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Transcatheter Approach
https://t.me/med1917
toCoarctation ofAorta
andIsolated Interrupted Aortic
Arch inAdults
AtaFirouzi, AnitaSadeghpour, andZahraHosseini
Abstract
Isolated coarctation of the aorta (COA) is a
localized aortic narrowing (usually postductal, near subclavian artery) that mainly
remains undiagnosed in adulthood and is
detected incidentally in a hypertensive patient
with Radial-Femoral pulse delay. Untreated
patients have poor prognoses. Previously, the
gold standard of treatment was the surgical
repair. However, during the last two decades,
balloon angioplasty with stenting has perceived their roles and long-term outcome trials conrmed the safety and effectiveness of
transcatheter (TC) coarctoplasty in adulthood.
Interrupted aortic arch (IAA) is aortic luminal
disruption between ascending and descending
aorta, a complex congenital heart disease
commonly associated with other congenital
anomalies. Although, the surgical approach
was the rst option for those with associated
other anomalies, in selected cases, percutane-
Supplementary Information The online version contains supplementary material available at https://doi.
org/10.1007/978- 3- 031- 50740- 3_14.
A. Firouzi · Z. Hosseini (*)
Cardiovascular Intervention Research Center, Rajaie
Cardiovascular Medical and Research Center, Tehran, Iran
A. Sadeghpour
MedStar Cardiovascular Corelabs, MedStar Health
Research Institute, Georgetown University,
Washington, DC, USA
e-mail: anita.sadeghpour@medstar.net
ous reconstruction of IAA before surgical
repair of other defects, facilitates the surgeon’s
point of view. In this section, we will discuss
the role of cardiac imaging in diagnosing
COA and IAA and their roles in characterizing
the appropriate cases for the TC approach, the
interventional tackle, and post-procedure
follow-up.
Keywords
Coarctation of aorta · Hypertension ·
Transcatheter coarctoplasty · Interrupted
aortic arch · Luminal disruption · Surgical
repair · Cardiac imaging
Abbreviations
BAV Bicuspid aortic valve
BES Balloon-expandable stent
CMRI Cardiac magnetic resonance imaging
COA Coarctation of aorta
CTA Computed tomography angiography
HTN Hypertension
IAA Interrupted aortic arch
PDA Patent ductus arteriosus
SES Self-expandable stent
TC Trans-catheter
TEVAR Thoracic endovascular aortic repair
TS Turner syndrome
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2024
A. M. Kelsey et al. (eds.), Cardiac Imaging in Structural Heart Disease Interventions,
https://doi.org/10.1007/978-3-031-50740-3_14
327
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A. Firouzi et al.
TTE Transthoracic echocardiography
VSD Ventricular septal defects
Test your learning and check your under-
standing of this book’s contents: use the
“Springer Nature Flashcards” app to
access questions using ▶ https://sn.pub/
ambACS.
To use the app, please follow the instruc-
tions in the chapter “Transcatheter
Aortic Valve Replacement.”
Learning Objectives
1. Emphasis on the timely diagnosis of COA.
2. Know the indications for coarctoplasty and
appropriate therapeutic approach
3. Know the role of imaging in diagnosis and
interventional therapeutic approach and
surveillance.
Case 1
A 29year-old-man was referred to the outpatient clinic for more evaluation of refractory systemic hypertension despite full
guideline directed medications. In physical
examination, Radial-Femoral pulse delay
was evident; upper extremities BP:
160/90mmHg, right lower popliteal artery
BP: 100/60 mmHg. There was an interscapular systolic murmur (Grade II) in his
heart auscultation. The Electrography
(ECG) demonstrated LVH pattern with a
strain pattern in pericardial leads.
