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Congenital Heart Disease. 2018;1–9.	 wileyonlinelibrary.com/journal/chd  |  1© 2018 Wiley Periodicals, Inc.
Received: 4 April 2018  |  Revised: 25 May 2018  |  Accepted: 3 June 2018
DOI: 10.1111/chd.12648
O R I G I N A L A R T I C L E
Prenatal detection of critical cardiac outflow tract anomalies
remains suboptimal despite revised obstetrical imaging
guidelines
Heather Y. Sun MD1
 | James A. Proudfoot MSc2
 | Rachel T. McCandless MD1
1
Division of Pediatric Cardiology,
Department of Pediatrics, Rady Children’s
Hospital, University of California, San Diego,
California
2
Altman Clinical and Translational Research
Institute, University of California, San Diego,
California
Correspondence
Heather Y. Sun, Division of Pediatric
Cardiology, Department of Pediatrics, Rady
Children’s Hospital, University of California,
3020 Children’s Way, MC 5004, San Diego,
CA 92123.
Email: hsun@rchsd.org
Funding information
This study was partially funded by the
National Institutes of Health, Grant/Award
Number: Grant UL1TR001442 of CTSA.
Abstract
Background: Fetal echocardiography can accurately diagnose critical congenital heart disease
prenatally, but relies on referrals from abnormalities identified on routine obstetrical ultra-
sounds. Critical congenital heart disease that is frequently missed due to inadequate outflow
tract imaging includes anomalies such as truncus arteriosus, double outlet right ventricle,
transposition of the great arteries, tetralogy of Fallot, pulmonary stenosis, and aortic stenosis.
Objective: This study evaluated the prenatal detection rate of critical outflow tract anomalies
in a single urban pediatric hospital before and after “AIUM Practice Guideline for the
Performance of Obstetric Ultrasound Examinations,” which incorporated outflow tract
imaging.
Design: Infants with outflow tract anomalies who required cardiac catheterization and/or sur-
gical procedure(s) in the first 3 months of life were retrospectively identified. This study evalu-
ated two time periods; pre‐guidelines from June 2010 to May 2013 and post‐guidelines from
January 2015 to June 2016. June 2013‐December 2014 was excluded as a theoretical period
necessary for obstetrical practices to implement the revised guidelines.
Results: Overall, prenatal diagnosis occurred in 55% of infants with critical outflow tract anom-
alies; of the three most common defects, prenatal diagnosis occurred in 53% of D‐transposition
of the great arteries, 63% of tetralogy of Fallot, and 80% of double outlet right ventricle pa-
tients. Pre‐guidelines, prenatal diagnosis occurred in 52% (52 of 102) infants with critical out-
flow tract anomalies requiring early cardiac intervention. Post‐guidelines, prenatal diagnosis
occurred in 61% (33 of 54) infants, not significantly different than the prenatal detection rate
pre‐guidelines (P = .31).
Conclusions: Despite revised obstetrical guidelines highlighting the importance of outflow
tract imaging, referrals and prenatal diagnosis of these types of critical congenital heart disease
remain low. Education of obstetrical sonographers and practitioners who perform fetal ana-
tomic screening is vital to increase referrals and prenatal detection of critical outflow tract
anomalies.
K E Y W O R D S
fetal cardiology, fetal echocardiography, prenatal diagnosis
2  |     SUN et al.
1 | INTRODUCTION
Congenital heart disease (CHD) is the most common congenital
anomaly, accounting for approximately 1% of live births.1
About one
in four infants with CHD have critical CHD requiring timely inter-
vention after birth to prevent significant morbidity or mortality.2
Prenatal diagnosis of critical CHD by fetal echocardiogram (ECHO)
leads to appropriate fetal cardiac care, delivery, and postnatal man-
agement in a pediatric cardiac center.3
Most referrals for fetal ECHO
and subsequent accurate prenatal diagnosis of CHD rely primarily on
abnormality detection on screening obstetrical anatomic ultrasound,
as many cases of CHD occur in otherwise low‐risk pregnancies.4,5
Until recently, obstetric anatomic ultrasound guidelines for
cardiac evaluation mandated only a four‐chamber view, with
“views of the outflow tracts attempted as part of the cardiac
screening.”6
Critical CHD that can be missed by imaging only the
four‐chamber view (Figure 1A) include outflow tract anomalies
such as D‐­transposition of the great arteries (TGA) (Figure 1B and
C), congenitally corrected‐TGA (cc‐TGA), tetralogy of Fallot (TOF),
double outlet right ventricle (DORV), pulmonary atresia with intact
ventricular septum (PA/IVS), truncus arteriosus, pulmonary steno-
sis, and aortic stenosis. Many of these defects require surgical or
cardiac catheterization intervention within the first few months
of life and usually prior to discharge after delivery. In this study,
we defined critical CHD as a cardiac defect requiring cardiac cath-
eterization and/or surgical procedure(s) in the first 3 months of
life, with a focus on outflow tract anomalies. Studies have demon-
strated the ability of fetal ECHO to predict the need for urgent
postnatal intervention,7‒9
as well as improved neonatal outcomes
with prenatal diagnosis.10‒12
With evidence supporting the value
of ventricular outflow tract imaging,13
in June 2013, the American
Institute of Ultrasound in Medicine (in conjunction with the
American College of Radiology, American College of Obstetricians
and Gynecologists, and the Society of Radiologists in Ultrasound)
updated its obstetrical ultrasound guidelines to include evaluation
of the ventricular outflow tracts.14
2 | OBJECTIVE
As the San Diego region’s only pediatric cardiac and cardiovascular
surgery center, all neonates with critical CHD potentially requir-
ing interventional management are cared for at Rady Children’s
Hospital. The primary objective of this retrospective study was
to evaluate the prenatal detection rate of critical outflow tract
anomalies in neonates at Rady Children’s Hospital with critical
outflow tract CHD before and after theoretical implementation
of the 2013 updated obstetrical imaging guidelines. Ideally, the
majority of neonates with critical outflow tract CHD should have
been detected by prenatal ventricular outflow tract imaging on
obstetrical ultrasound.
