Percutaneous treatment of native aortic coarctation in adults
P. Luijendijk & S. M. Boekholdt & N. A. Blom &
M. Groenink & A. P. Backx & B. J. Bouma &
B. J. M. Mulder & R. J. De Winter
Published online: 27 September 2011
# The Author(s) 2011. This article is published with open access at Springerlink.com
Abstract Aortic coarctation is a common congenital
cardiac defect, which can be diagnosed over a wide range
of ages and with varying degrees of severity. We present
two cases of patients diagnosed with aortic coarctation in
adulthood. Both patients were treated by an endovascular
approach. These cases demonstrate the variety of indica-
tions in which percutaneous treatment is an excellent
alternative for surgical treatment in adult native coarctation
Keywords Native aortic coarctation in adults.
Aortic coarctation is a congenital cardiovascular malforma-
tion comprising a circumscript narrowing of the aortic
lumen usually located distally of the left subclavian artery,
at the site where the ductus arteriosus enters the aorta.
Aortic coarctation accounts for 5–8% of all congenital heart
defects and may be associated with other left-sided cardiac
abnormalities, such as bicuspid aortic valve, hypoplastic
aortic arch, and ventricular septal defect. When diagnosed
in neonates, surgical reconstruction is the treatment of
choice. In infants, surgery is preferred as well, but balloon
angioplasty has also evolved rapidly over the past decade.
Both treatment options carry the risk of restenosis and late
aneurysm formation [1, 2].For recoarctation after initial
surgical treatment, good long-term results have been
reported [3, 4].
A small proportion of aortic coarctation is not diagnosed
until adolescence or adult age. The presenting symptoms
usually include hypertension, congestive heart failure, or
intermittent claudication. There is an indication for treat-
ment if the gradient is >20 mmHg in combination with
hypertension, either at rest or during exercise . When
intervention is warranted, the risk of surgery is considerable
because of the extensive collateral network surrounding the
coarctation which results in a high risk of bleeding
complications. As a consequence, balloon angioplasty with
or without stenting has evolved as a treatment modality for
adolescents with native aortic coarctation [6, 7].
Case A. Native aortic coarctation
The first case involves a male patient referred to our
hospital, who had been diagnosed with severe aortic
coarctation at the age of 24 in the Middle East. At the time
of diagnosis, the gradient measured by catheterisation was
90 mmHg. The coarctation was left untreated despite the
presence of hypertension. He presented to our hospital at
the age of 33 with fatigue, shortness of breath on exercise,
complaints of headache, and intermittent chest pain both at
Electronic supplementary material The online version of this article
(doi:10.1007/s12471-011-0198-x) contains supplementary material,
which is available to authorized users.
P. Luijendijk:S. M. Boekholdt:M. Groenink:B. J. Bouma:
B. J. M. Mulder (*):R. J. De Winter
Department of Cardiology, Academic Medical Center,
PO Box 22660, 1100 DD Amsterdam, the Netherlands
N. A. Blom:A. P. Backx
Department of Paediatric Cardiology, Academic Medical Center,
Amsterdam, the Netherlands
N. A. Blom
Department of Paediatric Cardiology,
Leiden University Medical Center,
Leiden, the Netherlands
Neth Heart J (2011) 19:436–439
rest and during exercise. His blood pressure was 160/
100 mmHg. On auscultation, normal heart sounds were
heard, as well as an ejection murmur grade II/ VI at the
right second intercostal space and a late-systolic murmur
grade III/VI at the apex. Palpation of the femoral arteries
revealed slightly decreased pulsations. The ECG showed a
left anterior hemiblock. Echocardiography showed mild
concentric left ventricular hypertrophy without dilatation. A
tricuspid aortic valve was present with mild aortic regurgi-
tation. The aortic root was dilated with a diameter of
41 mm. The diameter of the ascending aorta was 32 mm.
Cardiac magnetic resonance imaging (MRI) confirmed a
complete interruption of the descending aorta directly after
the dilated left subclavian artery. Distal from the occlusion,
the aorta was supported by many collaterals originating
from the left mammary artery and the intercostal arteries
(Fig. 1a and b). It was decided to treat this patient with a
percutaneous balloon-expandable 28 mm covered stent.