Background andDenitions
Coarctation of aorta (COA) is a congenital juxta
ductal localized aortic narrowing (usually postductal in adults) with a ridge consisting of local-
ized medial thickening and infolding with
superimposed neo-intimal tissue. Most commonly, it is located at the junction of the ductus
arteriosus with the aortic arch, just distal to the
left subclavian artery. COA accounts for 5–8% of
children born with congenital heart disease,
which is often associated with other congenital
cardiac anomalies including: VSD, PDA,
Hypoplastic aortic arch, Shone complex, and
Bicuspid aortic valve (BAV) [1].
BAV is commonly associated with COA and
is present in more than half of COA patients
which can lead to AI, AS or aortic dilatation and
dissection. Intracranial aneurysms may also
occur (2–10%). Timely diagnosis of COA is
mandatory for a good prognosis since early treatment is associated with lower risk of long-term
morbidity and mortality. The natural history of
this condition is miserable, with death ensuring
on average in the fourth decade of life and threequarters of patients dying before their 50th birthday [2].
The most common causes of death are congestive heart failure, aortic dissection, rupture of the
aorta, infective endocarditis, premature coronary
artery disease, and intra-cranial hemorrhage [3].
In adults, the most common presentation is systemic hypertension, accounts for 0.2% of all
hypertension cases in adults.
The transverse aortic arch (TAA) is a segment
of the arch between the innominate artery and the
left subclavian artery (LSA).Isthmus is a portion
of aorta distal to LSA.If TAA diameter is <60% of
ascending aortic diameter, it is called hypoplastic.
Isthmus is hypoplastic if its diameter is <40% of
ascending aortic diameter. Normally, aortic isthmus is 80–90% of TAA diameter and almost equal
to that of aorta at the diaphragmatic level [4].
An Interrupted aortic arch (IAA), is an anomaly that can be considered the most severe form
of coarctation of the aorta. In an IAA, there is an
anatomical and luminal disruption between the
ascending and descending aorta. The incidence is
about 1.5% in patients with congenital cardiac
anomaly [5]. If untreated, 90% of the affected
infants may die in the rst year of life, with the
majority in the rst few days. In the few cases
Transcatheter Approach toCoarctation ofAorta andIsolated Interrupted Aortic Arch inAdults
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reported in adults, the presentation varies from
being asymptomatic to differential blood pressure recordings in the extremities, refractory systemic arterial hypertension, headache,
claudication, and congestive heart failure [6].
Survival into adulthood is dependent upon the
development of collateral circulation. Although
in neonates the most common type of the IAA is
type B (The disruption is located between the left
carotid artery and the left subclavian artery), in
adults, type A is the most frequent type (The disruption is located distal to the left subclavian
artery) [7].
Diagnostic Workup
Physical Examination: Radial-Femoral pulse
delay is evident unless signicant AI coexists.
The systolic blood pressure gradient ≥20mmHg
between the upper (right arm) and lower extremities suggests signicant CoA that requires therapeutic intervention. The differential systolic
blood pressure of at least 10mmHg can be indicative for CoA. CoA patients might have interscapular early to mid-systolic or continuous
murmurs (due to collateral vessels) in
auscultation.
ECG: various degrees of LVH are seen.
CXR; So called “gure-3” conguration and
rib notching (in about 50% of cases) are diagnostic [8, 9].
Transthoracic Echocardiogram (TTE):
– Suprasternal window provides the best view
for visualizing the narrowing of the aorta and
accelerated ow proximal to the narrowing.
– Continuous wave Doppler study of the coarc-
tation site has a characteristic pattern called
“Sawtooth” appearance which is turbulent
antegrade systolic ow extending to the diastole. However, it might be difcult to nd the
narrowing and typical Doppler ndings in
adult patients.
– In signicant CoA, pulse wave Doppler study
of the abdominal Aorta shows delayed and
low velocity systolic ow that extends to the
diastole with absent early diastolic ow
reversal.
– The width and length of the stenotic segment,
gradients and collateral vessels should be
searched by TTE.Even in the absence of a sig-
nicant gradient through the stenotic segment,
a slow systolic upstroke and an antegrade dia-
stolic ow on spectral Doppler suggest signi-
cant COA or IAA with collateral vessels.
