S e m i n a r s i n Va s c u l a r S u r g e ry 3 5 ( 2 0 2 2 ) 3 2 0 – 3 3 3

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journal homepage: www.elsevier.com/locate/semvascsurg

Review article

Endovascular treatment of aortic aneurysms and

dissections in patients with genetically triggered
aortic diseases
Konstantinos Spanos∗, Yskertvon Kodolitsch, N. Christian Detter,
Giuseppe Panuccio, Fiona Rohlffs, Ahmed Eleshra, Tilo Kölbel
German Aortic Center Hamburg, University Heart Center, University Hospital Hamburg, Eppendorf Martinistrasse
52, 20246 Hamburg, Germany

a r t i c l e i n f o a b s t r a c t

Connective tissue disease (CTD) syndromes involve the ascending, aortic arch, and thora-
coabdominal aorta and are associated with higher risk of aortic aneurysm or dissection.
Currently, vascular societies generally recommend open repair as the first option for aortic
disease in patients with CTD. However, the implementation of endovascular techniques for
patients with CTD with aortic pathologies seems to have increased in recent years, mainly
in patients of high surgical risk or in urgent situations. Endovascular treatment of aortic
arch pathologies in patients with CTD have been feasible in experienced centers; how-
ever, the evidence is scarce. Thoracic endovascular aneurysm repair in patients with CTD is
more evident; in 15 studies, 304 patients with CTD were treated with thoracic endovascular
aneurysm repair with high technical success rates (88% to 100%) and a low early mortal-
ity rate (1.6%). During the median follow-up, 33 patients died and 64 patients underwent a
re-intervention. In 6 studies, 26 patients with CTD were treated with fenestrated/branched
endovascular aneurysm repair for thoracoabdominal aortic aneurysm, with a technical suc-
cess rate of 100%, without early mortality and morbidity. The endovascular approach to
thoracoabdominal aortic aneurysm, especially in post-dissection patients, mandates ad-
junctive techniques to achieve false lumen thrombosis with various approaches; in our ex-
perience, the Candy-Plug technique has been proven to be technically feasible with good
outcomes. Endovascular treatment of aortic pathologies in patients with CTD seems to be
feasible and safe in high-risk and urgent patients. Re-intervention remains an issue. The
constant development of endovascular techniques and devices may provide improved mor-
tality and morbidity outcomes.
© 2022 Elsevier Inc. All rights reserved.

