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Review

Diagnosis, imaging and clinical management of

aortic coarctation
Elles J Dijkema,1 Tim Leiner,2 Heynric B Grotenhuis1
1
Department of Pediatric ABSTRACT narrowing is commonly located opposite to the
Cardiology, Wilhelmina Coarctation of the aorta (CoA ) is a well-known insertion of the PDA (juxtaductal), but may also be
Children’s Hospital, The
University of Utrecht, Utrecht, congenital heart disease (CHD) , which is often located proximal (preductal) or distal (postductal)
The Netherlands associated with several other cardiac and vascular to the PDA.4 8 The underlying pathophysiology of
2
Department of Radiology, anomalies, such as bicuspid aortic valve (BAV), CoA is not fully understood. Three pathophysiolog-
University Medical Centre ventricular septal defect, patent ductus arteriosus and ical theories exist:
Utrecht, Utrecht, The 1. Abnormal embryogenetic development.
aortic arch hypoplasia. Despite echocardiographic
Netherlands
screening, prenatal diagnosis of C o A remains difficult. 2. Reduced intrauterine blood flow through the
Correspondence to Most patients with CoA present in infancy with absent, aorta causing aortic underdevelopment.
Dr Heynric B Grotenhuis, delayed or reduced femoral pulses, a supine arm-leg 3. Aberrant PDA tissue in the aortic wall, which
Pediatric Cardiology, blood pressure gradient (> 20 mm Hg), or a murmur constricts the aortic lumen at the isthmus, with
Universitair Medisch Centrum due to rapid blood flow across the CoA or associated postnatal PDA regression.9
Utrecht-Locatie Wilhelmina Several genes have been implicated in CoA-ae-
Kinderziekenhuis, Utrecht 3508
lesions (BAV). Transthoracic echocardiography is the
AB, The Netherlands; ​H.​B.​ primary imaging modality for suspected CoA. However, tiology, including the NOTCH1 gene, which
Grotenhuis@​umcutrecht.​nl cardiac magnetic resonance imaging is the preferred plays an important role in cardiac development
advanced imaging modality for non-invasive diagnosis and vasculogenesis.10 CoA is also associated with
Received 11 January 2017 and follow-up of CoA. Adequate and timely diagnosis several syndromic phenotypes, such as Turner,
Revised 6 March 2017 of CoA is crucial for good prognosis, as early treatment PHACE, DiGeorge, Noonan and velocardiofacial
Accepted 15 March 2017 syndromes.11
Published Online First
is associated with lower risks of long-term morbidity
4 April 2017 and mortality. Numerous surgical and transcatheter
treatment strategies have been reported for CoA. FOETAL DIAGNOSIS
Surgical resection is the treatment of choice in neonates, Prenatal diagnosis of CoA reduces neonatal
infants and young children. In older children (> 25 kg) mortality and morbidity, prevents costly and risky
and adults, transcatheter treatment is the treatment of transfers and reduces parental stress, as it allows
choice. In the current era, patients with CoA continue to for planned delivery in dedicated paediatric cardiac
have a reduced life expectancy and an increased risk of centres.12 13 Prenatal ultrasound is a crucial primary
cardiovascular sequelae later in life, despite adequate screening modality.14 15 Foetuses with an abnormal
relief of the aortic stenosis. Intensive and adequate foetal heart or positive family risk factor(s) should
