
either an oblique short-axis view or an oblique transverse
plane of the fetal thorax, giving a longitudinal view of the
main pulmonary artery and its bifurcation. With a frozen
frame from a real-time image, the diameters were measured.
If the real-time 2D images were not optimal for measure-
ment, the measurements also could be made with volume
data sets. Following our usual practice in fetal echocardio-
graphy, at least 3 measurements of each dimension were
taken from separate frames, and the mean value was used.
Fetal karyotyping was recommended for all cases. In
our institution, invasive testing for karyotyping is always
offered in cases with findings suspicious of chromosomal
anomalies, including congenital heart defects and multiple
soft markers, and in cases at increased risk after the first-
trimester screening. Detailed postnatal examination, sur-
gery, or autopsy findings were obtained in almost all cases.
The cases with intracardiac anomalies were divided into
2 groups: intracardiac defects only and intracardiac defects
with coexisting extracardiac defects. The associated anom-
alies and the outcomes were documented and compared.
The RV-LV diameter ratio and main pulmonary artery-to-
aortic root diameter ratio were calculated. Prevalence of
the right heart was defined as an RV-LV ratio greater than
1.20.12 Comparisons of the RV-LV ratios were performed
between different groups. A multivariable logistic regression
model for the outcome of prevalence of the right heart was
constructed using variables selected by stepwise selection
(significance level for entering into the model: P < .2).
Variables were as follows: coexisting intracardiac anomalies,
associated extracardiac anomalies, diagnosis before 24 ges-
tational weeks, fetal growth restriction, reversed flow across
the foramen ovale, reversed flow in the aortic arch, and RV-
LV ratio greater than 2.0.
The results of the first examinations were included in
the final analysis. Statistical analyses were performed with
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Peng et al—Prenatal Diagnosis of Prevalence of the Right Heart

SPSS version 16.0 software (IBM Corporation, Armonk,
NY). The RV-LV ratios in both outcome groups are shown
in box-and-whisker plots. The Mann-Whitney U test was
used to compare the RV-LV and pulmonary artery-to-
aorta ratios between groups. Multivariable logistic regres-
sion was used to evaluate the impact of different binary
variables on survival; P < .05 was considered significant.
Significance was also expressed by odds ratios and 95%
confidence intervals.
Results
in these 182 cases are shown in Tables 1 and 2. An abnor-
mal karyotype was present in 26 cases, including 6 fetuses
with trisomy 21, 3 with trisomy 13, 9 with trisomy 18,
3 with 45,XO, and 5 with others. This study also included
38 karyotypically normal live-born fetuses and 37 fetuses
with normal karyotypes. The 81 remaining cases did not
undergo fetal karyotyping.
Table 1. Associated Cardiac Defects in Fetuses With Prevalence of
the Right Heart
Defect n (%)

Ventricular septal defect 38 (20.9)

Between January 2008 and June 2012, a total of 27,035 Hypoplastic left heart syndrome 30 (16.5)
fetuses underwent comprehensive sonographic examina- Persistent left superior vena cava 30 (16.5)
tions and fetal echocardiography. Prevalence of the right Aortic coarctation 26 (14.3)
heart was identified in 223 fetuses. Eleven of the original Double-outlet RV 25 (13.7)
Tricuspid regurgitation 22 (12.1)
223 fetuses were lost to follow-up. The cardiac structures Pulmonary stenosis 18 (9.9)
could not be clearly identified because of early gestational Restrictive foramen ovale 9 (4.9)
age in 3 fetuses. Six cases with prevalence of the right heart Aortic stenosis/atresia 8 (4.4)
were excluded for twin-twin transfusion syndrome in Mitral valve stenosis/atresia 8 (4.4)
monochorionic twins, and 3 cases were excluded for con- Ebstein anomaly 7 (3.8)
Endocardial cushion defect 6 (3.3)
genital diaphragmatic hernias. Necropsy or surgical con- Absent pulmonary valve syndrome 5 (2.7)
firmation was not available in 18 cases. The remaining 182 Right-sided aortic arch 5 (2.7)
fetuses with prevalence of the right heart represented the Aberrant right subclavian artery 4 (2.2)
study population. The mean gestational age at the time of Anomalous pulmonary venous connection 4 (2.2)
diagnosis of prevalence of the right heart was 26 weeks Truncus arteriosus communis 3 (1.6)
Tetralogy of Fallot 3 (1.6)
(range, 15–37 weeks), with 76 cases diagnosed before 24 Restrictive ductus arteriosus 3 (1.6)
weeks. Twenty-three fetuses had isolated prevalence of the Atrial septal defect 2 (1.1)
right heart; 155 had coexisting intracardiac defects; and Aortic arch interruption 2 (1.1)
77 had extracardiac anomalies. Ventricular septal defects Coronary artery fistula 1 (0.5)
Transposition of the great arteries 1 (0.5)
(20.9%) were seen most frequently in these fetuses.
Interruption of inferior vena cava 1 (0.5)
The associated cardiac defects and extracardiac anomalies

