TAGEDENS E M I N A R S I N P E R I N A T O L O G Y 46 (2022) 151584

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Seminars in Perinatology
www.seminperinat.com

Coarctation of the aorta: Prenatal assessment,

postnatal management and neonatal outcomes
Bhawna Aryaa,*, and Shiraz A. Maskatiab
a
University of Washington School of Medicine, Seattle Children’s Hospital, Seattle, WA, United States
b
Stanford University School of Medicine, Lucile Packard Children’s Hospital, Stanford, CA, United States

A R T I C L E I N F O AB STR ACT

Keywords: Coarctation of the aorta (Coa) is a potentially life threatening diagnosis. It occurs in 0.3 per
Coarctation of the aorta 1000 live births and accounts for 6-8% of all infants with congenital heart defects. Neonates
Aortic arch hypoplasia with severe Coa may be completely asymptomatic at birth, as the ductus arteriosus can
Fetal Cardiology provide flow to the lower body. Those who are not diagnosed prenatally may be diagnosed
critical coarctation of the aorta only after constriction of the ductus arteriosus, when they present in cardiogenic shock.
This group has a higher risk for mortality and morbidity relative to those diagnosed prena-
tally. Despite the increasing practice of universal pulse oximetry screening, many cases
with significant coarctation of the aorta still go undiagnosed in the newborn period. In this
article, we present the pathophysiology, diagnosis, presentation, treatment and outcomes
of Coa.
Published by Elsevier Inc.

right and left pulmonary arteries bilaterally, while the left

Pathophysiology and natural history
Various segments of the aortic arch develop from different
embryologic origins. The embryologic truncus arteriosus
gives rise to the ascending aorta and the pulmonary trunk.
The left horn of the aortic sac gives rise to the segment of
the ascending aorta distal to truncus arteriosus, proximal
to the brachiocephalic trunk, while the right horn gives
rise to the brachiocephalic trunk itself. Paired pharyngeal
arches develop from the aortic sac, and give rise to various
segments of the aortic arch and major head and neck ves-
sels. The third aortic arches give rise to the bilateral com-
mon and internal carotid arteries. The right fourth aortic
arch gives rise to the proximal right subclavian artery,
while the left fourth arch gives rise to the segment of
transverse aortic arch between the carotid and the subcla-
vian artery. The sixth arches give rise to the proximal
sixth arch also gives rise to the ductus arteriosus. The left
sixth arch connects to the aorta immediately distal to the
origin of the seventh intersegmental artery. Upon comple-
tion of cardiac formation, the segments of the normal aor-
tic arch, from proximal to distal are: the ascending aorta,
the proximal transverse aortic arch (between the origin of
the brachiocephalic trunk and the left common carotid
artery), the distal transverse aortic arch (between the left
common carotid artery and the left subclavian artery) and
the aortic isthmus (between the left subclavian artery and
the ductus arteriosus) (Fig. 1). Because the ductus arterio-
sus is capable of providing all systemic blood flow to the
lower body, the aortic isthmus only carries approximately
20-30% of the systemic cardiac output in normal fetal
hearts.1,2 This aortic isthmus is therefore a watershed
area, at highest risk for poor development resulting in
anatomic narrowing.

*Corresponding author.
E-mail address: Bhawna.Arya@seattlechildrens.org (B. Arya).