follow-up of the left ventricular function, valvular be referred for foetal echocardiographic exam-
function, blood pressure and the anatomy of the heart ination in a tertiary centre (figure 1).14 Adequate
and the aorta are , therefore, critical in the management examination of the foetal heart involves four
of CoA. This review provides an overview of the current recommended cardiac views: the three-chamber
state-of-the-art clinical diagnosis, diagnostic imaging and four-chamber views, and the left ventricle and
algori thms, treatment and follow-up of patients right ventricle outflow tract views.13 15 Despite
with CoA. echocardiographic screening, prenatal diagnosis
of CoA remains difficult.16 17 CoA is the most
INTRODUCTION commonly missed foetal CHD diagnosis, with
Coarctation of the aorta (CoA) accounts for 5%–8% less than one-third of the cases being detected at
of all congenital heart disease (CHD) and occurs prenatal screening.16 17 Concurrently, prenatal
in 4 out of 1000 live births with a male predomi- CoA screening is associated with a high number of
nance.1 2 CoA can occur as a solitary lesion, but is false-positives, especially late in gestation.12 Several
often associated with other cardiovascular lesions, echocardiographic markers may improve prenatal
such as bicuspid aortic valve (BAV) (50%–75%), detection rates and reduce false-positives, including
aortic arch hypoplasia, subaortic stenosis, mitral aortic arch z-scores, isthmus/ductal ratio, pulmonary
valve abnormalities, ventricular and atrial septal artery/aorta ratio and the presence of continuous
defects and patent ductus arteriosus (PDA).3 4 The diastolic flow at the level of the isthmus.16 18
most important non-cardiac associated lesion is
cerebral aneurysm in 2.5%–10% of patients with NEONATAL AND INFANTILE PRESENTATION
CoA.5 Most patients with CoA present in infancy
CoA is defined as a localised narrowing of the (table 1).19
aortic lumen by a ridge, composed of medial wall When the PDA closes after birth, severe CoA
thickening and infolding of aortic wall tissue.4 6 7 will lead to aortic obstruction with hypoperfusion
CoA is also considered a general arteriopathy, given of the lower body, renal dysfunction and metabolic
To cite: Dijkema EJ, Leiner T, the often abnormal histology of the arterial wall acidosis.4 Increased afterload on the left ventricle
Grotenhuis HB. Heart adjacent to the site of coarctation and its associa- (LV) may lead to LV failure.4 The presence of a
2017;103:1148–1155. tion with long-term cardiovascular pathology. The PDA may obscure the diagnosis of CoA until it
1148   Dijkema EJ, et al. Heart 2017;103:1148–1155. doi:10.1136/heartjnl-2017-311173
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pressure difference or lead to a gradient over the transverse
aortic arch, respectively.21
Neonates with clinically significant CoA should be hospital-
ised for continuous administration of prostaglandin E1, to keep
the PDA open.22 Patients with subtle clinical and/or diagnostic
signs require close clinical and echocardiographic observation to
determine whether PDA-closure results in clinically significant
CoA.22 Diagnosis of milder cases of CoA is important because
hypertension and compensatory LV hypertrophy due to increased
LV afterload may occur later in life.6