3 | METHODS
3.1 | Study design
Infants with critical CHD requiring cardiac catheterization and/
or surgical procedure(s) in the first 3 months of life were retro-
spectively identified through Rady Children’s Hospital cardiac and
electronic medical record databases. Infants were subdivided into
two time frames; the pre‐guideline time frame consisted of infants
requiring procedures between June 2010 and May 2013, while
the post‐guideline time frame consisted of infants requiring pro-
cedures between January 2015 and June 2016. The timeframe be-
tween June 2013 and December 2014 was specifically excluded
to allow for theoretical time necessary for obstetrical providers to
FI G U R E 1 (A) Normal appearing cardiac four‐chamber view with abnormal parallel, (B) right ventricular, and (C) left ventricular outflow
tracts in a fetus with D‐transposition of the great arteries. Abbreviations: RV, right ventricle; LV, left ventricle; RA, right atrium; LA, left
atrium; Ao, aorta; PA, pulmonary artery
    | 3SUN et al.
implement the recommended guidelines into daily practice. Actual
implementation of outflow tract imaging per the new guidelines
was at the discretion of individual obstetrical practices; there
is currently no universal training of all obstetrical sonographers
given the large number of practices in San Diego county.
Infants with the following cardiac outflow tract anomalies
identifiable by outflow tract imaging were included: D‐TGA, cc‐
TGA, TOF, DORV, PA/IVS, truncus arteriosus, pulmonary stenosis,
and aortic stenosis. Although cc‐TGA should also be detectable
by the four‐chamber view, cc‐TGA was included in this study, as
it may be unrealistic to expect the general obstetrical screening
sonographer the nuance of differentiating the morphologic right
versus left ventricle. Those with other forms of CHD potentially
diagnosable by the four‐chamber view were excluded (ie atrio-
ventricular canal defects or single ventricle lesions such as hypo-
plastic right or left heart syndrome). Isolated aortic coarctation
without aortic valve stenosis was also excluded as it is not reliably
expected to be diagnosed prenatally. Rady Children’s Hospital
also serves as the primary referral center for infants with CHD
from Hawaii and the Pacific Islands; out‐of‐state patients were
excluded. The study was approved by the Institutional Review
Boards of Rady Children’s Hospital and University of California,
San Diego.
3.2 | Statistical analysis
Interval variables were expressed as mean  standard deviation or
median (range). Categorical variables were counted and expressed as
frequencies or percentages. Due to the non‐normality of most of our
continuous study outcomes, the Mann‐Whitney U test was used as
a robust rank‐based statistic to determine if there was a significant
difference between pre‐guidelines and post‐guidelines or prenatal
and postnatal diagnosis groups. Due to some small expected cell
counts, Fisher’s exact test was used to compare categorical variables
across groups. A p value less than .05 was considered statistically
significant. All analyses were performed using the latest version of R
software (version 3.3.2).
4 | RESULTS
All but one of the mothers in each time frame had reportedly re-
ceived standard prenatal care. There was no association between
maternal race or type of health insurance and prenatal versus post-
natal diagnosis (P = .99 and .41, respectively) (Table 1).
Overall, prenatal diagnosis occurred in 55% of infants with crit-
ical outflow tract anomalies. Prenatal diagnosis occurred at a mean
age of 26.7 ± 4.8 weeks gestational age. Postnatal diagnosis of a
critical outflow tract anomaly was mostly made on day of life 1 or 2
(range 1–71 d) (Table 2). The most common clinical findings leading
to postnatal diagnosis of CHD were hypoxemia, murmur, or failed
pulse oximetry screening. Seven percent of patients (11 of 153 new-
borns) were discharged prior to diagnosis; none of these patients had
ductal dependent lesions. Of the three most common defects, pre-
natal diagnosis occurred in 54% of D‐TGA, 63% of TOF, and 80% of
DORV patients (Figure 2).
Fifty percent of the infants required transfer from the delivery
hospital to our institution; the other infants were born at our ad-
joining women’s hospital. Sixty‐three percent had ductal dependent
lesions for systemic or pulmonary blood flow. Most infants (86%)
required intervention prior to hospital discharge. Three infants died
without procedural intervention due to withdrawal of care. The
first cardiac intervention occurred at a median age of 6 days (range
0–88 d), with 51 infants undergoing only cardiac catheterization, 69
infants undergoing only surgical palliation/repair, and 33 infants un-
dergoing both catheterization followed by surgical palliation/repair
within the first 3 months.
4.1 | Prenatal versus postnatal diagnosis
Prenatally diagnosed infants had a statistically significant slightly ear-
lier gestational age at birth (37.7 ± 2.4 wk vs. 37.9 ± 3.4 wk in post-
natally diagnosed infants, P = .03). Prenatally diagnosed infants had
statistically lower birth weights (2.97 ± 0.69 kg vs. 3.18 ± 0.64 kg,
P = .02) and 5‐minute Apgar scores (7.9 ± 1.6 vs. 8.4 ± 0.9, P = .01).
There was a higher frequency of ductal dependent lesions in those
Prenatal diagnosis
n = 86 (%)
Postnatal diagnosis
n = 70 (%) p value
Maternal race .99
White 34 (40%) 28 (40%)
Hispanic 32 (37%) 26 (37%)
Asian 10 (12%) 7 (10%)
African American 3 (3%) 3 (4%)
American Indian 0 1 (1%)
Hawaiian 1 (1%) 0
Unknown/not reported 6 (7%) 5 (7%)
Maternal insurance type .41
Private 53 (62%) 38 (54%)
Public 33 (38%) 32 (46%)
TA B LE 1 Maternal demographics, by
timing of diagnosis
4  |     SUN et al.
prenatally diagnosed (73% vs. 50% postnatally diagnosed, P = .004).
There was no significant difference in degree of pre‐intervention
lactic acidosis, need for pre‐intervention inotropic support, or num-
ber of days to first intervention (Table 2).
4.2 | Pre‐guidelines vs. post‐guidelines
Pre‐guideline revision, between June 2010 and May 2013, 102 of
424 infants with critical CHD had outflow tract anomalies poten-
tially identifiable by outflow tract imaging which required cardiac
catheterization or surgery within the first 3 months of life. Prenatal
diagnosis occurred in 52% of the pre‐guidelines group. Post‐guide-
lines, between January 2015 and June 2016, 54 of 176 infants with
critical CHD had outflow tract anomalies which required cardiac
catheterization or surgery within the first 3 months of life. Prenatal
diagnosis occurred in 61% of the post‐guidelines group, not sig-
nificantly different than the prenatal detection rate pre‐guide-
lines (P = .31) (Table 3 and Figure 3). The types of cardiac outflow
tract anomalies pre‐guidelines versus post‐guidelines were similar
(Table 3 and Figure 4). The timing of diagnosis was also not signifi-
cantly different based on type of cardiac defect pre‐guidelines vs.
post‐guidelines (Table 4 and Figure 5).