https://doi.org/10.1016/j.semperi.2022.151584
0146-0005/Published by Elsevier Inc.
2 S E M I N A R S I N P E R I N A T O L O G Y
Fig. 1 – This figure depicts the various embryologic origins of
the aortic arch and major arterial structures. Embryologic
origins include the truncus arteriosus and aortic sac (red),
third pharyngeal arch (purple), fourth pharyngeal arch
(green), sixth pharyngeal arch (blue), intersegmental arter-
ies (orange), dorsal aorta (maroon). The airway (grey) and
esophagus (yellow) are also depicted. Of note, color image is
only available in the online format.
Coarctation of the aorta remains one of the most difficult
cardiac defects to diagnose prenatally.3 Accurate prenatal
diagnosis of congenital heart disease requires detection of an
abnormality on screening obstetric anatomy ultrasound, as
most occur in pregnancies without maternal or fetal risk
factors4,5 and remains as low as 55% in the current era.6 The
prenatal detection of CoA is particularly challenging, as stan-
dard obstetric anatomy scan protocols for cardiac imaging
(cardiac four-chamber and outflow views) do not require
assessment of the aortic arch.3,6 Even when the aortic arch is
profiled in the sagittal and three-vessel views, accurate mea-
surement is difficult due to the presence of the ductus arterio-
sus in fetal circulation, which can mask aortic isthmus
narrowing.7,8 There are subtle findings in the standard four-
chamber and outflow view which predict a high risk for Coa
on routine fetal anatomy scan and should prompt referral for
fetal echocardiogram.8
Prenatal diagnosis of Coa allows for delivery in a center that
can initiate prostaglandin infusion to maintain ductal
patency and perform neonatal surgical repair. When Coa is
not detected prenatally, a thorough neonatal physical exam
or pulse oximetry screening may raise suspicion and result in
diagnostic transthoracic echocardiogram. Failure to detect
46 (2022) 151584
CoA prenatally or at birth can result in closure of the ductus
arteriosus and insufficient post-ductal systemic blood flow.
These infants present with poor distal perfusion and acidosis,
increased left ventricular afterload and ultimately cardio-
genic shock with end-organ damage.9 They are at risk for con-
siderable morbidity and mortality.10 Less severe cases may
remain asymptomatic until they are diagnosed with hyper-
tension.
Prenatal assessment
As early as the 1960’s a there has been recognition that anom-
alies of left sided structures—including the pulmonary veins,
mitral valve, left ventricle and aortic valve—cluster with
coarctation of the aorta.11-14 This clustering is perhaps best
exemplified in the collection of findings known as Shone’s
syndrome: parachute mitral valve, supravalvar mitral ring,
subaortic stenosis and Coa).13 The pathophysiology underly-
ing these associations is multifactorial, and the interplay
between genetic underpinning of cardiovascular develop-
ment, molecular signatures of congenital heart disease and
flow mediated vascular development continue to be
explored.15
In fetal life, ventricular size discrepancy, with dominance
of the right-sided structures relative to left-sided structures
has long been associated with the development of Coa
(Figure 2a).7,8 Ratios of the mitral to tricuspid valve dimen-
sions, left to right ventricular dimensions, aortic valve to pul-
monary valve dimensions, and aorta to main pulmonary
artery ratios have all been associated with neonatal surgery
for Coa, with mean values different between groups.8,16-19
These findings provide valuable insight into the pathophysi-
ology and risk factors associated development of Coa.20 How-
ever, the clinical utility of these ratios remain limited.
Receiver operative characteristic areas under the curve (AUC)
for the development of surgical coarctation are 0.67-0.69 for
mitral to tricuspid valve dimension ratios.17,18 Right to left
ventricular dimension and area ratios demonstrated similarly
poor discriminatory ability.16,17,19 The ratio of the aorta to
main pulmonary artery dimension, although different
between surgical and non-surgical groups, also has subopti-
mal discriminatory ability, with AUC of 0.71.18,19 Although
ventricular size discrepancy is associated with the develop-
ment of Coa, it should be used as an indication for fetal echo-
cardiography, and not as a discriminator to identify cases of
surgical Coa.
In addition to the associations noted above with other left-
sided structural anomalies, Coa can also be associated with
various other congenital heart defects. In large, postnatal
series of patients with Coa, bicuspid aortic valve is by far the
most prevalent associated defect.21,22 Although helpful post-
natally, it is rare that a fetus is identified with isolated bicus-
pid aortic valve prenatally. However, if an aortic valve is
noted to be thickened, stenotic, or otherwise abnormal, care-
ful evaluation of the aortic arch is warranted. Similarly,
mitral valve anomalies—particularly those resulting in less
flow through the left side of the heart—are quite common in
fetal and postnatal series of Coa.21,23 Persistent left superior
vena cavae also appear to be more common in those with
 TAGEDENS E M I N A R S I N P E R I N A T O L O G Y 46 (2022) 151584 3

Fig. 2 – Abnormal fetal echocardiogram demonstrating (A) Coronal view of the four-chamber heart with right ventricular and
tricuspid valve dilation and borderline small mitral valve and left ventricle. (B) Sagittal view of the aortic arch with transverse
aorta hypoplasia and increased distance between second and third head vessels. (C) Three-vessel view of the great vessels
with size discrepancy between ascending aorta and pulmonary artery and tapering of the transverse and distal aortic arch.