PRESENTATION AFTER THE NEONATAL PERIOD

Post-infantile symptoms of CoA mainly depend on the loca-
Figure 1  Foetal echocardiography in coarctation of the aorta: a tion and degree of CoA, the extent of collateral circulation and
shelf-like structure can be seen in (A) the ductal arch view and (B) two- presence of other CHD.4 Symptoms may be similar as for the
dimensional sagittal view of the aortic arch. AAo, ascending aorta; DA, neonatal period, but can be absent in case of extensive collat-
ductus arteriosus; DAo, descending aorta;  PA, pulmonary artery; RV, eral circulation.9 Adult patients with CoA classically present with
right ventricle. hypertension.9 Intracranial haemorrhage secondary to intra-
cranial aneurysms, LV hypertrophy and subsequent congestive
heart failure can also be seen in adult patients with undiagnosed
fully constricts, as aortic flow through the PDA may bypass the CoA.4 9
juxtaductal obstruction and supply output to the lower body.8
Classical findings of CoA on clinical examination are absent, IMAGING
delayed or reduced femoral pulses, a supine arm-leg blood pres-
Chest radiography
sure gradient (>20 mm Hg) and a murmur due to rapid blood
Despite low sensitivity, chest radiography can raise suspicion
flow across the CoA or associated lesions like BAV.8 20 Blood
for CoA.23 A leftward convexity of the descending aorta with
pressure readings should always include both arms, as an aber-
enlargement of the left subclavian artery can be seen in children
rant right subclavian artery (3%–4% of the patients with CoA)
and young adults with CoA, along with a figure-3 sign, formed
and diffuse aortic hypoplasia may obscure an arm-leg blood
by prestenotic and poststenotic dilatation of the descending
aorta.4 Rib notching of the posterior fourth to eigth ribs can be
seen in older patients, related to collateral blood flow through
Table 1  Recognising CoA in patients of all ages the intercostal arteries.4
Age Symptoms
Foetal Ventricular disproportion (RV>LV) Transthoracic and transesophageal echocardiography
Great vessel disproportio Transthoracic echocardiography (TTE) is the primary imaging
Continuous diastolic flow at isthmus level modality for suspected CoA, given its ready availability, safety
Associated congenital heart disease (BAV, and capacity to provide haemodynamic parameters such as
arch hypoplasia, subaortic stenosis, mitral
the CoA-gradient using Doppler (figure 2).5 In addition, TTE
valve abnormalities, VSD, ASD,PDA)
Syndromic phenotypes (Turner, PHACE,
can assess cardiac function and associated cardiac and valvular
DiGeorge, Noonan, velocardiofacial) abnormalities.4 Appropriate visualisation of the CoA-site can,
Neonatal and infantile Collapse, acidosis however, be difficult due to a poor acoustic window and oper-
Heart failure ator dependence.7 TTE also has limited value in the evaluation
Systolic/ continuous murmur of extracardiac structures and collateral circulation.4 5 7 Although
Weak or absent femoral pulses, radio- transesophageal echocardiography can provide accurate imaging
femoral pulse delay of the aorta, it is seldom used due to its invasive nature and
Supine arm-leg blood pressure gradient
limited additional value.23
Necrotizing enterocolitis
Renal failure
Cardiomyopathy (rare) Computed tomography
Childhood, adolescent, adult Systolic/ continuous murmur CT provides high spatial resolution data of both intracardiac
Weak or absent femoral pulses, radio- and extracardiac structures and allows for two-dimensional and
femoral delay three-dimensional reconstruction of relevant vascular anatomy.5
Supine arm-leg blood pressure gradient
Traditionally, the main disadvantage of CT has been the cumu-
Hypertension (headache, epistaxis,
retinopathy) lative radiation dose from repeat examinations, especially in the
Reduced exercise capacity, exercise paediatric population.4 23 The introduction of multidetector CT
induced hypertension with iterative reconstruction has significantly reduced radia-
Leg fatigue and claudication tion doses to values substantially below 1 mSv.7 24 Furthermore,
Cold feet current state-of-the-art CT scanners can obtain the entire volu-
Left ventricular hypertrophy, arrhythmia
metric data acquisition in a single or several cardiac cycles, which
and heart failure
Infective endocarditis
minimises motion artefacts and can eliminate the requirement
Aortic dissection, rupture for breath-holding.7 23 However, CT cannot provide haemo-
Intracranial haemorrhage dynamic information such as the pressure gradient across the
BAV, bicuspid aortic valve; LV, left ventricle; RV, right ventricle; VSD, ventricular CoA-site and the degree of collateral circulation, while the use
septal defect; ASD, atrial septal defect; PDA, patent ductus arteriosus. of iodinated contrast media is associated with nephropathy.4 5 23
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Figure 2  Transthoracic echocardiography with continuous Doppler showing high-velocity flow through the narrowed transverse arch and site of
coarctation in the suprasternal notch view (A+B). Low-velocity, continuous flow was observed in the abdominal aorta (C+D).