– Additionally, TTE should provide data about
the biventricular function, aortic valve (eg,
bicuspid aortic valves, aortic stenosis, and
aortic insufciency), left ventricular outow
tract obstructions associated with ventricular
septal defects, patent ductus arteriosus.
In CoA and IAA, cardiac magnetic resonance
(CMR) imaging and computed tomography (CT)
angiography are recommended as class I indications for initial and follow-up evaluation. The
exact location, width and length, site of the stenosis, collateral vessels, and the size of the aorta at
the level of the sinus of Valsalva, ascending,
proximal arch, transverse arch, descending aorta
at the level of the diaphragm, and any other
anomalies should be evaluated. Also they assist
in selecting the appropriate treatment approach
(surgical or transcatheter approach [TC]) in the
preprocedural planning and appropriate device
selection before invasive angiography and nding the optimal uoroscopic angles especially in
those with IAAs.
The standard management of native coarctation in infants and young children is surgical
repair, with preferred therapy in older children,
adolescents, and adults being percutaneous intervention [10].
Signicant aortic coarctation (native or
recurrent) based on the AHA/ACC 2018
guideline is dened as [10]:
• Resting upper extremity to lower extremity
peak-to-peak gradient >20 mmHg or mean
systolic Doppler pressure gradient >20mmHg
• Upper extremity to lower extremity pressure
gradient >10mmHg or mean systolic Doppler
gradient >10 mmHg in the presence of
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A. Firouzi et al.
decreased LV systolic function or aortic
regurgitation
• Upper extremity to lower extremity gradient
>10mmHg or mean Doppler pressure gradient
>10mmHg in the presence of collateral ow
Therapeutic intervention recommendations
based on the ESC 2020 Guideline [9]:
Class I: In a hypertensive patient with peak-to-
peak gradient ≥20 mmHg, stenting is preferred over surgery when technically feasible.
Class IIa: In a hypertensive patient with a peak-
to- peak gradient is <20mmHg, but aortic narrowing ≥50% relative to the diameter of the
aorta at the diaphragm, catheter treatment
(stenting) should be considered when technically feasible. In a normotensive patient with
peak to peak gradient ≥20 mmHg, stenting
should be considered when technically
feasible.
Class IIb: In a normotensive patient with peak-
to- peak gradient <20 mmHg, but aortic narrowing ≥50% compared to the diameter of the
aorta at the diaphragm, stenting may be considered when technically feasible
Diagnosis andPre-procedural
Evaluation inCase 1
As the patient was highly suspicious of the
COA, 2-D TTE was done and showed: Normal
size and function left ventricle with moderate
concentric LVH. There was BAV without AS
and mild to moderate eccentric AI.Aortic root
and ascending aorta evaluation revealed annulus-aortic ectasia with ST junction effacement
and aneurysmal ascending aorta: 5.3 cm
(Fig.1a). There was signicant narrowing with
systolic turbulency in proximal part of the thoracic descending aorta with systolic PG:
64 mmHg and diastolic antegrade ow (diastolic tail—Fig. 1b), limited abdominal aorta
pulsatility with early systolic upstroke consistent with COA (Fig. 1c). There was no other
associated cardiac abnormality (Fig.2).
CTA conrmed the echocardiography diagnosis of bicuspid aortic valve, aneurysmal dilatation
of aortic root and ascending aorta with juxtaductal discrete COA and associated multiple
large collaterals.
a
Fig. 1 (a–c) In TTE, Annulus-aortic ectasia with ST
junction effacement is denoted (a). A signicant narrowing with systolic turbulency in the proximal part of the
thoracic descending aorta with the diastolic antegrade
ow (diastolic tail) resulting typical Sawtooth pattern (b).
bc
Abdominal aorta pulse wave Doppler study shows slow
and low-velocity systolic ow extending to the diastole
with absent early diastolic ow reverse consistent with
severe COA (c)
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331
a
b
Fig. 2 (a, b) Transesophageal echocardiogram in long axis view of thoracic Aorta showing CoA narrowing by 2D and
color Doppler study
Heart Team Approach andDecision
with no AS and mild AR and aneurysmal dilatation of the aortic root. After that, aortic arch
In neonates and adolescents with coarctation,
surgical approach has been the gold standard
treatment.