diagnosed aortic coarctation (4-30%), although this relation- have neonatal CoA.17,18,32 Similarly, in the outflow tract view,
ship is somewhat unclear, as the incidence of this finding in the aortic valve z-score may be smaller and the pulmonary
the general population is difficult to elucidate.17,21,24 It is also valve diameter z-score larger.17,18,32 Finally, fetuses with con-
helpful to note which intracardiac defects carry particularly firmed CoA have a smaller ascending aorta and aortic isth-
high risk for development of Coa. Identification of these mus z-score in the sagittal and three-vessel trachea views
defects should also prompt careful interrogation of the aortic prenatally (Fig. 2b and c).16-18,32 There is also utility in calcu-
arch. Ventricular septal defects (8-13%), atrioventricular sep- lating the ratios of specific absolute diameters. Namely, an
tal defects (complete atrioventricular canal, 3-4%), double RV/LV ratio of greater than one and mitral valve/tricuspid
outlet right ventricle (1-9%), transposition of the great arteries valve ratio, aortic isthmus/ductus arteriosus ratio, and the
(2-8%), and double inlet left ventricle with ventricular arterial ascending aorta/main pulmonary artery ratio of less than one
discordance (1-4%) are common in series of Coa.21,22,24 While are predictive of CoA.33
a fetus with one of these defects may not warrant delivery at While these methodologies result in improved prenatal
a cardiac surgical center or admission to the neonatal inten- detection of CoA, the false positive rate has remained high,
sive care unit on the basis of the primary defect alone, con- upwards of 40-60%. Novel metrics aim to maintain the sensi-
cern about the risk of developing Coa with postnatal ductal tivity of prenatal detection while improving on specificity.
constriction may influence postnatal management. Similar to These include aortic arch morphometric measures such as
cases of ventricular size discrepancy or an associated left- increased distance between the second and third head and
sided structural anomaly, a cardiovascular evaluation of a neck vessel, as well as the absolute angles formed by the
fetus with one of these lesions should include careful evalua- descending aorta and surrounding structures (the ductus
tion of the aortic arch. arteriosus, ascending and transverse aorta).16,34 In conjunc-
Other, non-cardiac disease states also increase the risk for tion with standard measures, an acute ascending to descend-
Coa. The classic genetic association is with Monosomy X ing aorta angle of less than 21o, an obtuse transverse
(Turner syndrome), which occurs in 5-12% of females with descending aorta angle of greater than 96o improve the false
Coa; up to 35% of infants with Monosomy X have Coa.25,26
Less statistically significant, but still important associations
exist with Kabuki and Williams syndrome. Non-cardiac struc-
tural diseases associated with Coa include congenital dia- Table 1 – Fetal echocardiographic parameters for Coa
phragmatic hernia, with a reported incidence of 2-3%.27,28 assessment.
While there are likely multiple physiologic processes at play,
Fetal Echocardiographic Parameter Abnormal Value
there is evidence that increasing mass effect on the left heart Right ventricular to left ventricular Greater than 1.5 to 1
in this condition can result in reduced flow through the left ratio
side and predispose fetuses to develop Coa.29,30 Interestingly, Mitral valve z-score Less than -2
this relationship does not appear to be present in left sided Aortic valve Less than -2
lung masses, which develop later in gestation, after much of Ascending aorta z-score Less than -1.8
Aortic Isthmus z-score (sagittal view) Less than -2.4
cardiac development has occurred.31
Mitral to tricuspid valve ratio Less than 1
Several fetal echocardiographic parameters are useful for
Ascending aorta to main pulmonary Less than 1
accurate identification of CoA. Some are classified as stan- artery ratio
dard metrics, and include gestational age-adjusted z-scores Aortic isthmus to ductus ratio Less than 1
of the largest diameters of specific cardiac structures and Ascending to descending aorta angle Less than 20 degrees
their associated ratios (Table 1). In the four-chamber view, Transverse to descending aorta Greater than 96 degrees
the mitral valve z-score may be smaller (less than -2) and the angle
Descending aorta to ductal angle 82-125 degrees
tricuspid valve z-score is often larger in fetuses confirmed to
4 S E M I N A R S I N P E R I N A T O L O G Y
positive rate of prenatal. Color and spectral Doppler assess-
ment play a role in fetal diagnosis of Coa; the presence of a
left to right foramen ovale shunt or flow acceleration with
diastolic continuation and/or retrograde flow in the aortic
arch increase the likelihood that Coa is present when the sus-
picion is raised.8,35 More recently, advance imaging modali-
ties such as computational fluid dynamics36 and fetal cardiac
magnetic resonance imaging demonstrate utility in under-
standing the geometry and hemodynamics of the fetal aortic
ach.37 Cardiac magnetic resonance data suggests that prena-
tal Coa is associated with decreased flow in the ascending
aorta and aortic arch. Despite these advances, the false posi-
tive rate for fetal diagnosis of Coa remains unchanged.
Historically, fetal cardiologists perform multiple fetal echo-
cardiogram through gestation once there is concern for Coa.
Sometimes, multiple fetal echocardiograms are necessary if
there is concern for evolving hypoplasia of other left heart
structures. In cases of isolated fetal Coa, multiple echocardio-
grams may be performed in order to improve diagnostic accu-
raty. However, it is important to understand that the