Table 2. Extracardiac Anomalies Associated With Prevalence of the Right Heart by System

System Anomaly (n [%])

Central nervous system Strawberry-shaped skull (11 [6.0]),a choroid plexus cyst (6 [3.3]),a Dandy-Walker malformation (5 [2.7]),
ventriculomegaly (1 [0.5]), agenesis of corpus callosum (1 [0.5]), holoprosencephaly (1 [0.5]),
spina bifida (1 [0.5])
Face and neck Long eyebrow (7 [3.8]), cleft lip and palate (6 [3.3]), micrognathia (6 [3.3]), low-set ears (5 [2.7]),
hydroma (2 [1.1]), hypotelorism (1 [0.5])
Gastrointestinal Ascites (5 [2.7]), obstruction of duodenum (5 [2.7]), situs viscera inversus (5 [2.7]), omphalocele (2 [1.1]),
anorectal atresia (1 [0.5]), esophageal atresia (1 [0.5])
Urogenital Ectopic kidney (4 [2.2]), agenesis of kidney (2 [1.1]), horseshoe kidney (2 [1.1]), hypospadias (2 [1.1]),
multicystic kidney (2 [1.1]), pyelectasis (1 [0.5])
Skeletal Overlapping fingers (12 [6.6]),a short femur (10 [5.5]),a hypoplasia of radius (3 [1.6]), clinodactyly (2 [1.1]),
club foot (2 [1.1]),a rocker bottom foot (1 [0.5])
Chromosomal Trisomy 18 (9 [4.9]), trisomy 21 (6 [3.3]), trisomy 13 (3 [1.6]), monosomy X (3 [1.6]), 69,XXY (1 [0.5])
Other Fetal growth restriction (20 [11.0]), nuchal fold thickening (17 [9.3]),a single umbilical artery (15 [8.2]),
hypoplasia of nasal bone (14 [7.7]),a echogenic bowl (10 [5.5]), pleural effusion (1 [0.5]),
small cyst in lateral ventricle (1 [0.5])
aAssociated with chromosomal anomalies.