Cardiac magnetic resonance imaging Catheter angiography

Cardiac magnetic resonance (CMR) imaging is the preferred Catheter angiography is the gold standard for the assessment
advanced imaging modality for non-invasive diagnosis and of the pressure gradient across the CoA and provides high-res-
follow-up of CoA.5 25 A major advantage of CMR is the lack olution images of the aorta and aortic geometry in 3D space
of ionising radiation, making it ideally suitable for repeated (figure 4).5 Catheter angiography also allows for accurate assess-
imaging. Three-dimensional gadolinium enhanced CMR angiog- ment of collateral circulation.4 Catheter angiography is the
raphy provides excellent depiction of the aortic morphology and traditional reference standard for CoA imaging, but the invasive
the location and degree of stenosis, as well as the extent of collat- nature and radiation burden are important drawbacks.4 5 With
eral vessel formation (figure 3).25 Importantly, sole measurement the emergence of CMR and CT, CA is currently preferred when
of aortic dimensions may not be sufficient to assess the degree of treatment options such as balloon angioplasty (BA) or stent
CoA severity, as the haemodynamic effect of CoA is influenced insertion are considered.4
by a complex interplay of aortic geometry, vessel wall mechanics,
flow and ventricular function.26 27 Therefore, more indirect
measurements of stenosis—including the CoA-gradient—are TREATMENT
often utilised.27 If left untreated, the mean survival for patients with CoA is
Cine imaging provides analysis of LV function and myocar- reduced to mid-adulthood due to long-term sequelae such as
dial mass, whereas phase contrast flow analysis provides an congestive heart failure, endocarditis, aortic rupture or cerebro-
estimated pressure gradient across the CoA and calculation of vascular haemorrhage.4 5 Life expectancy largely depends on the
collateral flow.23 25 Novel four-dimensional flow CMR allows severity of the stenosis and the presence of associated anoma-
for the assessment of flow velocities and pressure fields along lies.5 8 9 Indications for CoA intervention are listed in box 1.9 21
the aorta, and the pressure gradient across a CoA, wall-shear- Normotensive but significant CoA with substantial collat-
stress (WSS) and oscillatory shear index (OSI) can be calculated eral flow should also be considered for treatment, as collateral
using computational fluid dynamics.28 29 Despite these advan- flow reduces the perceived aortic gradient despite significant
tages, CMR is hampered by the long acquisition time and stenosis.8 9
need for breath-holds during scanning, which restricts its use Clinicians should aim for complete elimination of the aortic
in smaller children and claustrophobic patients.23 Sedation or pressure gradient to preserve systolic and diastolic ventricular
general anaesthesia may then be necessary. In addition, metal functions over time and prevent or at least reduce systemic
stent implantation causes CMR artefacts, which limits adequate hypertension.21 Early intervention is preferred, as older age
follow-up assessment.23 at the time of treatment is associated with increased risk of
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Figure 3  A 28-year-old man with known aortic coarctation. The examination was performed for follow-up reasons. Colour volume renderings in the
left anterior oblique (A) and right posterior (B) views show the coarctation in the proximal descending aorta and the post-stenotic dilatation (arrows
in A and B). Orthogonal reformations of the source data in the left anterior oblique (C), transverse (D) and right posterior (E) orientations show the
aortic narrowing and kinking in more detail and reveal a small web in the inner aortic curvature (asterisks in C, D and E).