Retrograde passage proved impossible, but using the radial
approach balloon passage was successful. After stent
implantation with deployment to 18 mm, angiography
revealed a well-expanded stent and there was no residual
pressure gradient (Fig. 2a and b) (Tape a and b). Two
months later, the patient was without complaints and
follow-up angiography showed good stent position, no
aortic aneurysms and there was no pressure gradient.
Case B. Native aortic coarctation
The second case concerns a 43-year-old man who had
recently been diagnosed with severe aortic coarctation and
a hypoplastic aortic arch, accompanied by a bicuspid aortic
valve and moderate aortic valve regurgitation. In addition,
he had a dilated left ventricle with impaired systolic
function. He admitted severe alcohol and cannabis abuse
over the past several years.
He presented with increasing fatigue but no exertional
dyspnoea or orthopnoea. Physical examination revealed an
important blood pressure difference between the right and
left arm (125/77 versus 99/72 mmHg, respectively). On
auscultation normal heart sounds with a grade II/IV
crescendo/decrescendo murmur and a short diastolic murmur
diaphragm, and weak peripheral pulsations. The electrocar-
diogram showed mild intra-ventricular conduction delay
(QRS 120 ms), and left ventricular hypertrophy with
secondary repolarisation abnormalities. Echocardiography
showed a dilated, hypertrophied left ventricle with a moder-
ately impaired function (Fig. 3a). Diffuse left ventricular wall
motion disturbances with hypokinesis and akinesis of the
interventricular septum were seen. The aortic valve was
bicuspid with a maximal gradient of 26 mmHg and mild
aortic regurgitation. The aortic root and ascending aortic
diameters were 39 mm and 33 mm, respectively. Turbulent
flow was seen in the descending aorta with a maximum
velocity of 3.5 m/s and a typical saw-tooth shape (Fig. 3b).
This patient was treated with balloon dilatation and
implantation of a 22 mm covered stent. Before the
procedure, mean systolic blood pressures were 70 mmHg
proximal to the coarctation and 45 mmHg distal of the
coarctation. Stent implantation was successful and resulted in
Coarctation of the aorta can be diagnosed across a wide age
spectrum and with a range of symptoms. An untreated
Fig. 1 Magnetic resonance
imaging in native aortic coarc-
tation. a. MRI reconstruction
image demonstrating the many
collateral arteries b. Late
gadolinium MRI image demon-
strating the severe coarctation
accompanied by the collateral
Neth Heart J (2011) 19:436–439437
coarctation of the aorta presenting later in life has a
negative impact on long-term survival, as compared with
patients diagnosed and treated in early life . Late
complications such as aortic aneurysm formation, infective
endocarditis, hypertension, premature (coronary) athero-
sclerosis and cerebrovascular accidents all account for the
increased morbidity and mortality [9, 10]. The two patients
described above were both diagnosed in adulthood.
Traditionally, surgical repair has been an effective treatment
option for adult patients with native coarctation, and it has
been shown to improve the efficacy of postoperative
antihypertensive treatment. Adult patients with native
coarctation are usually at lower operative risk than re-
coarctation patients because there are no concerns related to
pleural or peri-aortic scar tissue associated with previous
surgical repair. However, these patients do have many
associated cardiovascular comorbidities including left ven-
tricular hypertrophy and arrhythmias . The first case
presented with a severe aortic coarctation, with many
collaterals due to the severe obstruction. In the second case
the severe aortic coarctation was accompanied by a
hypoplastic arch and an impaired left ventricular function
possibly due to long-lasting alcohol abuse. In both cases,
the risk of cardiac surgery was estimated to be too high. In
case 1, the extensive collateral network surrounding the
coarctation caused a substantial risk of peri-operative
bleeding complications. In case 2 surgical risk was
increased because of the impaired left ventricular function.
In both cases endovascular repair with balloon angioplasty
and stent placement was performed. Endovascular repair
with or without stenting has been demonstrated to be an
acceptable alternative to surgical repair with similar
outcome in native coarctation. The use of covered stents
has been promoted to avoid vascular complications . The
incidence of recoarctation after stent placement is lower
than after balloon angioplasty alone, due to a lower degree
of elastic recoil and avoidance of vessel overdilation and, as
a consequence, reduced aortic injury. Concerns that arise in
coarctation patients after endovascular repair are strut
fractures, metal fatigue or aortic deterioration or aortic
disruption at the coarctation site . However, studies
documenting the long-term outcomes of stent implantation
confirmed the low rate of procedure-related adverse events
and a long-term procedural success in both native and
recurrent aortic coarctation or aneurysmal disease [12, 13].