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Fetal and neonatal outcomes were as follows: 106 pregnancy and selective reduction, the remaining 75 cases
(58.2%) terminations of pregnancy, 1 (0.5%) intrauterine were classified into 2 groups according to whether they
death, 1 (0.5%) selective reduction, 25 (13.7%) neonatal survived (n = 49) or not (n = 26). The mean RV-LV ratios
deaths, and 49 (26.9%) survivors (Figure 1). The mean in these groups were 1.59 and 1.73 (P = .022; Figure 3).
gestational age at birth was 38 weeks (range, 28–40 weeks). To assess the prognostic impact of these characteris-
Of the 25 neonatal deaths, major extracardiac anomalies tics on survival, the remaining 75 fetuses, which excluded
were present in 4 cases. Excluding cases undergoing termi- cases undergoing termination of pregnancy and selective
nation of pregnancy and selective reduction, the adjusted reduction, were classified into 2 groups according whether
overall survival rate was 65.3% (49 of 75), of whom 11 they survived (n = 49) or not (n = 26). The impact of the
needed cardiac surgery postnatally. Considering only cases above-mentioned variables on survival was then evaluated
diagnosed before 24 gestational weeks, 54 of 76 (71.1%) by multivariable logistic regression with survival as the
underwent termination of pregnancy, and only 4 neonates independent variable. The following binary variables were
survived at the time of this writing. Excluding cases under- entered in the model: coexisting intracardiac anomalies,
going termination of pregnancy and selective reduction, associated extracardiac anomalies, diagnosis before 24 ges-
the adjusted survival rate in the fetuses with a diagnosis tational weeks, fetal growth restriction, reversed flow across
before 24 gestational weeks was 19.0% (4 of 21), compared the foramen ovale, reversed flow in the aortic arch, and RV-
with 83.3% (45 of 54) in the fetuses with a diagnosis after LV ratio greater than 2.0. The significant independent
24 weeks (P < .001). In comparison with the fetuses with- prognostic factors were coexisting intracardiac anomalies
out extracardiac anomalies, those with extracardiac anom- (odds ratio, 17.75; 95% confidence interval, 4.18–75.26)
alies had slightly poorer prognoses, with adjusted survival and diagnosis before 24 weeks (odds ratio, 17.26; 95%
rates of 60.0% (12 of 20) and 67.3% (37of 55), respectively confidence interval, 1.80–165.39).
(P = .558).
The fetuses with intracardiac defects were divided into Discussion
2 groups: intracardiac defects only (n = 82) and intracar-
diac defects with extracardiac defects (n = 73). The asso- Normally, the heart is divided symmetrically into two equal
ciated anomalies and outcomes are presented in Table 3. parts. The RV and LV were found to be of approximately
Excluding cases undergoing termination of pregnancy and equal size at 16 gestational weeks.2 However, the RV
selective reduction, the survival rates in these groups were becomes progressively more dilated than the LV.The relative
similar (P = .539). An abnormal karyotype was present in
4 of the 82 fetuses with associated intracardiac defects only,
Figure 1. Fetuses with suspected prevalence of the right heart accord-
which included 1 fetus with trisomy 18, 1 with trisomy 21, ing to the selection criteria and fetal and neonatal outcomes. GW indi-
1 with 45,XO, and 1 with another abnormality. In the 73 cates gestational weeks; IUD, intrauterine death; TOP, termination of
fetuses with intracardiac and extracardiac defects, 8 fetuses pregnancy; and TTTS, twin-twin transfusion syndrome.
with trisomy 18, 3 with trisomy 21, 2 with trisomy 13,
2 with 45,XO, and 3 with others were detected. The differ-
ence in abnormal karyotypes between these groups was
significant (P < .001).
In the whole series, left-to-right flow across the foramen
ovale was identified in 2 cases (1.1%), and retrograde flow
in the aortic arch was seen in 31 (17.0%). In all fetuses,
the mean RV-LV ratio was 1.67 (range, 1.20–8.80), and the
mean pulmonary artery-to-aorta ratio was 1.63 (range,
0.25–8.42). The mean RV-LV ratios were 1.59 in the 49
surviving cases and 1.72 in the remaining cases (P = .001;
Figure 2), and the mean pulmonary artery-to-aorta ratios
in these groups were 1.46 and 1.65, respectively (P = .103).
Considering only cases diagnosed before 24 gestational
weeks, the mean RV-LV ratio was 1.76; the mean RV-LV
ratio was 1.72 in the cases diagnosed after 24 weeks
(P = .043). Excluding cases undergoing termination of

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Peng et al—Prenatal Diagnosis of Prevalence of the Right Heart

size ratios of the cardiac ventricles in fetuses were reported
as 0.82 to 1.1410 and 0.82 to 1.20,12 and the ratio will grad-
ually increase with advanced gestational age. The fetuses in
our cohort had a diagnosis of prevalence of the right heart
between 15 and 37 gestational weeks. In our study, the
diagnostic criterion was defined as an RV-LV ratio of 1.20
or greater, whereas Jung et al13 chose to define a dominant
right heart as a right-to-left chamber ratio of 1.5, by which
some fetuses with mild to moderate ventricular dispropor-
tion may not be identified.
Cardiac asymmetry, either ventricular or great arterial,
was present in two-thirds of fetuses with cardiac anomalies
diagnosed prenatally but not across the spectrum of fetal
heart defects.14 Cardiac defects that can cause a larger
right heart or a smaller left heart may lead to prevalence of
the right heart; therefore, prevalence of the right heart may

Table 3. Associated Anomalies and Outcomes in Fetuses With Intracardiac Defects With and Without Coexisting Extracardiac Defects