5 | DISCUSSION
Screening for congenital anomalies should occur during routine
anatomic obstetrical ultrasound examination at approximately
18‐20 weeks gestational age in all pregnancies.14
The most com-
mon congenital anomaly is CHD, affecting approximately 1% of live
Prenatal
diagnosis n = 84
Postnatal diagnosis
n = 65 p value
Timing of diagnosis Gestational age
26.7 ± 4.8 wk
Day of life 1 (1−71) n/a
Gestational age at birth (weeks) 37.7 ± 2.4 37.9 ± 3.4 .03
Birth weight (kg) 2.97 ± 0.69 3.18 ± 0.64 .02
Apgar score
1 min of life 6.9 ± 2.1 7.2 ± 1.9 .47
5 min of life 7.9 ± 1.6 8.4 ± 0.9 .01
Genetic syndrome and/or extracardiac
anomaly
28 (33%) 14 (20%) .10
Ductal dependent 63 (73%) 35 (50%) .004
Highest pre‐intervention lactate 3.07 ± 2.05 4.30 ± 4.48 .08
Pre‐intervention inotropic support 21 (26%) 17 (26%) .99
Timing of first Intervention (day of life) 6 (0−88) 5 (0−84) .19
Continuous data are reported as mean ± SD or median (range), as appropriate, and categorical data
are reported as count (percentage).
TA B LE 2 Patient characteristics, by
timing of diagnosis
FI G U R E 2 Overall cardiac diagnoses
and timing of diagnosis
010203040
NumberofinfantsAortic
Stenosis
D
−TG
A
D
O
R
V
PA/IVS
Pulm
onary
Stenosis
TO
F
Truncus
Arteriosus
cc−TG
A
Cardiac Diagnoses and Timing of Diagnosis
Postnatal Diagnosis Prenatal Diagnosis
    | 5SUN et al.
infants.1
Of these, approximately 25% have critical CHD requir-
ing timely intervention to prevent significant infant morbidity or
mortality.2
Although a multitude of maternal and/or fetal factors can prompt
referrals for fetal ECHO to evaluate for the presence (or absence)
of prenatal CHD,4
most CHD occurs in pregnancies without mater-
nal or fetal risk factors.5
Referrals for fetal ECHO in these partic-
ular cases rely solely on the identification of cardiac abnormalities
on screening obstetrical ultrasound. Studies have shown that less
experienced obstetric sonographers have lower detection rates of
cardiac abnormalities on screening ultrasound.15
In the mid‐1990s,
a single major referral center in New South Wales showed that ob-
stetrics screening detected only 6.7% of ventricular outflow tract
anomalies prenatally.16
In 1992, a study at a single obstetrics center
in the US also demonstrated significant limitations to the four‐cham-
ber view, with only 63% of all CHD detected, while the detection
rate increased to 83% when ventricular outflow tract imaging was
included.13
Outflow tract imaging is practically feasible when incor-
porated into a standard screening protocol.17
Despite increased awareness and education, prenatal detection
of various forms of CHD remains low,18,19
with outflow tract anom-
alies historically lower than for other types of critical CHD (such
as single ventricle lesions).20
Contemporary studies have shown
a wide range of prenatal diagnosis rates for conotruncal anoma-
lies (Table 5).21‒28
Many outflow tract anomalies can appear nor-
mal when visualizing only the four‐chamber view (Figure 1A). The
ability of obstetric anatomic ultrasound to identify outflow tract
anomalies increases substantially when ventricular outflow tract
imaging supplements the more traditional four‐chamber view.18,19,29
In June 2013, the American Institute of Ultrasound in Medicine
officially recognized the importance of imaging ventricular outflow
tracts by updating its guidelines on obstetric anatomic ultrasound
to include this critical outflow tract view into its basic cardiac ex-
amination.14
These guidelines (developed in conjunction with the
American College of Radiology, American College of Obstetricians
and Gynecologists, and the Society of Radiologists in Ultrasound)
are the standard of care reference for obstetric practices in the
United States. The International Society of Ultrasound in Obstetrics
TA B LE 3 Patient characteristics, by time frame
Pre‐guidelines n = 102 (%) Post‐guidelines n = 54 (%) p value
Timing of diagnosis .31
Prenatal diagnosis 53 (52%) 33 (61%)
Postnatal diagnosis 49 (48%) 21 (39%)
Type of cardiac defect .97
D‐TGA 26 (26%) 13 (24%)
TOF 19 (18%) 14 (26%)
DORV 17 (16%) 9 (17%)
Pulmonary stenosis 15 (15%) 6 (11%)
Truncus arteriosus 10 (10%) 5 (9%)
Aortic stenosis 8 (8%) 4 (7%)
PA/IVS 6 (6%) 2 (4%)
CC‐TGA 1 (1%) 1 (2%)
Prenatal care (if postnatal diagnosis) .52
Yes 48 (98%) 20 (95%)
No 1 (2%) 1 (5%)
Gestational age (weeks) 38 ± 2.3 37.4 ± 3.8 .31
Birth weight (kg) 3.1 ± 0.7 3 ± 0.7 .33
FIGURE 3 Timing of diagnosis: pre‐guidelines versus ­
post‐guidelines
6  |     SUN et al.
and Gynecology also published similar guidelines to include outflow
tract views.30
Fetal ECHO performed by fetal/pediatric cardiac sonographers
with interpretation by fetal/pediatric cardiologists provide a more
accurate prenatal cardiac diagnosis31,32
as compared to obstetri-
cal ultrasound. Fetal ECHO accurately detects simple and complex
CHD,3
such as outflow tract anomalies,33‒35
and allows for directed
prenatal parental counseling as well as delivery management in an
appropriate setting.36
The most common outflow tract anomalies,
such as TOF, d‐TGA, DORV with pulmonary stenosis, or DORV with
malposed great arteries, can cause significant cyanosis and hypoxia
after birth leading to poor neonatal outcomes if not appropriately
treated in a timely manner; the long‐term survival rates are high if
appropriately treated. Prenatal detection with timely post‐delivery
access to cardiac medical and surgical management improves mor-
tality rates and outcomes.12,35,37,38
The overall prenatal detection rate of 55% in this study remains
low in the modern era of specialty medicine capable of prenatally
diagnosing and managing critical cardiac outflow tract anomalies.
Despite the official revised obstetrical ultrasound guidelines, this
study shows no significant difference in the prenatal diagnosis rate
of these critical outflow tract anomalies following guideline revision.
The limitation stems primarily from lack of obstetrical identification
of abnormalities—presumably from inadequate imaging of the ven-
tricular outflow tracts on routine anatomic screening ultrasounds.
This study supports the need for more resources dedicated to ed-
ucating obstetrical sonographers and practitioners in optimizing
imaging of ventricular outflow tracts and/or encouraging referrals
for fetal echocardiograms if unable to confidently confirm normal
outflow tracts.