encode for structural proteins (eg, FBN1, COL1A1, COL3A1,

1. Introduction COL5A1, and BGN), modifying enzymes (eg, ADAMTS2 and
PLOD1), or components of the transforming growth factor–
During the past few decades, many genes have been identi-
β signaling pathways (eg, SMAD2/3, TGFBR1/2, and TGFB2/3)
fied as a cause of connective tissue disease (CTD). These genes
[1,2]. The most common CTD syndromes involving the ascend-
ing aorta, aortic arch, and thoracoabdominal aorta are Marfan
∗
Corresponding author.
E-mail address: spanos.kon@gmail.com (K. Spanos).
https://doi.org/10.1053/j.semvascsurg.2022.06.001
0895-7967/$ – see front matter © 2022 Elsevier Inc. All rights reserved.
 S e m i n a r s i n Va s c u l a r S u r g e ry 3 5 ( 2 0 2 2 ) 3 2 0 – 3 3 3
Fig. 1 – Typical aortic manifestations in patients with Loeys-Dietz syndrome. (A) Aortic root aneurysm. (B) Type A aortic
dissection.
syndrome (MFS), Loeys-Dietz syndrome (LDS), Ehlers-Danlos
syndrome (EDS), and Turner syndrome [3–6].
These syndromes also affect the connective tissue of vari-
ous organ systems, including heart, blood vessels, skin, joints,
bone, eyes, and lungs. CTD syndromes show some degree
of phenotypical overlap of clinical features [1]. MFS is typ-
ically characterized by cardiovascular, ocular, and skeletal
manifestations. LDS can be distinguished from MFS by the
unique presence of hypertelorism, bifid uvula or cleft palate,
and widespread aortic and arterial aneurysm and tortuosity,
which both tend to be more severe. EDS refers to a group
of clinically and genetically heterogeneous connective tissue
disorders, and all subtypes are characterized by variable ab-
normalities of skin, ligaments and joints, blood vessels, and
internal organs [7,8]. The latest EDS nosology distinguishes 13
subtypes. In Turner syndrome, approximately 75% of individ-
uals have an abnormal cardiovascular anatomy [9].
Patients with CTD are at risk of aneurysmatic aortic di-
latation of the entire aorta and also have a higher risk for
aortic dissection (AoD) (Fig. 1). AoD can occur despite pro-
phylactic surgical repair of aortic aneurysms; 52% of patients
with AoD have previously undergone aortic graft implantation
that typically involved the aortic root and/or ascending aorta
[10]. Consistent with this, AoD originates most commonly in
the distal aortic arch or descending thoracic aorta. Regard-
ing mechanism, it is likely that patients with thoracic aortic
aneurysm (TAA) undergoing aortic repair represent a high-
risk group in terms of intrinsic vascular properties that pre-
dispose them to aortic dilatation and AoD. It is also possible
that aortic hemodynamics contribute to AoD in nonsurgical
or postoperative patients. Results from the multicenter Gen-
321
TAC (National Registry of Genetically Triggered Thoracic Aor-
tic Aneurysms and Cardiovascular Conditions) registry high-
light that patients with genetically associated TAA remain at
substantial risk for AoD, despite state-of-the-art care and con-
ventional imaging at experienced centers [10]. Although in-
creased aortic size is a risk factor for subsequent AoD, events
typically occur below established thresholds for prophylactic
TAA repair [10].
The treatment for patients with CTD with thoracic aortic
lesions is complex and is associated with high rates of mor-
bidity, mortality, and surgical intervention. More than one-half
of patients will undergo more than one cardiovascular proce-
dure. Although there is some variability in the age at which
their first operation was performed, most patients are oper-
ated on as young adults [11].
Generally, the proportion of patients with CTD receiving
an aortic procedure is 2.5-fold higher than for patients with-
out CTD. Although patients with CTD are younger and have
fewer comorbidities, there is an overall higher rate of proce-
dural complications and increased length of stay and cost in
patients with CTD syndrome undergoing aortic surgery, due to
the unique pathogenesis of their underlying connective tissue
disorder [12].
2. Indications for treatment
Currently, vascular societies generally recommend open re-
pair for aortic disease in patients with CTD. Regarding the ab-
dominal aorta, the European Society for Vascular Surgery rec-
ommends that, in young patients with suspected connective
322 S e m i n a r s i n Va s c u l a r S u r g e ry 3 5 ( 2 0 2 2 ) 3 2 0 – 3 3 3

Fig. 2 – A 47-year-old patient with Marfan syndrome after previous ascending aortic repair treated with thoracic
endovascular aneurysm repair for type B aortic dissection in landing zone 2. (A) Multiplanar reconstruction of immediate
postoperative computed tomography angiography (CTA). (B) CTA at 1-year follow-up showing full aortic remodeling without
signs of proximal landing zone erosion.

tissue disorder and abdominal aortic aneurysm, open surgi-

cal repair is recommended as the first option (recommenda-
3. Summary of evidence on endovascular
tion class: I, level of evidence: C) [13]. Referral to a multidis-
treatment
ciplinary aortic team at a highly specialized center is recom-
During the past decade, an increasing number of studies on
mended to manage patients with CTD with an aortic disorder
endovascular repair in patients with CTD have been pub-
(recommendation class: I, level of evidence: C). In the thoracic
lished, varying from case reports and case series to retrospec-
aorta, the European Society for Vascular Surgery highlights is
tive studies and registries. Despite clear recommendations
not recommended in patients with CTD if the proximal land-
against TEVAR, the implementation of endovascular tech-
ing zone is in native aortic tissue of zone 0 to 2 (recommenda-
niques for patients with CTD and aortic pathologies seemed to
tion class: III, level of evidence: C) [14]. It was emphasized that,
increase not just in patients of high surgical risk or in urgent
in the near future, studies should resolve the controversy over
situations (Fig. 2). However, patients with CTD should be re-
the use of stent-grafts in CTD.
ferred to a multidisciplinary team in a highly specialized aor-
The American Heart Association guidelines also recom-
tic center for evaluation, genetic counseling, and further man-
mend considering surgical repair of the aorta in all adult pa-
agement.
tients with CTD (level of evidence: C) [15]. The European Soci-
ety of Cardiology recommends that when intervention is in-
dicated in cases of MFS or other elastopathies, surgery should
be indicated, rather than thoracic endovascular repair (TEVAR) 4. Summary of evidence on ascending and
(recommendation class: IIa, level of evidence: C). The Euro- aortic arch disease treatment
pean Society of Cardiology highlights that there is no evidence
supporting any use of TEVAR in patients with CTD, except in Endovascular treatment evolution has widened the spectrum
emergency situations to obtain initial stabilization as a bridge of patients that can be treated for aortic disease using hybrid
to definitive surgical therapy [16]. techniques or completely endovascular methods with encour-
However, those recommendations were published from aging outcomes [18]. The previous sole option for the manage-
2010 to 2019. Recently, a 2021 Expert Consensus highlighted ment of proximal segments of the aorta (ascending and aor-
that, at the time of that writing, TEVAR was still not recom- tic arch) was open surgery, which has also improved periop-
mended for patients with CTD except as a bail-out proce- erative patient outcomes and techniques [19,20]. In patients
dure or for patients with at least a proximal, alloplastic land- with CTD and proximal aortic disease, such as type A aortic
ing zone, as well as in patients with anastomotic pseudoa- dissection (TAAD), the frozen elephant trunk (FET) technique
neurysm in the thoracic aorta [17]. has had favorable outcomes promoting positive remodeling
 S e m i n a r s i n Va s c u l a r S u r g e ry 3 5 ( 2 0 2 2 ) 3 2 0 – 3 3 3 323