limitations of prenatal Coa are related to poor prediction of
the changing physiology from a fetus to a neonate as it per-
tains to the ductus arteriosus and isthmus. Recognizing the
challenges of accurate diagnosis of Coa in the fetus, the cost-
effectiveness of multiple fetal echocardiographic assess-
ments through a gestation, recognizing the risks of a missed
diagnosis and socioeconomic burden of a false positive.
When a concern for Coa is raised on fetal echocardiogram,
subsequent fetal echocardiogram do not improve diagnostic
accuracy, but do increase cost. Thus, once a diagnosis of Coa
is made in the fetus, the most cost-effective management
strategy is a postnatal clinical assessment for Coa and post-
natal transthoracic echocardiogram to evaluate the aortic
arch shortly after birth.38 Realizing parental stressors and
need to finalized delivery plans, it is reasonable to perform a
second fetal echocardiogram in the third trimester, around
34-36 weeks, for confirmation of the degree of Coa concern.
Perinatal management
Once the concern for fetal coarctation of the aorta has been
raised, a cascade of events occurs. As with diagnosis of any
congenital heart defect, maternal anxiety increases, resulting
in increased risk for peripartum depression, traumatic stress
and decreased happiness about being pregnant.39 Further
compounding this increased stress and anxiety is the inher-
ent uncertainty that accompanies a fetal diagnosis of Coa.
Fetal identification of Coa, however, does appear to be associ-
ated with reduced postnatal morbidity. The prenatal team is
therefore wary of failing to identify cases of Coa and may
diagnose fetuses that demonstrate minor, non-specific find-
ings with Coa. This binary approach results in false positive
rates of up to 66%, particularly if ventricular size discrepancy
alone is used to make the diagnosis.40 This highlights the
importance of careful assessments to optimize both specific-
ity and sensitivity as much as possible.
Upon delivery, this period of uncertainty continues. If the
first echocardiogram is performed within the first few hours
after delivery, the presence of the ductus arteriosus will
46 (2022) 151584
obscure the anatomy of the aortic isthmus. As the ductus
arteriosus can provide blood flow to the lower body, reduced
flow through the transverse aortic arch and isthmus pre-
cludes the use of a pressure gradient to diagnose Coa. Even
after spontaneous closure of the ductus arteriosus, residual
ductal tissue at the level of the aortic isthmus can further
constrict the aortic arch, resulting in clinically significant
obstruction. Although this period of uncertainty is inherently
unavoidable, the initial echocardiogram can be quite useful.
A transthoracic echocardiogram is not subject to the same
limited acoustic windows of many fetal echocardiograms.
Many of the same predictive measurements used during fetal
life are quite powerful when applied in the neonatal period.41
When the prenatal or postnatal suspicion for Coa is high
while the ductus arteriosus remains patent, venous access
(peripheral or central) is obtained and intravenous prostra-
glandin E1 is initiated. These patients are transferred to a
neonatal or cardiac intensive care unit away from their moth-
er’s bedside. Once the diagnosis is confirmed by transthoracic
echocardiogram, neonates with Coa remain in the intensive
care unit until intervention for continuous infusion of prosta-
glandin E1, but also for clinical monitoring. Central access,
via umbilical venous catheter or peripheral intravenous cen-
tral catheter is obtained for infusions and frequent blood test-
ing. Because neonates with aortic arch obstruction are at
increased risk for poor intestinal perfusion and necrotizing
enterocolitis, the volume of milk fed to the newborn is lim-
ited, necessitating total parenteral nutrition. An arterial cath-
eter is placed for continuous blood pressure monitoring in
conjunction with at least daily 4-extremity cuff blood pres-
sures. Risks associated with instrumentation of these large
vessels include infection, bleeding and thrombus formation.
Pre- and post-ductal oxygen saturations45,46 along with cere-
bral (pre-ductal) and renal (post-ductal) near infrared spec-
troscopy are monitored continuously in the intensive care
unit.44 Arterial blood gases and lactates are collected at a
minimum of once daily as markers for evolving end-organ
damage. Prostaglandin E1 infusion is associated with apnea
and fever, which can result in escalating respiratory support
or work-up for neonatal sepsis, respectively. For the neonate
with moderate or high Coa concern, the clinical team may
monitor in the CICU without initiation of prostaglandin. In
these cases, multiple transthoracic echocardiograms are
often performed prior to complete ductus arteriosus closure
to ensure that no intervention is necessary. If the aortic arch
anatomy is unusual or echocardiographic imaging does not
definitively demonstrate the extent of Coa, advanced cross
sectional imaging such a cardiac computed tomography is
performed.
The above challenges prompted the development of a
tiered clinical management pathway for the perinatal man-
agement of fetuses identified prenatally with increased con-
cern for Coa.24 Fetuses were stratified into those with mild-,
moderate-, or high-risk for development of Coa relative to the
general population (Table 2). Fetuses in the mild-risk category
had mild aortic isthmus or transverse aortic arch hypoplasia,
and did not meet criteria for the moderate-risk category.
Moderate-risk criteria included isthmus narrowing and other
anatomic features consistent with development of COA.
High-risk criteria included moderate-risk anatomic features
 TAGEDENS E M I N A R S I N P E R I N A T O L O G Y 46 (2022) 151584 5