hypertension, aneurysm formation and mortality.30 31 Timing re-CoA and involves sacrificing the left subclavian artery (main
of early intervention should, however, be weighed against the arterial blood supply to the left arm).9 CoA-resection and patch
higher risk of restenosis and reintervention.32 33 augmentation of the hypoplastic segment of the aortic arch
are, therefore, preferred.
Surgical repair In adults, resection of the CoA-segment with graft interposi-
In neonates, infants and young children, surgical resection of tion can be considered.9 A bypass graft across the CoA-segment
the CoA-segment is the treatment of choice.32 Several surgical can also be considered in case of complex CoA, extensive collat-
techniques have been described. The surgical approach of choice erals or other significant cardiac abnormalities in need of repair.9
is based on age and aortic arch anatomy.9 34 Extended CoA resec- Prosthetic-patch aortoplasty should be avoided due to high risk of
tion with end-to-end anastomosis through a left thoracotomy is aortic aneurysms and rupture.9 Surgical mortality in CoA-inter-
the preferred technique in neonates and children with discrete vention is rare (<1%).35 Early postoperative morbidity includes
CoA.32 In infants with long-segment CoA, subclavian-flap paradoxical hypertension, injury to the recurrent laryngeal nerve
aortoplasty through a median sternotomy can be performed.9 (or other adjacent nerves) and subclavian steal syndrome (with
However, subclavian-flap aortoplasty has a higher chance of subclavian patch angioplasty).9 Although very rare, spinal cord
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Stent placement
Endovascular stent placement provides a solution to elastic
tissue-recoil after BA.9 In older children (>25 kg) and adults,
transcatheter treatment is the treatment of choice because of
good results and the less invasive nature of this technique.9
Endovascular stenting is preferred over BA alone, with lower
risk of restenosis and aneurysm formation.38 Stent implanta-
tion in young children remains controversial due to the need
for frequent redilation to accommodate the growing aorta,
lack of available redilatable stents, high incidence of intimal
proliferation and restenosis and risk of poststent aneurysms.37
Recently, the usage of growth stents and biodegradable stents
in children has been described,39 40 although long-term results
of these new techniques have not been reported yet.8
With endovascular stenting, a stent is dilated with inflation
Figure 4  (A) Sub-atretic coarctation of the aorta in a 5-month-old of a balloon until the stent is anchored in the aorta and the
girl with a gradient of 50 mm Hg across the sub-atretic segment. (B) stenosis is sufficiently relieved (figure 4). Rapid ventricular
The aortic arch after stent implantation with complete reduction of the pacing can be used to lower the stroke volume and facilitate
aortic gradient. proper stent placement.21 Stent placement provides a more
sustainable relief of the CoA-gradient, with more even distri-
bution of forces across the vessel wall.9 The introduction of
ischaemia with paraplegia (due to prolonged clamping of the covered stents—as an alternative to bare-metal stents—has
aorta) has been reported.9 further reduced the risk of aneurysm formation.30 Despite
these advantages, stent insertion is technically more compli-
Balloon angioplasty cated than BA and requires a bigger catheterisation sheath,
BA is generally performed after 3–6 months of age because of resulting in higher risks of complications at the insertion site.21
high risk of re-CoA in younger patients due to reactive PDA Rare complications of stent placement include stent migration
tissue and the highly elastic properties of the aorta.36 BA can and/or embolisation, occlusion of aortic side branches with
be used in the neonatal period as palliative strategy in high- covered stents and aortic dissection and rupture, associated
risk patients (ie, small or premature neonates) to stabilise their with cerebrovascular accidents in very rare cases.21 Long-term
condition prior to definitive correction.8 37 With BA, a catheter complications include restenosis, stent fracture and aneurysm
is brought up to the CoA-segment. The balloon size is chosen formation.30
based on a maximum of 300% of the minimal aortic diam-
eter.21 To prevent aortic recoil, a therapeutic tear of the aortic FOLLOW-UP
wall into the media is created by overdilation of the vessel. Despite excellent long-term survival after CoA-repair,
After dilation, the restored blood pressure across the CoA-seg- patients have a reduced life expectancy with increased risk
ment causes aortic wall remodelling, intended to create a of  morbidity.33 41 Long-term complications occur despite
lasting result.8 21 Because of tearing of the aortic intima, BA adequate and timely repair, warranting life-long follow-up
predisposes for aortic dissection and rupture.21 Long-term (table 2). Annual blood pressure evaluation (four limbs) and
complications are recurrent stenosis and aneurysm formation.21 regular imaging of the heart and aorta are recommended.32 42
In the past, a high incidence of 20% for aneurysm formation
was reported for BA, but the risk is significantly reduced with
Re-stenosis and reintervention
technique-modifications, such as low-pressure, progressive or
Cardiovascular reinterventions are common (25%) among
stepwise BA and smaller balloon sizes.9 21 Re-CoA risks are rela-
patients with CoA.43 Indications for reintervention are re-CoA,
tively low for adults, but increase significantly with younger
aortic valve dysfunction and other cardiac malformations (ie,
age at intervention (>50% for patients <1 year of age).21
VSD).43 Recoarctation is seen in 4%–14% of the patients,

Box 1  Indication for treatment of native coarctation of Table 2  Long-term complications of CoA
the aorta (CoA) and re-CoA
Long-term complications

Indications for treatment: Local Recoarctation, aneurysm, dissection, rupture,

fistulae, endocarditis
►► Supine non-invasive pressure gradient >20 mm Hg between
Ascending aorta Aneurysm, dissection, rupture, sinus of Valsalva
upper and lower limbs fistula
►► Peak-to-peak coarctation gradient ≥20 mm Hg
Aortic valve (Bicuspid valve), stenosis, regurgitation
►► Peak to peak coarctation gradient <20 mm Hg with
Left ventricle  Hypertrophy, dilation, systolic dysfunction and/
radiological evidence of significant coarctation with or diastolic dysfunction, heart failure, sudden
significant collateral flow cardiac death
►► Pathological blood pressure response during exercise Coronaries Premature atherosclerosis, ischaemic heart
►► Significant left ventricular hypertrophy disease
►► Hypertension with ≥50% aortic narrowing relative to the Cerebral Berry aneurysms, intracranial bleeds,
aortic diameter at the level of the diaphragm atherosclerosis, stroke
►► Upper limb hypertension Systemic Hypertension, reduced exercise capacity