Intracardiac Defects Intracardiac and Extracardiac

Group Only, n (%) Defects, n (%) P
Intracardiac defects
Hypoplastic left heart syndrome 20 (24.4) 10 (13.7) .093
Ventricular septal defect 15 (18.3) 23 (31.5) .056
Aortic coarctation 12 (14.6) 14 (19.2) .450
Persistent left superior vena cava 12 (14.6) 18 (24.7) .115
Tricuspid regurgitation 12 (14.6) 10 (13.7) .868
Pulmonary stenosis 10 (12.2) 8 (11.0) .810
Double-outlet RV 9 (11.0) 16 (21.9) .064
Restrictive foramen ovale 7 (8.5) 2 (2.7) .123
Ebstein anomaly 5 (6.1) 2 (2.7) .323
Absent pulmonary valve syndrome 5 (6.1) 0 (0) .033
Right-sided aortic arch 4 (4.9) 1 (1.4) .237
Aortic stenosis/atresia 4 (4.9) 4 (5.5) .866
Mitral valve stenosis/atresia 3 (3.7) 5 (6.8) .370
Restrictive ductus arteriosus 3 (3.7) 0 (0) .099
Tetralogy of Fallot 2 (2.4) 1 (1.4) .630
Endocardial cushion defect 2 (2.4) 4 (5.5) .327
Anomalous pulmonary venous connection 2 (2.4) 2 (2.7) .906
Aortic arch interruption 2 (2.4) 0 (0) .179
Aberrant right subclavian artery 2 (2.4) 2 (2.7) .906
Interruption of inferior vena cava 1 (1.2) 0 (0) .344
Transposition of great arteries 1 (1.2) 0 (0) .344
Coronary artery fistula 1 (1.2) 0 (0) .344
Truncus arteriosus communis 1 (1.2) 2 (2.7) .493
Atrial septal defect 1 (1.2) 1 (1.4) .934
Karyotype
Normal 23 (50.0)a 11 (28.2)b .041
Karyotypically normal live born 19 (41.3)a 10 (25.6)b .129
Abnormal 4 (8.7)a 18 (46.2)b <.001
Trisomy 18 1 (2.2)a 8 (20.5)b .006
Trisomy 21 1 (2.2)a 3 (7.7)b .231
Trisomy 13 0 (0) 2 (5.1)b .120
45,XO 1 (1.2)a 2 (5.1)b .462
Other 1 (1.2)a 3 (7.7)b .231
Fetal/neonatal outcomes
Termination of pregnancy 50 (60.9) 55 (75.3) .056
Survival 14 (43.8)c 9 (52.9)d .539
Neonatal death 18 (56.3)c 7 (41.1)d .315
Intrauterine death 0 (0) 1 (5.9)d .166
Selective reduction 0 (0) 1 (1.4)d .288
aExcluding cases in which fetal karyotyping was rejected, the total number was 46.
bExcluding cases in which fetal karyotyping was rejected, the total number was 39.
cExcluding cases undergoing termination of pregnancy and selective reduction, the total number was 32.
dExcluding cases undergoing termination of pregnancy and selective reduction, the total number was 17.