Table 2 – Prenatal risk stratification for development of aortic coarctation.

Mild Moderate High

 Aortic isthmus or transverse arch z-score < -2  Aortic isthmus z-score < -3 OR  Moderate-risk criteria with:
and > -3  Aortic isthmus z-score < -2 or isthmus:ductus  Any retrograde flow in the aortic arch or
 sthmus:ductus ratio < 0.7 ratio < 0.7 with one of the following:  Any left to right PFO flow
- Combined aortic and mitral valve z-score < -4
- RV/LV width ratio >2
- MPA/Asc Ao diameter ratio > 2
- Carotid subclavian index < 0.7
- Identifiable posterior shelf
- CHD which increases risk for coarctation*

*VSD, AVSD, DORV with subpulmonary VSD, aortic valve stenosis, TGA (VSD).

plus physiologic features associated with development of intervention is necessary. If the aortic arch anatomy is
postnatal COA. These included any aortic arch flow reversal unusual or echocardiographic imaging does not definitively
or any foramen ovale left-to-right flow, including bidirec- demonstrate the extent of Coa, advanced cross sectional imag-
tional flow at these levels. Mild-risk criteria were designed to ing such a cardiac computed tomography may be performed.
be sensitive, while high-risk criteria were designed to be spe-
cific. A standard delivery and postnatal plan was designed for
each group, allowing low risk neonates to have close monitor-
Criteria for intervention
ing without immediate medical interventions and for high
risk patient to be delivered with postnatal Coa management.
In the current era, despite widespread adoption of fetal echo-
Of 34 fetuses designated as mild risk, only one underwent
cardiography, coarctation of the aorta unfortunately often
surgery for Coa. This fetus had a coincident diagnosis of
remains undiagnosed prior to birth. In this setting, the pre-
Ebstein’s anomaly, and therefore was already slated to deliver
sentation of neonatal coarctation varies with the severity of
at a surgical center and was admitted to the neonatal inten-
the disease. Neonates may present with a systolic ejection
sive care unit upon delivery. Additionally, 30 (88%) mild-risk
murmur as the lone finding if the narrowing is mild in sever-
fetuses had a completely normal aortic arch without any
ity. However, with increasing severity, the presentation can
degree of narrowing. However, among 19 fetuses designated
extend to diminished or absent pulses, diminished cardiac
as high-risk, only 12 (63%) underwent surgery for Coa. The
output, poor end organ perfusion, and cardiogenic shock.
use of a management pathway based on the above tiered
Cases such as this present in the neonatal period, soon after
diagnostic algorithm resulted in reduced delivery location
spontaneous closure of the ductus arteriosus. Administration
changes and instrumentation in infants who did not have
of prostaglandin E1 via a continuous infusion is life-saving,
surgical Coa, with trends towards shorter hospital stays,
and these patients undergo surgery in the neonatal period to
shorter intensive care unit stays and time to enteral feeding.
repair the aortic arch prior to leaving the hospital. However,
Further work is needed to optimize specificity in the moder-
the timing of surgery in less severe cases of coarctation are
ate- and high-risk group; the false positive rate in this group
less clear. Patients with untreated coarctation of the aorta are
remained high at 37%. Based on the above data, we adjusted
at high risk for suffering a number of potential long-term com-
our postnatal management pathway with the aim of further
plications, including systemic hypertension, coronary artery
reducing unnecessary delivery location changes and medical
disease, cerebrovascular disease, heart failure and death. In
procedures. Fetuses designated mild-risk for Coa can be
fact, in adults with untreated significant Coa, the median age
safely delivered at the previously designated location, as long
of death is only 35 years old.45 It is in this setting that well-
as a postnatal echocardiogram can be arranged within the
established criteria for surgical or transcatheter intervention
first week of life. Those designated as moderate- or high-risk
were developed (Table 3). At many large scale surgical cen-
should deliver at a cardiac surgical center and admitted to
ters, these criteria are ascribed to the neonatal setting. Data
the neonatal intensive care unit for continuous monitoring.
on the importance of intervention in infancy or early child-
Continuous near infrared spectroscopy and pulse oximetry is
hood to prevent long-term systemic hypertension and the
used to surveil for hemodynamic deterioration. Umbilical
venous catheters are not empirically placed and prostaglan-
Table 3 – Indications for intervention (surgery or
din infusions are not empirically begun. The only difference catheterization)
in management between moderate and high-risk fetuses is
that high-risk fetuses undergo an echocardiogram with the Indications for intervention (surgery or catheterization)