Fig. 3 – A 46-year-old patient with Marfan syndrome after previous supracoronary ascending aortic repair for acute type A
aortic dissection. (A) A residual false lumen thoracoabdominal aneurysm with a maximum aortic diameter of 52 mm at the
proximal descending aorta has developed 6 months after initial repair. (B) A modified frozen elephant trunk has been
implanted in landing zone 2 with retrograde false lumen perfusion necessitating further distal extension to the celiac artery
and false lumen occlusion. Three-dimensional volume rendering of pre- and postoperative computed tomography
angiography.

[21]. The FET technique, and also total arch replacement pro-
cedure, can be performed for TAAD in patients with CTD with
low operative mortality (Fig. 3), favorable long-term survival,
and freedom from reoperation, but may require further dis-
tal extension with TEVAR and concomitant false lumen oc-
clusion or thoracoabdominal repair (Fig. 4). Even a concomi-
tant Bentall procedure might provide good long-term survival,
but with an increased risk for late reoperation compared with
other techniques [22]. Studies have found that the total arch
replacement and FET techniques are even feasible and effica-
cious for chronic TAAD after previous Bentall procedure in pa-
tients with CTD. Early and late survival did not differ between
acute and chronic dissections [23].
The use of endovascular techniques in patients with CTD
is not broadly adopted because of anticipated adverse events
and poor durability due to progressive aortic dilatation, es-
pecially at the level of ascending and aortic arch. A more ro-
bust approach might be the hybrid approach, as described by
Preventza et al. [24], who presented 19 patients that under-
went a hybrid repair with total arch replacement and con-
comitant endovascular stenting of the aortic arch or the de-
scending thoracic aorta. The technical success rate was 100%
and the authors suggested that endovascular techniques may
be helpful in treating selected young patients with CTD with
thoracic aortic disease. However, recently custom-made aor-
tic arch branched devices have been designed with one, two,
or three inner branches, as needed, to accommodate preserva-
tion of flow to the supra-aortic arteries. In their study cohort,
Spear et al. [25] included two patients with CTD post AoD, who
were treated successfully with fenestrated/branched (F/B)-
TEVAR. This study confirmed the feasibility and safety of F/B-
TEVAR repair of post-dissection aortic arch aneurysms and
TAAAs at any level of the aorta. They also highlighted that
these procedures are considerably more demanding techni-
cally than “standard” F/BEVAR and may be associated with
a higher rate of secondary procedures. Patient selection, dili-
gent preoperative planning (especially of bridging stents), pre-
cise operative execution, attentive early postoperative care,
and comprehensive long-term follow-up, may, however, re-
duce the need for reinterventions [25]. Recently, Tsilimparis
et al. [26] published a study of patients who underwent exten-
sive endovascular aortic stent-graft coverage (from the aor-
tic arch to abdominal aorta). Two patients had a CTD and
were treated successfully in two stages. The mean interval be-
tween the procedures was 12.9 months. Clough et al. [27] also
presented patients with CTD who were treated with a dou-
324 S e m i n a r s i n Va s c u l a r S u r g e ry 3 5 ( 2 0 2 2 ) 3 2 0 – 3 3 3