However, in recent decades, coarctoplasty and
primary stenting techniques have been suggested
for young adults and adults as the best therapeutic approach considering the technical improvement in this eld. There are several surgical
methods; while end-to-end anastomosis and subclavian ap repair have a high risk of recoarctation (10%), patch aortoplasty has a high
risk of aneurysm formation (10%).
Our patient was a candidate for the transcatheter approach regarding refractory hypertension
and signicant focal CoA to facilitate the surgical
aortoplasty as the next step.
injection was done to characterize the residual
segment, length, the distance to the left subclavian artery and the size of the aorta before and
after the coarctatrion (Fig. 3a and b). After
advancing a long delivery sheath (Cook-10F)
toward the aortic arch, the dilator was removed
and the sheath was de-aired. According to the
size of the aortic arch (22mm) and distal aortic
size (26mm), a self-expandable stent (Sinus-XL
22*60) was chosen. The delivery stent was passed
through the sheath to reach the tip of the sheath.
In LAO view by aortography through the sheath,
the correct position of the stent was conrmed.
For optimal stent positioning, we covered the
proximal stent with the delivery sheath and
slowly expanded the distal part of the stent to its
full size, then by pulling the sheath off of the
stent catheter, the remainder of the stent was
Procedural Technique
deployed across the coarctation segment.
Following stent deployment, postdilatation was
Under conscious sedation, after right CFA sheath
(6F) insertion, 5000 units IV Heparin was
injected. A Multipurpose catheter wiring of the
narrowed segment was done with 0.035- inch
hydrophilic wire (260 cm), then the wire was
exchanged with a supper-stiff long wire. Aortic
root injection in LAO projection showed: BAV
performed by ATLAS GOLD 18*40 balloon
(Fig.3c). A Pigtail catheter was passed to obtain
simultaneous pressure measurements across the
stent and to rule out any complications after mul-
tiple angiograms in LAO and RAO projections.
The procedure was terminated with good nal
result without any residual gradient (Fig.3d).
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Fig. 3 (a–d) Aortic arch injection in deep LAO projec-
tion demonstrated a discrete juxtaductal coarctation
(COA) with multiple well developed collateral vessels (a).
Sizing of the proximal and distal to the COA was done
and a self- expandable stent (Sinus-XL 22*60) was
selected based on the size of the aorta at the level of the
transverse arch (b). The delivery stent was passed through
the sheath to reach the tip of the sheath. In LAO view and
angiography through the sheath, the correct position of the
stent was conrmed. For optimal stent positioning, we
covered the proximal stent with the delivery sheath and
slowly expanded the distal stent to its full size, then by
pulling the sheath off of the stent catheter, the remainder
of the stent was deployed across the coarctation segment.
As the waist of the stent was not dilated appropriately,
postdilatation was done by ATLAS GOLD balloon
(18*40) (c). Final aortography in multiple projections
conrmed well expanded stent without any complications
and the nal residual gradient was zero (d)
ab
Transcatheter Approach toCoarctation ofAorta andIsolated Interrupted Aortic Arch inAdults
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Fig. 4 (a and b) Follow-up aortic CTA 1 month later demonstrated patent stent at proximal and distal portions of
descending aorta, no evidence of recoarctation, and no sign of complication at the site of stenting (a and b)
333
Post-procedural Follow Up
Case 2
In our case, aortic CTA, 1 month later, denoted:
Patent stent at the proximal portion of descending
aorta, no evidence of recoarctation, no sign of
complication at the site of stenting (Fig.4a and b).