results relayed directly to the intensive care unit physician
within the first 4 hours of delivery.
For the neonate with a borderline aortic arch, infants are
typically monitored inpatient (often in an intensive care set-
ting) without initiation of prostaglandin. In these cases, multi-
ple transthoracic echocardiograms are often performed prior
to complete ductus arteriosus closure to ensure that no
 Systolic upper to lower extremity blood pressure gradient > 20mmHg
on non-invasive blood pressure measurement, echocardiography or
cardiac catheterization
 Radiologic evidence of significant collateralization
 Loss of lower extremity pulses
 Systemic hypertension in the presence of significant coarctation
 Heart failure attributable to coarctation
6 S E M I N A R S I N P E R I N A T O L O G Y
low mortality rate in neonatal period support this
approach.46,47 However, in pre-term or particularly young
infants, the risk of intervention is higher and must be bal-
anced with the severity of coarctation.48
In the era of prenatal diagnosis of congenital heart disease,
there is a growing cohort of patients with concern for Coa.
The postnatal management of this group of patients is trou-
blesome, as infants will be clinically asymptomatic upon
delivery due to the presence of the ductus arteriosus. While
in some cases it seem obvious based on the fetal echocardio-
gram that surgery will be necessary and a prostaglandin infu-
sion should be begun to maintain systemic blood flow, this
scenario represents a minority of cases. In fact, even in fetal
cases with clear aortic arch hypoplasia, relative left-sided
hypoplasia and flow reversal in the aortic arch, the aortic
arch may be unobstructed upon spontaneous closure of the
ductus arteriosus after birth.24 Several clinical conundrums
exist in this setting due to the inherent uncertainty. The neo-
natologist, cardiologist and other members of the manage-
ment team must decide whether or not to: administer a
prostaglandin E1 infusion, place umbilical lines, feed the
infant, use near infrared spectroscopy monitors, and draw
arterial or venous blood gas samples. Preoperative imaging is
most often includes only the transthoracic echocardiogram,
but may include cross-sectional imaging (cardiac MRI or CT
angiography). However, despite the availability high-level
cardiovascular imaging, the uncertainty of the amount of
ductal tissue at the level of the aortic isthmus often necessi-
tates a period of hemodynamic monitoring of the infant off of
a prostaglandin infusion. With careful, non-invasive monitor-
ing, this period of uncertainty is typically low-risk, without
high risk for hemodynamic compromise.
Interventional Management
In the neonate, surgery remains the gold standard for Coa
repair. Surgical repair of Coa is usually performed via a left
lateral thoracotomy (without cardiopulmonary bypass) when
the stenosis is discrete or a short segment. The standard
approach is resection of the narrowed aorta followed by an
end-to end anastomosis.49 For longer segment narrowing, an
extended prosthetic patch aortoplasty is performed. The duc-
tal tissue is ligated and divided, a longitudinal incision is
made across the Coa and the prosthetic patch is sutured in
place to enlarge the stenotic region. This technique mini-
mizes aortic mobilization and avoids a circumferential suture
line, decreasing the risk for re-stenosis. The subclavian flap
approach to Coa repair, which was the original approach to
Coa repair in 1966 is rarely utilized in the current era, due to
the need to sacrifice the left subclavian artery and impaired
growth of the affected arm.50
Extensive aortic arch hypoplasia that requires augmenta-