Fig. 4 – A 55-year-old patient with Marfan syndrome after previous supracoronary ascending aortic repair, frozen elephant
trunk (FET) repair and thoracic endovascular aneurysm repair for chronic type A aortic dissection. (A) A residual false lumen
thoracoabdominal aneurysm with a maximum aortic diameter of 60 mm has developed 8 years after FET at the level of the
diaphragm. (B) A branched thoracoabdominal prosthesis has been implanted with bi-iliac extension and bilateral
false-lumen embolization at the level of the common iliac arteries. Three-dimensional volume rendering of pre- and
postoperative computed tomography angiography.

ble inner-branch device to treat the arch aneurysm with a
CTD.
5. Summary of evidence of descending
thoracic aorta disease treatment
Patients with CTD are at a high lifetime risk of developing TAA
or AoD due to fragile structural integrity of the aortic wall [28].
Indication thresholds and surgical technique, including en-
dovascular repair options, can vary among CTDs [29]. However,
open surgical repair of descending thoracic aortic pathologies
carries a high complication risk due to its invasive nature, ren-
dering TEVAR an attractive option for older and high-risk pa-
tients. Recent studies [30–45] have highlighted endovascular
options for patients with CTD and a high risk for complica-
tions from an open repair and in emergency situations as a
bridge to subsequent open operation or as a life-saving mea-
sure.
GenTAC was initiated in 2006 by the National Heart, Lung,
and Blood Institute and the National Institute of Arthritis and
Musculoskeletal and Skin Diseases. GenTAC established a reg-
istry of 3,706 patients with genetic conditions that may be re-
lated to TAAs and collected medical data and biologic sam-
ples (ClinicalTrials.gov Identifier: NCT01322165). GenTAC has
shown that TEVAR can be life-saving in patients with CTD
with acute AoD, although it may be associated with risk for
retrograde dissection [30]. For patients with CTD with chronic
AoD and dissecting aneurysm, TEVAR may be a valid treat-
ment option in patients who are considered high risk for open
surgical repair [30,31].
Le Huu et al. [32] presented 37 patients (27 MFS, 6 LDS, 2
Turner syndrome, 1 neurofibromatosis, and 1 familial thoracic
aneurysm) treated for post-dissecting aneurysm and pseu-
doaneurysm with acceptable early outcomes in patients with
CTD. Most of the patients had previous aortic intervention;
25 underwent ascending aorta or aortic root surgery previ-
ously, and 21 underwent open thoracoabdominal surgery pre-
viously. Eleshra et al. [33] presented eight patients who under-
went TEVAR in proximal native zone 2 aorta for chronic type B
AoD, highlighting that TEVAR in native proximal landing zone
(PLZ) 2 seems safe and effective, with no early or midterm
complications in patients with MFS. However, one should be
cautious with the same technique for patients with LDS and
Table 1 – Study characteristics and outcomes on patients with CTD who were treated with thoracic endovascular aneurysm repair for thoracic aortic disease pathologies,
published from 2005 through 2021.

Study, first Year No. of CTD type, n Pathology Age, y Technical Morbidity Mortality Follow-up Follow-up Follow-up
author patients success, % reinterven- mortality, n
tion (%)

Le Huu [32] 2021 37 MFS, 27 Dissection, n = 26 46.6 — Cardiac 3 Median: 3.6 y 13 patients, 12/37 (30)
LDS, 6 Pseudoaneurysm, complications 22
TS, 2 n=6 (n = 6; 16.2%), interventions
FD, 2 DTA, n = 4 renal failure
Other, n = 1 (n = 5; 13.5%), SCI
(n = 3; 8.1%).
Eleshra [33] 2021 31 MFS, 24 — — 100 0 0 40 mo 2 ETIa, FET, 1/31 (3)