In this study, prenatally diagnosed infants had a statistically sig-
nificant earlier gestational age at birth, although the majority were
still born at or near term. We speculate that prenatally detected CHD
placed these pregnancies into a higher risk category and may have
prompted increased pregnancy and fetal monitoring and a lower
threshold for induction or delivery. Prenatally diagnosed infants also
had slightly lower birth weights and 5‐minute Apgar scores, which may
have been related to their earlier gestational age at birth. The majority
of infants had ductal dependent lesions and/or required intervention
prior to hospital discharge, indicating high risk for hemodynamic com-
promise if not diagnosed and treated in an expeditious manner.
No differences were seen in pre‐operative lactic acidosis,
need for inotropic support, or number of days to first proce-
dure between the prenatal and postnatal diagnosis groups. The
FI G U R E 4 Cardiac diagnoses: pre‐guidelines and post‐guidelines
Pre‐guideline %
(# prenatal diagnosis/total)
Post‐guideline %
(# prenatal diagnosis/total) p value
D‐TGA 50% (13/26) 61% (8/13) .73
TOF 58% (11/19) 71% (10/14) .48
DORV 76% (13/17) 88% (8/9) .62
Pulmonary stenosis 27% (4/15) 50% (3/6) .35
Truncus arteriosus 50% (5/10) 40% (2/5) .99
Aortic stenosis 50% (4/8) 0% (0/4) .21
PA/IVS 33% (2/6) 50% (1/2) .99
TA B LE 4 Prenatal detection rate of
cardiac diagnoses, by time frame
    | 7SUN et al.
FI G U R E 5 Timing of diagnosis by
cardiac diagnoses: (A) Pre‐Guidelines and
(B) Post‐Guidelines
0510152025
NumberofinfantsAortic
Stenosis
D
−TG
A
D
O
R
V
PA/IVS
Pulm
onary
Stenosis
TO
F
Truncus
Arteriosus
cc−TG
A
Pre−Guidelines
Postnatal Diagnosis Prenatal Diagnosis
051015
NumberofinfantsAortic
Stenosis
D
−TG
A
D
O
R
V
PA/IVS
Pulm
onary
Stenosis
TO
F
Truncus
Arteriosus
cc−TG
A
Post−Guidelines
Postnatal Diagnosis Prenatal Diagnosis
A
B
TA B LE 5 Prenatal diagnosis rates of conotruncal anomalies from recent studies
Location Author Years Number of patients Prenatal diagnosis rate (%)
D‐TGA Texas Lara 1999−2007 468 10
Czech Republic Marek 2000−2006 223 25
Netherlands van Velzen 2002−2012 172 36
Paris Khoshnood 2005−2008 85 70
Belgium De Groote 2006−2014 79 24
TOF Czech Republic Marek 2000−2006 142 37
Netherlands van Velzen 2002−2012 111 30
Paris Khoshnood 2005−2008 60 68
Belgium De Groote 2006−2014 117 23
Truncus arteriosus Boston Swanson 1992−2007 136 32
Czech Republic Marek 2000−2006 47 80
Netherlands van Velzen 2002−2012 46 62
PA/IVS Italy Tuo 1993−2009 60 60
Czech Republic Marek 2000−2006 88 68
cc‐TGA Toronto Wan 1999−2006 54 29
8  |     SUN et al.
heterogeneous nature of cardiac diagnoses in the two groups lim-
ited the power to detect statistical differences and draw conclu-
sions between pre‐intervention course between the prenatal and
postnatal diagnosis groups. Prenatal diagnosis of critical CHD has
been shown to decrease preoperative mortality in other studies.12
6 | LIMITATIONS
Given the large number of hospitals delivering infants in San Diego
county, infants with postnatal diagnosis of critical CHD that did not
survive to admission to our hospital were not able to be identified, as
it was outside the scope of our study. Theoretically, the addition of
these infants would further decrease the prenatal detection rate in
both timeframes.
This study did not aim to encompass all critical CHD, only those
detectable by outflow tract imaging. Other critical CHD, such as sin-
gle ventricle lesions and isolated aortic arch anomalies, were spe-
cifically excluded as the authors aimed to highlight the importance
of obstetric screening ultrasounds incorporating adequate outflow
tract imaging.
7 | CONCLUSIONS
Despite revised obstetrical guidelines highlighting the importance
of outflow tract imaging, referrals and prenatal diagnosis of these
types of critical CHD remain suboptimal.  Education of obstetri-
cal sonographers and practitioners who perform fetal anatomic
screening is vital to increase referrals for fetal cardiac evalu-
ation and optimize prenatal detection of critical outflow tract
anomalies.
ACKNOWLEDGMENTS
The project was partially supported by the National Institutes of
Health, Grant UL1TR001442 of CTSA funding. The content is solely
the responsibility of the authors and does not necessarily represent
the official views of the National Institutes of Health.
DISCLOSURE STATEMENT
The authors report no conflicts of interest.
AUTHOR CONTRIBUTIONS
All authors have read and approved the final version of the
manuscript.
Concept/Design, Data collection/interpretation, Drafting/revision
of article: Sun
Statistics: Proudfoot
Critical revision of article: McCandless
ORCID
Heather Y. Sun  http://orcid.org/0000-0001-9309-3565
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	22.	 Swanson TM, Selamet Tierney ES, Tworetzky W, Pigula F,
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	23.	 Marek J, Tomek V, Skovranek J, Povysilova V, Samanek M.
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	24.	 Khoshnood B, Lelong N, Houyel L, et al. Impact of prenatal diagnosis
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	25.	 van Velzen CL, Clur SA, Rijlaarsdam ME, et al. Prenatal detection of
congenital heart disease‐results of a national screening programme.
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	26.	 De Groote K, Vanhie E, Roets E, et al. Outcome after prenatal and
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	27.	 Lara DA, Fixler DE, Ethen MK, Canfield MA, Nembhard WN, Morris
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on outcome of pulmonary atresia and intact ventricular septum. J
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	29.	 Zhang YF, Zeng XL, Zhao EF, Lu HW. Diagnostic value of fetal echo-
cardiography for congenital heart disease: a systematic review and
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	30.	 Carvalho JS, Allan LD, Chaoui R, et al. ISUOG Practice Guidelines
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	31.	 Meyer‐Wittkopf M, Cooper S, Sholler G. Correlation between fetal
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	32.	 Lai CW, Chau AK, Lee CP. Comparing the accuracy of obstetric so-
nography and fetal echocardiography during pediatric cardiology
consultation in the prenatal diagnosis of congenital heart disease. J
Obstet Gynaecol Res. 2016;42:166–171.