tion of the transverse and distal aortic arch using patch mate-
rial is done via midline sternotomy procedures performed on
cardiopulmonary bypass.51,52 The most common technique
in infants and small children is an extended end-to-end anas-
tomosis, where a broader longitudinal incision and anasto-
mosis are created across the proximal aorta. This technique
demonstrates low mortality rates and restenosis rates of 4-
46 (2022) 151584
11%.53 The introduction of a patch material in surgical repair
of Coa may increase the risk for aneurysm formation, or alter-
natively, poor growth of non-native tissue and re-stenosis in
the future.54 Lifelong need for re-intervention is approxi-
mately 20-30%. The aortic arch advancement technique,
which involves a coarctectomy and end-to-side anastomosis
of the descending aorta to the distal ascending/proximal
transverse aorta attempts to utilize native tissue in the repair.
Mid-term follow-up (median 6 years) demonstrates need for
re-intervention in 3%.55
Post-operative complications of surgical repair of Coa include
bleeding, paradoxical hypertension, paraplegia, cerebral ische-
mia (in the case of extended aortoplasty), as well as injury to
structures surrounding the repair site. Recurrent laryngeal nerve
injury may result in vocal cord paresis/paralysis and injury to
the thoracic duct may result in chylothoraces.50 Long-term com-
plications, which can extend into adulthood include abnormali-
ties along the surgical site including re-coarctation or aneurysm
which can lead to dissection and/or rupture. The ascending
aorta, proximal to the repair site is also at risk for aneurysm for-
mation. Systemic hypertension is common into adulthood and
poor anti-hypertensive management can lead to Berry aneurysm
formation within the circle of Willis.50
Transcatheter repair of a native Coa can be performed with
balloon dilation, which stretches and tears the thickened inti-
mal “shelf” and adjacent layers. Limiting the damage to the Coa
layers is not guaranteed, and acute rupture and dissection can
rarely occur.56 This is particularly challenging in infants, and
therefore it is reserved for children over 6-12 months of age. In
this younger sub-group of infants less than 6 months of age,
risk for re-coarctation after native balloon dilation is increased
as well.57 A common risk after balloon dilation of Coa is aneu-
rysm formation over time.58 Stenting of native or recurrent Coa
can be performed in children and adults, furthermore, aneu-
rysm formation in the setting of surgically or interventionally
repaired Coa can be treated with stent placement. Unfortu-
nately, stenting of the aortic arch cannot be performed in
infants and very young children due to risk for vascular damage
in the setting of large catheterization sheaths.59
Conclusion
When undetected in the neonatal period, delayed diagnosis
of Coa is associated with significant morbidity and ultimately
can result in mortality. Despite improvements in fetal and
neonatal screening techniques, over a quarter of infants pres-
ent in extremis due to delayed diagnosis. Recognition of sub-
tle fetal cardiac findings on anatomy scan results in detailed
fetal echocardiographic assessment and risk stratification of
Coa risk. Appropriate delivery plans based on risk, along with
standardized postnatal monitoring and imaging allow for
improved diagnosis of Coa requiring intervention prior to
neonatal hospital discharge.
Conflict of interest statement
The authors report no proprietary or commercial interest in
any product mentioned or concept discussed in this article.
 TAGEDENS E M I N A R S I N P E R I N A T O L O G Y 46 (2022) 151584 7

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