Long-term outcome was comparable with other surgical
and interventional modalities. This case report demonstrates
the variety of indications in which percutaneous treatment
Fig. 3 Transthoracic echocardi-
ography in case B a. Apical
four-chamber view demonstrat-
ing the dilated left ventricle b.
The typical sawtooth pattern
with continuous flow on contin-
uous wave Doppler interroga-
tion in aortic coarctation
Fig. 2 Percutaneus
stent-implantation for aortic
coarctation a. Angiographic
image before stent expansion in
case A b. Angiographic image
after stent expansion in
438 Neth Heart J (2011) 19:436–439
is preferable to surgical treatment in adult patients with Download full-text
native aortic coarctation. More research on the long-term
assessment of adult native coarctation patients after
percutaneous intervention will be important to determine
the impact on survival in these patients.
Creative Commons Attribution Noncommercial License which per-
mits any noncommercial use, distribution, and reproduction in any
medium, provided the original author(s) and source are credited.
This article is distributed under the terms of the
1. McElhinney DB, Yang SG, Hogarty AN, et al. Recurrent arch
obstruction after repair of isolated coarctation of the aorta in
neonates and young infants: is low weight a risk factor? J Thorac
Cardiovasc Surg. 2001;122:883–90.
2. Rao PS, Galal O, Smith PA, Wilson AD. Five- to nine-year
follow-up results of balloon angioplasty of native aortic
coarctation in infants and children. J Am Coll Cardiol.
3. Ince H, Petzsch M, Rehders T, et al. Percutaneous endovascular
repair of aneurysm after previous coarctation surgery. Circulation.
4. Reich O, Tax P, Bartakova H, et al. Long-term (up to 20 years)
results of percutaneous balloon angioplasty of recurrent aortic
coarctation without use of stents. Eur Heart J. 2008;29:2042–8.
5. Baumgartner H, Bonhoeffer P, De Groot NM, et al. ESC
Guidelines for the management of grown-up congenital heart
disease (new version 2010). Eur Heart J. 2010;31:2915–57.
6. Paddon AJ, Nicholson AA, Ettles DF, Travis SJ, Dyet JF. Long-
term follow-Up of percutaneous balloon angioplasty in adult
aortic coarctation. Cardiovasc Intervent Radiol. 2000;23:364–7.
7. Pedra CA, Fontes VF, Esteves CA, et al. Stenting vs. balloon
angioplasty for discrete unoperated coarctation of the aorta in
adolescents and adults. Catheter Cardiovasc Interv. 2005;64:495–506.
8. Wheatley III GH, Koullias GJ, Rodriguez-Lopez JA, Ramaiah
VG, Diethrich EB. Is endovascular repair the new gold standard
for primary adult coarctation? Eur J Cardiothorac Surg.
9. Vriend JW, Mulder BJ. Late complications in patients after repair
of aortic coarctation: implications for management. Int J Cardiol.
10. Vriend JW, de Groot E, de Waal TT, et al. Increased carotid and
femoral intima-media thickness in patients after repair of aortic
coarctation: influence of early repair. Am Heart J. 2006;151:242–7.
11. Tanous D, Collins N, Dehghani P, Benson LN, Horlick EM.
Covered stents in the management of coarctation of the aorta in
the adult: initial results and 1-year angiographic and hemodynamic
follow-up. Int J Cardiol. 2010;140:287–95.
12. Holzer R, Qureshi S, Ghasemi A, et al. Stenting of aortic
coarctation: acute, intermediate, and long-term results of a
prospective multi-institutional registry–Congenital Cardiovascular
Interventional Study Consortium (CCISC). Catheter Cardiovasc
13. Shennib H, Rodriguez-Lopez J, Ramaiah V, et al. Endovascular
management of adult coarctation and its complications: interme-
diate results in a cohort of 22 patients. Eur J Cardiothorac Surg.
Neth Heart J (2011) 19:436–439439