	33.	 Gelehrter S, Owens ST, Russell MW, van der Velde ME, Gomez‐
Fifer C. Accuracy of the fetal echocardiogram in double‐outlet right
ventricle. Congenit Heart Dis. 2007;2:32–37.
	34.	 Galindo A, Mendoza A, Arbues J, Graneras A, Escribano D, Nieto
O. Conotruncal anomalies in fetal life: accuracy of diagnosis, as-
sociated defects and outcome. Eur J Obstet Gynecol Reprod Biol.
2009;146:55–60.
	35.	 Dominguez‐Manzano P, Mendoza A, Herraiz I, et al. Transposition
of the great arteries in fetal life: accuracy of diagnosis and short‐
term outcome. Fetal Diagn Ther. 2016;40:268–276.
	36.	 Donofrio MT, Levy RJ, Schuette JJ, et al. Specialized delivery room
planning for fetuses with critical congenital heart disease. Am J
Cardiol. 2013;111:737–747.
	37.	 Dominguez‐Manzano P, Herraiz I, Mendoza A, et al. Impact of pre-
natal diagnosis of transposition of the great arteries on postnatal
outcome. J Matern Fetal Neonatal Med. 2017;30:2858–2863.
	38.	 Pruetz JD, Carroll C, Trento LU, et al. Outcomes of critical congeni-
tal heart disease requiring emergent neonatal cardiac intervention.
Prenat Diagn. 2014;34:1127–1132.
How to cite this article: Sun HY, Proudfoot JA, McCandless
RT. Prenatal detection of critical cardiac outflow tract
anomalies remains suboptimal despite revised obstetrical
imaging guidelines. Congenital Heart Disease. 2018;00:1–9.
https://doi.org/10.1111/chd.12648

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Prenatal detection of critical cardiac outflow tract anomalies

  • 1. Congenital Heart Disease. 2018;1–9. wileyonlinelibrary.com/journal/chd  |  1© 2018 Wiley Periodicals, Inc. Received: 4 April 2018  |  Revised: 25 May 2018  |  Accepted: 3 June 2018 DOI: 10.1111/chd.12648 O R I G I N A L A R T I C L E Prenatal detection of critical cardiac outflow tract anomalies remains suboptimal despite revised obstetrical imaging guidelines Heather Y. Sun MD1  | James A. Proudfoot MSc2  | Rachel T. McCandless MD1 1 Division of Pediatric Cardiology, Department of Pediatrics, Rady Children’s Hospital, University of California, San Diego, California 2 Altman Clinical and Translational Research Institute, University of California, San Diego, California Correspondence Heather Y. Sun, Division of Pediatric Cardiology, Department of Pediatrics, Rady Children’s Hospital, University of California, 3020 Children’s Way, MC 5004, San Diego, CA 92123. Email: hsun@rchsd.org Funding information This study was partially funded by the National Institutes of Health, Grant/Award Number: Grant UL1TR001442 of CTSA. Abstract Background: Fetal echocardiography can accurately diagnose critical congenital heart disease prenatally, but relies on referrals from abnormalities identified on routine obstetrical ultra- sounds. Critical congenital heart disease that is frequently missed due to inadequate outflow tract imaging includes anomalies such as truncus arteriosus, double outlet right ventricle, transposition of the great arteries, tetralogy of Fallot, pulmonary stenosis, and aortic stenosis. Objective: This study evaluated the prenatal detection rate of critical outflow tract anomalies in a single urban pediatric hospital before and after “AIUM Practice Guideline for the Performance of Obstetric Ultrasound Examinations,” which incorporated outflow tract imaging. Design: Infants with outflow tract anomalies who required cardiac catheterization and/or sur- gical procedure(s) in the first 3 months of life were retrospectively identified. This study evalu- ated two time periods; pre‐guidelines from June 2010 to May 2013 and post‐guidelines from January 2015 to June 2016. June 2013‐December 2014 was excluded as a theoretical period necessary for obstetrical practices to implement the revised guidelines. Results: Overall, prenatal diagnosis occurred in 55% of infants with critical outflow tract anom- alies; of the three most common defects, prenatal diagnosis occurred in 53% of D‐transposition of the great arteries, 63% of tetralogy of Fallot, and 80% of double outlet right ventricle pa- tients. Pre‐guidelines, prenatal diagnosis occurred in 52% (52 of 102) infants with critical out- flow tract anomalies requiring early cardiac intervention. Post‐guidelines, prenatal diagnosis occurred in 61% (33 of 54) infants, not significantly different than the prenatal detection rate pre‐guidelines (P = .31). Conclusions: Despite revised obstetrical guidelines highlighting the importance of outflow tract imaging, referrals and prenatal diagnosis of these types of critical congenital heart disease remain low. Education of obstetrical sonographers and practitioners who perform fetal ana- tomic screening is vital to increase referrals and prenatal detection of critical outflow tract anomalies. K E Y W O R D S fetal cardiology, fetal echocardiography, prenatal diagnosis
  • 2. 2  |     SUN et al. 1 | INTRODUCTION Congenital heart disease (CHD) is the most common congenital anomaly, accounting for approximately 1% of live births.1 About one in four infants with CHD have critical CHD requiring timely inter- vention after birth to prevent significant morbidity or mortality.2 Prenatal diagnosis of critical CHD by fetal echocardiogram (ECHO) leads to appropriate fetal cardiac care, delivery, and postnatal man- agement in a pediatric cardiac center.3 Most referrals for fetal ECHO and subsequent accurate prenatal diagnosis of CHD rely primarily on abnormality detection on screening obstetrical anatomic ultrasound, as many cases of CHD occur in otherwise low‐risk pregnancies.4,5 Until recently, obstetric anatomic ultrasound guidelines for cardiac evaluation mandated only a four‐chamber view, with “views of the outflow tracts attempted as part of the cardiac screening.”6 Critical CHD that can be missed by imaging only the four‐chamber view (Figure 1A) include outflow tract anomalies such as D‐­transposition of the great arteries (TGA) (Figure 1B and C), congenitally corrected‐TGA (cc‐TGA), tetralogy of Fallot (TOF), double outlet right ventricle (DORV), pulmonary atresia with intact ventricular septum (PA/IVS), truncus arteriosus, pulmonary steno- sis, and aortic stenosis. Many of these defects require surgical or cardiac catheterization intervention within the first few months of life and usually prior to discharge after delivery. In this study, we defined critical CHD as a cardiac defect requiring cardiac cath- eterization and/or surgical procedure(s) in the first 3 months of life, with a focus on outflow tract anomalies. Studies have demon- strated the ability of fetal ECHO to predict the need for urgent postnatal