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Figure 5  A 26-year-old man with known aortic coarctation imaged four times over a 10-year period shows mildly progressive dilatation of the
poststenotic part of the proximal descending aorta over time (arrows). The study in A was obtained in 2007; the study in B was obtained in 2010; the
study in C was obtained in 2013 and the most recent follow-up examination was performed in 2016 (D).

caused by residual ductal tissue, scarring, unrelieved arch anastomosis.32 33 The exact pathophysiology of late-onset
hypoplasia and intimal hyperplasia after stenting.34 43 Repair hypertension after CoA-repair remains to be elucidated.
before 1 year of age carries a greater risk of re-CoA.32 Re-in- Reduced aortic compliance, abnormal baroreceptor function
terventions occur more frequently after stent placement or BA, and altered WSS dynamics may play a role.31 Hypertension is
but these reinterventions are often anticipated with growth also associated with an abnormal geometry of the aortic arch
of the child.30 Unplanned reintervention rates are similar for (‘Gothic arch’ configuration) when the arch is sharply angulated
surgical and transcatheter treatments.8 Indications for treat- in the sagittal plane.33 Mild residual morphological obstruc-
ment are similar as for native CoA.9 38 More than half of the tion and a disturbed renin–angiotensin–aldosterone system
patients with CoA suffer from a BAV, which is associated with appear to play no major role in late-onset hypertension.19
a high risk of aortic valve repair or replacement surgery during Post-treatment hypertension is a risk factor for premature
long-term follow-up.3 Indeed, half of all reinterventions in death and requires aggressive treatment.31 Follow-up should
patients with CoA are due to aortic valvulopathy, so assess- include 24 hours of blood pressure measurement and exercise
ment of aortic valve function should be part of follow-up.3 testing, as exercise-induced hypertension can predict future
systemic hypertension.31
Generalised vasculopathy and aneurysm formation
The prestenotic arch and conduit arteries of patients with Left ventricular function
CoA show abnormal vascular compliance due to reduced Patients with CoA may suffer from LV pressure overload,
smooth muscle tissue and increased collagen, leading to which can cause compensatory hypertrophy.3 Ventricular
increased intimal-media thickness.19 44 The prestenotic arch hypertrophy can lead to myocardial fibrosis, associated with
also demonstrates endothelial dysfunction, possibly related to LV systolic and diastolic dysfunctions.3 Histological studies
altered aortic flow dynamics.28 Associated increased WSS and have demonstrated predominantly subendocardial fibrosis
decreased OSI result in higher atherosclerotic susceptibility.45 of the LV, suggesting insufficient coronary flow (reserve)
Abnormal compliance and endothelial dysfunction can persist with compromised diastolic myocardial perfusion.3 Although
after CoA-repair and are associated with generalised arterial LV changes tend to normalise after CoA-repair, LV function
vasculopathy later in life.46 Vascular complications include remains subnormal in many patients.3 Older age at the time
aortic aneurysm, hypertension, aortic root dilation, aortic of repair is associated LV hypertrophy and increased LV
and mitral valve insufficiency, premature coronary artery mass.31 32 34 The ejection fraction and fractional shortening
disease and cerebrovascular disease (figure 5).32 42 Aortic may be preserved in the early stages of ventricular dysfunc-
aneurysm is defined as >150% dilation of the repair site in tion.47 However, abnormal myocardial deformation indices
relation to the aorta at the level of the diaphragm and occurs (strain, strain rate and torsion) are found even in patients with
with an incidence of 13%.43 Aneurysms are mostly seen after well-repaired CoA, preceding systolic dysfunction.3 44 Assess-
patch angioplasty and less often after BA or subclavian flap ment of LV function is, therefore, important in CoA follow-up
surgery.38 43 and should include myocardial velocity and deformation
parameters.3
Hypertension
Chronic hypertension remains present in 35%–68% of the Pregnancy
patients with CoA, even in the presence of an anatomically Women with CoA have an increased risk of cardiovascular
satisfactory repair.31 33 42 Furthermore, exercise-induced complications during pregnancy.48 Hypertensive complications