All CoA patients require regular annual follow up. 2-D TTE is the rst imaging modality to
estimate the immediate and late residual gradient
and also to evaluate the degree of the AI and aortic size in patients with concomitant BAV.
However, CTA or preferably MRA are suggested imaging modalities to assess the postinterventional anatomy (Fig. 5) and possible
complications every 3–5years, although it might
be different based on the aortic pathology [9, 10].
A 25-year-old lady (G1P0), was referred
to the outpatient clinic with a history of
abortion 2 months ago (at 22 weeks of
gestation). She was a known case of
uncontrolled hypertension 5years ago and
was under guideline-directed medical
therapy. In physical examination, the general appearance was normal. Bilateral
upper extremities BP was 150/90mmHg
and right lower popliteal artery BP was
70/30 mmHg. Undetectable femoral
pulses bilaterally.
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Fig. 5 CMR of a patient with severe CoA and ow acceleration at the site of stenosis (Left upper image, Movie 1).
Associated bicuspid aortic valve in steady-state free precession CMR (right upper image, Movies 2 and 3), signicant collaterals with reversal of intercostal ow, in 3D
contrast-enhanced magnetic resonance angiography
(MRA), note that collateral ow can be assessed via ow
differential between proximal and distal aorta (Left lower
panel) Status post aortic stent placement with reduced
aortic gradient from 46 to 6mmHg and reduced collateral
(Right lower, Movie 4)
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Diagnosis andPre-procedural
Evaluation
2-D TTE illustrated: Tricuspid AV, with normal
size ascending aorta. In the supra sternal view,
the descending thoracic aorta after left subclavian artery was not visible (Fig. 6 left panel)
There was signicant turbulent ow at the site of
the interruption with multiple collateral vessels
and no signicant gradient (Fig.6 right panel).
Abdominal aorta Doppler study showed continu-
ous low systolic and antegrade diastolic ow in
favor of sever COA or interrupted aorta (IAA).
Interestingly, CT angiography of the aorta
revealed interrupted aortic arch just before left
subclavian artery origin with no residual lumen,
the length of the interruption: 6 mm, tapered
proximal and distal ends—Fig. 7a–c). Numerous
well developed collateral vessels were visible
(Fig.7d). The best working view to characterize
the alignment of the end parts: RAO 10 and caudal 22.
Fig. 6 In TTE, descending aorta was not evaluable after left subclavian artery with signicant turbulent ow at the site
of the interruption (left panel) and nonsignicant gradient at the site of the obstruction (right panel)
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A. Firouzi et al.
a
b
cd
Fig. 7 (a–d) In axial, coronal and sagittal views, short
interrupted aorta just before the origin of left subclavian
artery is demonstrated (a–c). Numerous collateral vessels
Procedural Technique
Under conscious sedation, after ultra-sonography
guided right CFA puncture and sheath (6F) insertion, and right radial artery access, 5000units IV
Heparin was injected (the base-line gradient was
70mmHg). Right CFA angiography showed an
acceptable diameter of the SFA, CFA, EIA, and
CIA (Fig.8a). So, 2 Perclose Proglide™ [Abbott
Medical, Santa Clara, CA, USA] was inserted in
the right CFA.Simultaneous antegrade and retrograde aortography at the level of the aortic isth-
and the size of the descending aorta at the level of the
diaphragm and lower extremities are shown (d)
mus and descending aorta with Pigtail catheter
and Multipurpose catheter at deep LAO and AP
projections depicted interrupted aorta just at the
level of left subclavian artery (Fig.8b). The inter-
rupted segment length was discrete and both
proximal and distal ends were tapered and their
alignment in the suggested working view by CTA
was not complex. Under meticulous hemodynamic monitoring, antegrade wiring was performed by CTO guidewire (Gaia 2nd) and after
conrming the position of the wire to be in the
true lumen by aortography in several projections
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