intervention,7‒9 as well as improved neonatal outcomes with prenatal diagnosis.10‒12 With evidence supporting the value of ventricular outflow tract imaging,13 in June 2013, the American Institute of Ultrasound in Medicine (in conjunction with the American College of Radiology, American College of Obstetricians and Gynecologists, and the Society of Radiologists in Ultrasound) updated its obstetrical ultrasound guidelines to include evaluation of the ventricular outflow tracts.14 2 | OBJECTIVE As the San Diego region’s only pediatric cardiac and cardiovascular surgery center, all neonates with critical CHD potentially requir- ing interventional management are cared for at Rady Children’s Hospital. The primary objective of this retrospective study was to evaluate the prenatal detection rate of critical outflow tract anomalies in neonates at Rady Children’s Hospital with critical outflow tract CHD before and after theoretical implementation of the 2013 updated obstetrical imaging guidelines. Ideally, the majority of neonates with critical outflow tract CHD should have been detected by prenatal ventricular outflow tract imaging on obstetrical ultrasound. 3 | METHODS 3.1 | Study design Infants with critical CHD requiring cardiac catheterization and/ or surgical procedure(s) in the first 3 months of life were retro- spectively identified through Rady Children’s Hospital cardiac and electronic medical record databases. Infants were subdivided into two time frames; the pre‐guideline time frame consisted of infants requiring procedures between June 2010 and May 2013, while the post‐guideline time frame consisted of infants requiring pro- cedures between January 2015 and June 2016. The timeframe be- tween June 2013 and December 2014 was specifically excluded to allow for theoretical time necessary for obstetrical providers to FI G U R E 1 (A) Normal appearing cardiac four‐chamber view with abnormal parallel, (B) right ventricular, and (C) left ventricular outflow tracts in a fetus with D‐transposition of the great arteries. Abbreviations: RV, right ventricle; LV, left ventricle; RA, right atrium; LA, left atrium; Ao, aorta; PA, pulmonary artery
  • 3.     | 3SUN et al. implement the recommended guidelines into daily practice. Actual implementation of outflow tract imaging per the new guidelines was at the discretion of individual obstetrical practices; there is currently no universal training of all obstetrical sonographers given the large number of practices in San Diego county. Infants with the following cardiac outflow tract anomalies identifiable by outflow tract imaging were included: D‐TGA, cc‐ TGA, TOF, DORV, PA/IVS, truncus arteriosus, pulmonary stenosis, and aortic stenosis. Although cc‐TGA should also be detectable by the four‐chamber view, cc‐TGA was included in this study, as it may be unrealistic to expect the general obstetrical screening sonographer the nuance of differentiating the morphologic right versus left ventricle. Those with other forms of CHD potentially diagnosable by the four‐chamber view were excluded (ie atrio- ventricular canal defects or single ventricle lesions such as hypo- plastic right or left heart syndrome). Isolated aortic coarctation without aortic valve stenosis was also excluded as it is not reliably expected to be diagnosed prenatally. Rady Children’s Hospital also serves as the primary referral center for infants with CHD from Hawaii and the Pacific Islands; out‐of‐state patients were excluded. The study was approved by the Institutional Review Boards of Rady Children’s Hospital and University of California, San Diego. 3.2 | Statistical analysis Interval variables were expressed as mean  standard deviation or median (range). Categorical variables were counted and expressed as frequencies or percentages. Due to the non‐normality of most of our continuous study outcomes, the Mann‐Whitney U test was used as a robust rank‐based statistic to determine if there was a significant difference between pre‐guidelines and post‐guidelines or prenatal and postnatal diagnosis groups. Due to some small expected cell counts, Fisher’s exact test was used to compare categorical variables across groups. A p value less than .05 was considered statistically significant. All analyses were performed using the latest version of R software (version 3.3.2). 4 | RESULTS All but one of the mothers in each time frame had reportedly re- ceived standard prenatal care. There was no association between maternal race or type of health insurance and prenatal versus post- natal diagnosis (P = .99 and .41, respectively) (Table 1). Overall, prenatal diagnosis occurred in 55% of infants with crit- ical outflow tract anomalies. Prenatal diagnosis occurred at a mean age of 26.7 ± 4.8 weeks gestational age. Postnatal diagnosis of a critical outflow tract anomaly was mostly made on day of life 1 or 2 (range 1–71 d) (Table 2). The most common clinical findings leading to postnatal diagnosis of CHD were hypoxemia, murmur, or failed pulse oximetry screening. Seven percent of patients (11 of 153 new- borns) were discharged prior to diagnosis; none of these patients had ductal dependent lesions. Of the three most common defects, pre- natal diagnosis occurred in 54% of D‐TGA, 63% of TOF, and 80% of DORV patients (Figure 2). Fifty percent of the infants required transfer from the delivery hospital to our institution; the other infants were born at our ad- joining women’s hospital. Sixty‐three percent had ductal dependent lesions for systemic or pulmonary blood flow. Most infants (86%) required intervention prior to hospital discharge. Three infants died without procedural intervention due to withdrawal of care. The first cardiac intervention occurred at a median age of 6 days (range 0–88 d), with 51 infants undergoing only cardiac catheterization, 69 infants undergoing only surgical palliation/repair, and 33 infants un- dergoing both catheterization followed by surgical palliation/repair within the first 3 months. 4.1 | Prenatal versus postnatal diagnosis Prenatally diagnosed infants had a statistically significant slightly ear- lier gestational age at birth (37.7 ± 2.4 wk vs. 37.9 ± 3.4 wk in post- natally diagnosed infants, P = .03). Prenatally diagnosed infants had statistically lower birth weights (2.97 ± 0.69 kg vs. 3.18 ± 0.64 kg, P = .02) and 5‐minute Apgar scores (7.9 ± 1.6 vs. 8.4 ± 0.9, P = .01). There was a higher frequency of ductal dependent lesions in those Prenatal diagnosis n = 86 (%) Postnatal diagnosis n = 70 (%) p value Maternal race .99 White 34 (40%) 28 (40%) Hispanic 32 (37%) 26 (37%) Asian 10 (12%) 7 (10%) African American 3 (3%) 3 (4%) American Indian 0 1 (1%) Hawaiian 1 (1%) 0 Unknown/not reported 6 (7%) 5 (7%) Maternal insurance type .41 Private 53 (62%) 38 (54%) Public 33 (38%) 32 (46%) TA B LE 1 Maternal demographics, by timing of diagnosis