hypertension occurs in over one-third of the normotensive occur in 25% of pregnant patients with CoA.48 49 Both pre-ex-
patients.44 Prevalence of systemic hypertension is significantly isting- and pregnancy-induced hypertension occur more often
lower in patients treated in the neonatal period or infancy and in patients with CoA, associated with adverse foetal outcome
in patients who underwent CoA resection with end-to-end due to preterm delivery, low birthweight for gestational age
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and admission to the neonatal intensive care unit.49 Female 14 Mirza FG, Bauer ST, Williams IA, et al. Early fetal echocardiography: ready for prime
patients with CoA with pregnancy wish should preferably time? Am J Perinatol 2012;29:313–8.
15 Yeo L, Romero R. Fetal intelligent navigation echocardiography (FINE): a novel method
undergo assessment of their haemodynamic status, severity of for rapid, simple, and automatic examination of the fetal heart. Ultrasound Obstet
(re-)CoA and associated lesions before conception.50 Gynecol 2013;42:268–84.
16 Jowett V, Aparicio P, Santhakumaran S, et al. Sonographic predictors of surgery in fetal
coarctation of the aorta. Ultrasound Obstet Gynecol 2012;40:47–54.
CONCLUSION 17 Liberman RF, Getz KD, Lin AE, et al. Delayed diagnosis of critical congenital heart
Despite widespread availability of foetal cardiac screening in the defects: trends and associated factors. Pediatrics 2014;134:e373–81.
current era, CoA often remains undiagnosed prenatally which 18 Gómez-Montes E, Herraiz I, Mendoza A, et al. Prenatal prediction of surgical approach
for coarctation of the aorta repair. Fetal Diagn Ther 2014;35:27–35.
poses significant risks of mortality and morbidity to the affected 19 Kenny D, Polson JW, Martin RP, et al. Hypertension and coarctation of the aorta:
child. Prenatal screening for aortic arch abnormalities, adequate an inevitable consequence of developmental pathophysiology. Hypertens Res
clinical examination and (if necessary) cardiovascular imaging of 2011;34:543–7.
the neonate are, therefore, of great importance. Neonatal CoA 20 Alpert M. Systolic murmurs. In: Walker HK, Hall WD, Hurst JW, eds. Clinical methods:
the history, physical, and laboratory examinations. 3rd edn. Boston: Butterworths,
is preferably repaired surgically, whereas BA and stent place-
1990.
ment are the treatment of choice for older children and adults. 21 Gewillig M, Budts W, Boshoff D, et al. Percutaneous interventions of the aorta. Future
Despite adequate treatment, patients with CoA have a reduced Cardiol 2012;8:251–69.
life expectancy and increased risk of cardiovascular complica- 22 Soslow JH, Kavanaugh-McHugh A, Wang L, et al. A clinical prediction model to
tions later in life, related to hypertension, LV dysfunction and estimate the risk for coarctation of the aorta in the presence of a patent ductus
arteriosus. J Am Soc Echocardiogr 2013;26:1379–87.
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and cerebrovascular diseases. Timely intervention is crucial, 2011;84 Spec No 3:S338–54.
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Recoarctation should be proactively addressed. Cardiovas- diagnosis and management of coarctation of the aorta. J Thorac Cardiovasc Surg
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25 Shepherd B, Abbas A, McParland P, et al. MRI in adult patients with aortic coarctation:
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27 Turner DR, Gaines PA. Endovascular management of coarctation of the aorta. Semin
Contributors EJD collected data and wrote the manuscript. TL reviewed the
Intervent Radiol 2007;24:153–66.
manuscript and provided images. HBG served as scientific advisor and was a large
28 Frydrychowicz A, Markl M, Hirtler D, et al. Aortic hemodynamics in patients with and
contributor to the manuscript.
without repair of aortic coarctation: in vivo analysis by 4D flow-sensitive magnetic
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Dijkema EJ, et al. Heart 2017;103:1148–1155. doi:10.1136/heartjnl-2017-311173 1155

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Diagnosis, imaging and clinical management

of aortic coarctation
Elles J Dijkema, Tim Leiner and Heynric B Grotenhuis

Heart2017 103: 1148-1155 originally published online April 4, 2017

doi: 10.1136/heartjnl-2017-311173

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