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Peng et al—Prenatal Diagnosis of Prevalence of the Right Heart
be absolute or relative. The former could be caused by an
RV pressure or volume overload, and the later is mainly
associated with aortic valve stenosis, aortic coarctation, and
hypoplastic left heart syndrome. Other causes of ventricular
disproportion include a total anomalous pulmonary venous
connection, a persistent left superior vena cava, and a
restrictive foramen ovale.
Prevalence of the right heart is an important indirect
sign of aortic coarctation,15 especially in early pregnancy,
although it still might be difficult to make a definite diag-
nosis of aortic coarctation in fetuses with this condition.
In our data, 26 fetuses with aortic coarctation were identi-
fied. In these fetuses, the mean RV-LV ratio was 1.61, and
only 7 cases (26.9%) were diagnosed before 24 gestational
Figure 2. Distribution of the RV-LV ratio in all 182 fetuses.
Figure 3. Distribution of the RV-LV ratio in the remaining 75 fetuses,
excluding cases undergoing termination of pregnancy and selective
reduction.
1160
weeks. It is well known to us that aortic coarctation is pro-
gressive prenatally,16 but in late pregnancy, it is difficult to
discriminate aortic coarctation from a normal fetal heart.
Jung et al13 reported that only 4 of 29 fetuses presenting
with a dominant right heart in late pregnancy were found
to have aortic coarctation postnatally. Using the size of
the ventricle as the only marker for aortic coarctation
would have given high rates of both false-negative and
false-positive diagnoses of the anomaly.
The left heart is smaller than normal when it receives
less blood flow than normal. All pulmonary veins drain
directly or indirectly into the RA in the fetuses with a total
anomalous pulmonary venous connection, so the left heart
is small compared with the right one. We should find at least
2 pulmonary veins draining into the LA to exclude a total
anomalous pulmonary venous connection on prenatal
sonographic screening. A persistent left superior vena cava
is a remnant of the proximal segment of the left anterior car-
dinal vein, resulting from failure of the left anterior cardinal
vein to undergo atrophy after formation of the oblique anas-
tomosis with the right cardinal vein. The vessel most often
drains into the RA through the coronary sinus. The dilated
coronary sinus will dynamically obstruct mitral valve inflow,
which will shift the valve of the foramen ovale and will lead
to redistribution of the pressure gradient of the heart. This
process may explain the association between a persistent
left superior vena cava and prevalence of the right heart.
In our study, the fetuses with intracardiac anomalies
were divided into 2 groups according to the presence or
absence of coexisting extracardiac defects. The fetuses with
chromosomal anomalies usually had multiple-system
involvement,17 which may be the reason why the fetuses
with both extracardiac and intracardiac defects had a much
higher risk of abnormal karyotypes. However, the survival
rates in these groups were not significantly different
(P = .407); even when excluding cases undergoing termi-
nation of pregnancy and selective reduction, the adjusted
survival rates were approximately similar (P = .539).
The most interesting finding of this study is the strong
prognostic significance of coexisting intracardiac anomalies
and diagnosis before 24 gestational weeks, as shown by the
multivariable logistic regression analysis. Parameters such as
associated extracardiac anomalies, fetal growth restriction,
reversed flow across the foramen ovale, reversed flow in the
aortic arch, and an RV-LV ratio greater than 2.0 were not
very predictive of outcomes. In this study, most cases diag-
nosed before 24 gestational weeks underwent elective termi-
nation of pregnancy; even when continuing the pregnancy,
only 4 neonates survived. The diagnosis may be accurately
established in the mid second trimester, but follow-up scans
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may further increase the degree of certainty of the poten-
tial for progression of prevalence of the right heart.18
Weight represents one of the most important variables
affecting neonatal survival during cardiac surgery.19 Paladini
et al20 showed that fetal growth restriction was a significant
predictor of poor prognosis for cases with aortic coarcta-
tion. Although prevalence of the right heart is an indirect
sign of aortic coarctation, our data showed that the prog-
nosis of fetuses with prevalence of the right heart was not
heavily influenced by fetal growth restriction. A retrospec-
tive study on conventional repair showed the relationship
between the probability of hospital death and the ventricu-
lar dominance ratio, with greater than 60% mortality in chil-
dren with complete atrioventricular septal defects and RV
dominance with an RV-LV volume ratio of 2.0.7 In our
study, an RV-LV ratio greater than 2.0 was not significantly
associated with a poor prognosis for prevalence of the right
heart. The RV-LV volume ratio may be more practical for
pediatric cardiologists and cardiac surgeons.
Our study had several limitations, one being that more
than half of the fetuses examined underwent termination of
pregnancy for 1-child family planning in China, which did
not allow a comprehensive evaluation of the natural clinical
outcomes of these fetuses. Another limitation was the lack
of karyotype information for the cases in which fetal kary-
otyping was rejected.
In conclusion, we have confirmed that prevalence of
the right heart, when diagnosed in the fetus, is significantly
associated with intracardiac anomalies. In addition, we
have shown that, at least as found this study, coexisting
intracardiac anomalies and diagnosis before 24 gestational
weeks are consistent risk factors, which represent the most
important poor prognostic indicators. These data should be
considered during prenatal counseling in cases with sus-
pected prevalence of the right heart. The degree of accu-
racy may be improved by combining size-based cardiac
parameters with the gestational age at diagnosis.
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