  • 4. 4  |     SUN et al. prenatally diagnosed (73% vs. 50% postnatally diagnosed, P = .004). There was no significant difference in degree of pre‐intervention lactic acidosis, need for pre‐intervention inotropic support, or num- ber of days to first intervention (Table 2). 4.2 | Pre‐guidelines vs. post‐guidelines Pre‐guideline revision, between June 2010 and May 2013, 102 of 424 infants with critical CHD had outflow tract anomalies poten- tially identifiable by outflow tract imaging which required cardiac catheterization or surgery within the first 3 months of life. Prenatal diagnosis occurred in 52% of the pre‐guidelines group. Post‐guide- lines, between January 2015 and June 2016, 54 of 176 infants with critical CHD had outflow tract anomalies which required cardiac catheterization or surgery within the first 3 months of life. Prenatal diagnosis occurred in 61% of the post‐guidelines group, not sig- nificantly different than the prenatal detection rate pre‐guide- lines (P = .31) (Table 3 and Figure 3). The types of cardiac outflow tract anomalies pre‐guidelines versus post‐guidelines were similar (Table 3 and Figure 4). The timing of diagnosis was also not signifi- cantly different based on type of cardiac defect pre‐guidelines vs. post‐guidelines (Table 4 and Figure 5). 5 | DISCUSSION Screening for congenital anomalies should occur during routine anatomic obstetrical ultrasound examination at approximately 18‐20 weeks gestational age in all pregnancies.14 The most com- mon congenital anomaly is CHD, affecting approximately 1% of live Prenatal diagnosis n = 84 Postnatal diagnosis n = 65 p value Timing of diagnosis Gestational age 26.7 ± 4.8 wk Day of life 1 (1−71) n/a Gestational age at birth (weeks) 37.7 ± 2.4 37.9 ± 3.4 .03 Birth weight (kg) 2.97 ± 0.69 3.18 ± 0.64 .02 Apgar score 1 min of life 6.9 ± 2.1 7.2 ± 1.9 .47 5 min of life 7.9 ± 1.6 8.4 ± 0.9 .01 Genetic syndrome and/or extracardiac anomaly 28 (33%) 14 (20%) .10 Ductal dependent 63 (73%) 35 (50%) .004 Highest pre‐intervention lactate 3.07 ± 2.05 4.30 ± 4.48 .08 Pre‐intervention inotropic support 21 (26%) 17 (26%) .99 Timing of first Intervention (day of life) 6 (0−88) 5 (0−84) .19 Continuous data are reported as mean ± SD or median (range), as appropriate, and categorical data are reported as count (percentage). TA B LE 2 Patient characteristics, by timing of diagnosis FI G U R E 2 Overall cardiac diagnoses and timing of diagnosis 010203040 NumberofinfantsAortic Stenosis D −TG A D O R V PA/IVS Pulm onary Stenosis TO F Truncus Arteriosus cc−TG A Cardiac Diagnoses and Timing of Diagnosis Postnatal Diagnosis Prenatal Diagnosis
  • 5.     | 5SUN et al. infants.1 Of these, approximately 25% have critical CHD requir- ing timely intervention to prevent significant infant morbidity or mortality.2 Although a multitude of maternal and/or fetal factors can prompt referrals for fetal ECHO to evaluate for the presence (or absence) of prenatal CHD,4 most CHD occurs in pregnancies without mater- nal or fetal risk factors.5 Referrals for fetal ECHO in these partic- ular cases rely solely on the identification of cardiac abnormalities on screening obstetrical ultrasound. Studies have shown that less experienced obstetric sonographers have lower detection rates of cardiac abnormalities on screening ultrasound.15 In the mid‐1990s, a single major referral center in New South Wales showed that ob- stetrics screening detected only 6.7% of ventricular outflow tract anomalies prenatally.16 In 1992, a study at a single obstetrics center in the US also demonstrated significant limitations to the four‐cham- ber view, with only 63% of all CHD detected, while the detection rate increased to 83% when ventricular outflow tract imaging was included.13 Outflow tract imaging is practically feasible when incor- porated into a standard screening protocol.17 Despite increased awareness and education, prenatal detection of various forms of CHD remains low,18,19 with outflow tract anom- alies historically lower than for other types of critical CHD (such as single ventricle lesions).20 Contemporary studies have shown a wide range of prenatal diagnosis rates for conotruncal anoma- lies (Table 5).21‒28 Many outflow tract anomalies can appear nor- mal when visualizing only the four‐chamber view (Figure 1A). The ability of obstetric anatomic ultrasound to identify outflow tract anomalies increases substantially when ventricular outflow tract imaging supplements the more traditional four‐chamber view.18,19,29 In June 2013, the American Institute of Ultrasound in Medicine officially recognized the importance of imaging ventricular outflow tracts by updating its guidelines on obstetric anatomic ultrasound to include this critical outflow tract view into its basic cardiac ex- amination.14 These guidelines (developed in conjunction with the American College of Radiology, American College of Obstetricians and Gynecologists, and the Society of Radiologists in Ultrasound) are the standard of care reference for obstetric practices in the United States. The International Society of Ultrasound in Obstetrics TA B LE 3 Patient characteristics, by time frame Pre‐guidelines n = 102 (%) Post‐guidelines n = 54 (%) p value Timing of diagnosis .31 Prenatal diagnosis 53 (52%) 33 (61%) Postnatal diagnosis 49 (48%) 21 (39%) Type of cardiac defect .97 D‐TGA 26 (26%) 13 (24%) TOF 19 (18%) 14 (26%) DORV 17 (16%) 9 (17%) Pulmonary stenosis 15 (15%) 6 (11%) Truncus arteriosus 10 (10%) 5 (9%) Aortic stenosis 8 (8%) 4 (7%) PA/IVS 6 (6%) 2 (4%) CC‐TGA 1 (1%) 1 (2%) Prenatal care (if postnatal diagnosis) .52 Yes 48 (98%) 20 (95%) No 1 (2%) 1 (5%) Gestational age (weeks) 38 ± 2.3 37.4 ± 3.8 .31 Birth weight (kg) 3.1 ± 0.7 3 ± 0.7 .33 FIGURE 3 Timing of diagnosis: pre‐guidelines versus ­ post‐guidelines