S e m i n a r s i n Va s c u l a r S u r g e ry 3 5 ( 2 0 2 2 ) 3 2 0 – 3 3 3
LDS, 5 and arch
EDS, 2 branch
Burdess [34] 2021 6 DTA aneurysm 41.5 100 — 0 23 0 0
Qato [35] 2020 102 MFS, 88 Degenerative 50.6 (range, 95.2 2 SCI (2%), 4 limb 2 (2%) 15.6 mo 3% early; 2% 6
EDS, 9 aneurysm 25%; 57 to 75) ischemia (4%); 3 late
LDS, 5 TBAD 75% arterial injury
(3%)
Pellenc [36] 2020 18 MFS Dissection Mean ± SD: — 1 renal failure 0 Mean ± SD: 23.5 10 1
47 ± 17 ± 14
Shalhub [30] 2018 31 MFS, 7 DTA dissection Mean ± SD: — 3 retrograde 0 Median: 2 y 10 2/31
LDS, 1 42.9 (11.2) dissection; 1
EDS, 1 renal failure
Other, 22
Kölbel [37] 2018 3 MFS, 2 Dissection n = 2 35.6 100 — 0 — 1 at 4 mo 1 at 4 mo
LDS, 1 Aneurysm n = 1
Kalra [38] 2015 2 LDS Aneurysm and 100 0 0 — 0 0
dissection
Eid-Lidt [39] 2013 10 MFS Dissection Mean ± SD: — — 1 Mean ± SD: 59.6 33.3% 1
35.1 ± 9.4 ± 38.9
Waterman 2012 16 MFS Aneurysm and — — — Median: 9.3 mo 7 4
[40] dissection
Nordon [41] 2009 7 MFS Aneurysm and Mean ± SD: 100 0 1 16 mo 2 0
dissection 45.9 ± 10
Botta [42] 2009 12 MFS Dissection Mean ± SD: 100 0 0 Median: 31 mo 1 0
37.8 ± 11
Geisbüsch 2008 8 MFS, 6 Dissection 48 (range, 32 88 2 0 31 mo (range, 3 38% 1
[43] EDS, 2 to 67) to 79 mo)
Marcheix [44] 2008 15 MFS Aneurysm and Mean ± SD: 100 1 TIA 0 Mean ± SD: 2.1 ± 3 early, 5 late 3
dissection 38.7 ± 12 1.4 y
Ince [45] 2005 6 MFS Dissection Mean ± SD: — — 0 Mean ± SD: 51 ± 3 1
33 ± 15 22 mo
2005- 304 — — — 88 to 100 (only 1 — 5/295 (1.6%) — — 33 deaths
2021 study 88)

Abbreviations: CTD, connective tissue disease; DTA, descending thoracic aneurysm; EDS, Ehlers-Danlos syndrome; ETIa, endoleak Type Ia; FD, familial disease; FET, frozen elephant trunk; LDS,

325
Loeys-Dietz syndrome; MFS, Marfan syndrome; SCI, spinal cord ischemia; TS, Turner syndrome; TBAD, type B aortic dissection; mo: months; y: years.
 326
S e m i n a r s i n Va s c u l a r S u r g e ry 3 5 ( 2 0 2 2 ) 3 2 0 – 3 3 3
Table 2 – Study characteristics and outcomes on patients with connective tissue disease who were treated for thoracoabdominal aortic aneurysm using fenestrated
and/or branched devices, published from 2017 through 2021.

Study, first Year Patients, n CTD type Pathology Device Technical Morbidity Mortality Follow-up Follow-up Follow-up
author success reinterven- mortality
tion

Eleshra [48] 2021 4 — TAAA T-branch 100 0 0 — — —

Eleshra [31] 2021 2 EDS TAAA T-branch/5- 100 0 0 3.5 y and 1 y 1-y LSA PTA 0
FEVAR and 3 mo PTA
renal stents
Tsilimparis 2020 2 — Post- F/BEVAR 100 — — — —
[26] dissection
Spear [25] 2018 1 — Post- FEVAR 100 0 0 — — 0
dissection
Clough [27] 2017 16 4 MFS/12 TAAA 13 FEVAR, 1 100 0 0 Mean: 3.4 y 1 at 2 y 1 at 2 y
highly BEVAR, 2
suspicion F/BEVAR
Hashizume 2017 1 LDS TAAA FEVAR 100 0 0 — — —
[49]