  • 6. 6  |     SUN et al. and Gynecology also published similar guidelines to include outflow tract views.30 Fetal ECHO performed by fetal/pediatric cardiac sonographers with interpretation by fetal/pediatric cardiologists provide a more accurate prenatal cardiac diagnosis31,32 as compared to obstetri- cal ultrasound. Fetal ECHO accurately detects simple and complex CHD,3 such as outflow tract anomalies,33‒35 and allows for directed prenatal parental counseling as well as delivery management in an appropriate setting.36 The most common outflow tract anomalies, such as TOF, d‐TGA, DORV with pulmonary stenosis, or DORV with malposed great arteries, can cause significant cyanosis and hypoxia after birth leading to poor neonatal outcomes if not appropriately treated in a timely manner; the long‐term survival rates are high if appropriately treated. Prenatal detection with timely post‐delivery access to cardiac medical and surgical management improves mor- tality rates and outcomes.12,35,37,38 The overall prenatal detection rate of 55% in this study remains low in the modern era of specialty medicine capable of prenatally diagnosing and managing critical cardiac outflow tract anomalies. Despite the official revised obstetrical ultrasound guidelines, this study shows no significant difference in the prenatal diagnosis rate of these critical outflow tract anomalies following guideline revision. The limitation stems primarily from lack of obstetrical identification of abnormalities—presumably from inadequate imaging of the ven- tricular outflow tracts on routine anatomic screening ultrasounds. This study supports the need for more resources dedicated to ed- ucating obstetrical sonographers and practitioners in optimizing imaging of ventricular outflow tracts and/or encouraging referrals for fetal echocardiograms if unable to confidently confirm normal outflow tracts. In this study, prenatally diagnosed infants had a statistically sig- nificant earlier gestational age at birth, although the majority were still born at or near term. We speculate that prenatally detected CHD placed these pregnancies into a higher risk category and may have prompted increased pregnancy and fetal monitoring and a lower threshold for induction or delivery. Prenatally diagnosed infants also had slightly lower birth weights and 5‐minute Apgar scores, which may have been related to their earlier gestational age at birth. The majority of infants had ductal dependent lesions and/or required intervention prior to hospital discharge, indicating high risk for hemodynamic com- promise if not diagnosed and treated in an expeditious manner. No differences were seen in pre‐operative lactic acidosis, need for inotropic support, or number of days to first proce- dure between the prenatal and postnatal diagnosis groups. The FI G U R E 4 Cardiac diagnoses: pre‐guidelines and post‐guidelines Pre‐guideline % (# prenatal diagnosis/total) Post‐guideline % (# prenatal diagnosis/total) p value D‐TGA 50% (13/26) 61% (8/13) .73 TOF 58% (11/19) 71% (10/14) .48 DORV 76% (13/17) 88% (8/9) .62 Pulmonary stenosis 27% (4/15) 50% (3/6) .35 Truncus arteriosus 50% (5/10) 40% (2/5) .99 Aortic stenosis 50% (4/8) 0% (0/4) .21 PA/IVS 33% (2/6) 50% (1/2) .99 TA B LE 4 Prenatal detection rate of cardiac diagnoses, by time frame
  • 7.     | 7SUN et al. FI G U R E 5 Timing of diagnosis by cardiac diagnoses: (A) Pre‐Guidelines and (B) Post‐Guidelines 0510152025 NumberofinfantsAortic Stenosis D −TG A D O R V PA/IVS Pulm onary Stenosis TO F Truncus Arteriosus cc−TG A Pre−Guidelines Postnatal Diagnosis Prenatal Diagnosis 051015 NumberofinfantsAortic Stenosis D −TG A D O R V PA/IVS Pulm onary Stenosis TO F Truncus Arteriosus cc−TG A Post−Guidelines Postnatal Diagnosis Prenatal Diagnosis A B TA B LE 5 Prenatal diagnosis rates of conotruncal anomalies from recent studies Location Author Years Number of patients Prenatal diagnosis rate (%) D‐TGA Texas Lara 1999−2007 468 10 Czech Republic Marek 2000−2006 223 25 Netherlands van Velzen 2002−2012 172 36 Paris Khoshnood 2005−2008 85 70 Belgium De Groote 2006−2014 79 24 TOF Czech Republic Marek 2000−2006 142 37 Netherlands van Velzen 2002−2012 111 30 Paris Khoshnood 2005−2008 60 68 Belgium De Groote 2006−2014 117 23 Truncus arteriosus Boston Swanson 1992−2007 136 32 Czech Republic Marek 2000−2006 47 80 Netherlands van Velzen 2002−2012 46 62 PA/IVS Italy Tuo 1993−2009 60 60 Czech Republic Marek 2000−2006 88 68 cc‐TGA Toronto Wan 1999−2006 54 29
  • 8. 8  |     SUN et al. heterogeneous nature of cardiac diagnoses in the two groups lim- ited the power to detect statistical differences and draw conclu- sions between pre‐intervention course between the prenatal and postnatal diagnosis groups. Prenatal diagnosis of critical CHD has been shown to decrease preoperative mortality in other studies.12 6 | LIMITATIONS Given the large number of hospitals delivering infants in San Diego county, infants with postnatal diagnosis of critical CHD that did not survive to admission to our hospital were not able to be identified, as it was outside the scope of our study. Theoretically, the addition of these infants would further decrease the prenatal detection rate in both timeframes. This study did not aim to encompass all critical CHD, only those detectable by outflow tract imaging. Other critical CHD, such as sin- gle ventricle lesions and isolated aortic arch anomalies, were spe- cifically excluded as the authors aimed to highlight the importance of obstetric screening ultrasounds incorporating adequate outflow tract imaging. 7 | CONCLUSIONS Despite revised obstetrical guidelines highlighting the importance of outflow tract imaging, referrals and prenatal diagnosis of these types of critical CHD remain suboptimal.  Education of obstetri- cal sonographers and practitioners who perform fetal anatomic screening is vital to increase referrals for fetal cardiac evalu- ation and optimize prenatal detection of critical outflow tract anomalies. ACKNOWLEDGMENTS The project was partially supported by the National Institutes of Health, Grant UL1TR001442 of CTSA funding. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. DISCLOSURE STATEMENT The authors report no conflicts of interest. AUTHOR CONTRIBUTIONS All authors have read and approved the final version of the manuscript. Concept/Design, Data collection/interpretation, Drafting/revision of article: Sun Statistics: Proudfoot Critical revision of article: McCandless ORCID Heather Y. Sun  http://orcid.org/0000-0001-9309-3565 REFERENCES 1. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39:1890–1900. 2. Oster ME, Lee KA, Honein MA, Riehle‐Colarusso T, Shin M, Correa A. Temporal trends in survival among infants with critical congeni- tal heart defects. Pediatrics. 2013;131:e1502–e1508. 3. Berkley EM, Goens MB, Karr S, Rappaport V. Utility of fetal echo- cardiography in postnatal management of infants with prenatally di- agnosed congenital heart disease. Prenat Diagn. 2009;29:654–658. 4. Donofrio MT, Moon‐Grady AJ, Hornberger LK, et al. 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