Abbreviations: BEVAR, branched endovascular aneurysm repair; CTD, connective tissue disease; EDS, Ehlers-Danlos syndrome; F/B, fenestrated/branched; FEVAR, fenestrated endovascular aneurysm
repair; LDS, Loeys-Dietz syndrome; LSA, left subclavian artery; MFS, Marfan syndrome; PTA, percutaneous angioplasty; TAAA, thoracoabdominal aortic aneurysm.
 S e m i n a r s i n Va s c u l a r S u r g e ry 3 5 ( 2 0 2 2 ) 3 2 0 – 3 3 3 327

it should only be used in emergencies as a bridge to open
repair [33].
In 15 studies [30,32–45] on patients with CTD that were
treated with TEVAR for thoracic aortic disease pathologies,
published from 2005 to 2021, 304 patients were included (Table
1). The technical success rate ranged from 95% to 100%, except
one from 2008 that provided an 88% technical success rate.
The early mortality rate was 1.6% (5 of 295). Most common
complications were renal failure, spinal cord ischemia, and
cardiac (Table 1). The median follow-up ranged from 9 to 43
months and mean follow-up ranged from 15.6 to 59.6 months.
During follow-up, 33 patients died from various causes and 64
patients underwent a re-intervention.
Recently another technique was used in patients with CTD
for AoD treatment; stent-assisted, balloon-induced, intimal
disruption and re-lamination of AoD was demonstrated as a
feasible and safe option with a 100% technical success rate
[46]. There was no in-hospital death, 30-day postoperative
stroke, spinal cord ischemia, ischemic colitis, or renal failure
requiring dialysis. At a median follow-up of 15 months (range,
7 to 28 months), this endovascular approach was associated
with an immediate and midterm persisting aortic remodeling
[46].
6. Summary of evidence on
thoracoabdominal aortic disease treatment
Traditionally, thoracoabdominal aortic pathology in patients
with CTD was treated with open surgical repair. Recently, it
was shown that, in experienced centers during a 16-year pe-
riod, 300 patients with presumed CTD underwent 178 thora-

Fig. 5 – Computed tomography angiography (CTA) of 49-year-old female patient with Marfan syndrome with aortic root
aneurysm and a chronic type B aortic dissection with a symptomatic large false-lumen aneurysm after thoracic
endovascular repair (TEVAR) and provisional extension to induce complete attachment stenting. (A, B) Preoperative CTA. (C,
D) During urgent TEVAR relining and TEVAR extension to the celiac artery and Candy-Plug implantation in the false lumen.
(E) CTA after aortic root and arch repair and frozen elephant trunk implantation.
328 S e m i n a r s i n Va s c u l a r S u r g e ry 3 5 ( 2 0 2 2 ) 3 2 0 – 3 3 3
Fig. 6 – Distal stent-graft–induced new entry tear after thoracic endovascular repair in chronic aortic dissection.
coabdominal aortic repairs, the operative treatment enabled
excellent long-term survival [47]. Guidelines state an open sur-
gical preference in patients with CTD, despite the known sur-
gical challenges due to vessel-wall weakness or the morbidity
that open repair might have [13–17].
Experienced centers have published their outcomes on en-
dovascular treatment of patients with TAAA using F/B devices
(Table 2). In 6 studies [25–27,31,48,49], 26 patients with CTD
were treated with F/BEVAR for TAAA, with a technical suc-
cess of 100% in all cases, without early mortality and mor-
bidity (Table 2). During the mid-term follow-up, only one pa-
tient died, and three patients needed a re-intervention. Other
options are still hybrid repair [50], which can include two
stages—first total debranching of the visceral vessels and then
TEVAR—however, this remains an invasive procedure. In some
cases, plain EVAR can also be a treatment option [51] when
anatomy allows such an approach or proximal neck stabiliza-
tion is needed [52].
7. Strategy approach and areas of concern
TEVAR complications associated with the PLZ, such as retro-
grade TAAD, endoleaks, aneurysmal progression, and rupture,
which may mandate a re-intervention (14% to 60%), remain a
matter of concern in patients with CTD [30,39,40,46]. We gen-
erally prefer a PLZ within Dacron grafts. Thus, the first step of
elective aortic repair in patients with CTD with an indication
for intervention in the ascending aorta is an ascending aortic
repair that could be followed in a second stage by a branched
arch endograft for the aortic arch segment, which may be com-
bined with a TEVAR procedure (Fig. 5). Regarding the distal part
for TEVAR of the landing zone, some centers have established
treatment protocols including sizing rules when landing in
native distal aorta [34–36,40].
Patients with CTD are generally considered poor candi-
dates for TEVAR, although this may differ between CTD en-
tities and PLZs [33]. Standard TEVAR devices exert high radial
forces on the vessel wall in the PLZ in order to achieve ade-
quate sealing. This may be a risk for patients with CTD with
known fragile aortic segments. The radial force of the distal
stent-graft may tear the dissection membrane, thereby caus-
ing a new entry tear at the distal edge of the stent-graft, the
so-called stent-graft–induced new entry tear (SINE) (Fig. 6) [53].
We recently presented a technique in 3 patients with
good outcomes using a physician-modified thoracic stent-
graft with low distal radial force to prevent distal SINE tears
in patients with CTD and AoD [37]. A commercially available
 S e m i n a r s i n Va s c u l a r S u r g e ry 3 5 ( 2 0 2 2 ) 3 2 0 – 3 3 3 329

Fig. 7 – (A) Physician-modified, low distal radial force (LDRF) stent-graft. The distal seal stent is removed from a conventional
stent-graft and the distal graft edge marked by hemostatic clips. The diameter of the lowest remaining stent is reduced by
sutures. (B) The LDRF stent-graft is used in the true lumen with a Candy-Plug placed in the false lumen to prevent distal
backflow.

thoracic stent-graft was modified on a back table by remov-
ing the distal Z-stent and marking the distal fabric with vas-
cular clips to reduce the stent-induced pressure on the dis-
section membrane [37] (Fig. 7). A recent study [34] presented
the short- and mid-term outcomes of a similar custom-made
dissection-specific stent-graft specifically designed to address
the features of chronic type B AoD and reduce the risk of dis-
tal SINE. Six of 16 patients that were treated had a known CTD.
The technical success was 100%, without early mortality [34].
As CTD has been suggested as a risk factor for SINE in gen-
eral, these specific techniques may reduce the risk of SINE in
patients with CTD.
It is known that the fate of false lumen plays a role in aor-
tic remodeling in AoD; persistent perfusion from distal en-
try tears may lead to late false lumen expansion (up in 30%)
requiring additional re-interventions [54,55]. Patients with
patent false lumen have worse long-term survival in chronic
type B AoD [56,57]. Another important issue is the monitor-
ing of patients with CTD treated with complex endovascular
techniques, as those mandate close follow-up; especially in
330 S e m i n a r s i n Va s c u l a r S u r g e ry 3 5 ( 2 0 2 2 ) 3 2 0 – 3 3 3

Fig. 8 – (A, B, C) Follow-up computed tomography angiography of the same patient 6 years after repair demonstrating
continued full exclusion of the false-lumen aneurysm with significant remodeling.
 S e m i n a r s i n Va s c u l a r S u r g e ry 3 5 ( 2 0 2 2 ) 3 2 0 – 3 3 3
patients with CTD, when the risk of secondary intervention
remains high through the follow-up period [40]. In addition,
endovascular approach of TAAA, especially in post-dissection
patients, mandates adjunctive techniques to achieve false lu-
men thrombosis at the level of splanchnic vessels with various
approaches [57]. In our experience, the Candy-Plug technique
has been proven to be technically feasible with low mortality
and morbidity and a high rate of aortic remodeling [58] (Figs.
5E, 7B, and 8). In addition, the improved-generation Candy-
Plug reduces the procedural steps and provides a longer seal-
ing segment [58]. This device can be also used in patient with
CTD, as we have shown in a recent cohort study on patients
with CTD [37].
Another issue may be the presence of re-entry tears located
in the target vessels. A treatment method is to position a wire
in the false lumen of such vessel. Someone can complete the
deployment of endograft and the covered bridging stents, and
then occlude the false lumen either with an Amplatzer plug
or coils using the wire in place.
Finally, in the near future, it will be important to establish
the role of the vascular physician throughout the cycle of care
of patients with CTD and the evolving inclusion of such pa-
tients’ outcomes in research. The understanding of manage-
ment of patients with CTD is essential for the development of
effective health care policies that support equitable, appropri-
ate, and patient-centered clinical practices [59].
8. Conclusions
Endovascular treatment of aortic pathologies in patients with
CTD seems to be feasible and safe in high-risk and urgent pa-
tients during the early and mid-term periods. Re-intervention
remains an issue. The constant development of endovascular
techniques and devices may provide improved mortality and
morbidity outcomes, but longer follow-up is needed.
Declaration of Competing Interest
The authors declare that they have no known competing fi-
nancial interests or personal relationships that could have ap-
peared to influence the work reported in this paper.
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