General Review

The Shaggy Aorta Syndrome: An Updated

Review
Raffaele Serra,1,2 Umberto Marcello Bracale,3 Federica Jiritano,4 Nicola Ielapi,1,5
Noemi Licastro,2,3 Michele Provenzano,6 Michele Andreucci,6 Armando Pingitore,7
Stefano de Franciscis,1,2 Pasquale Mastroroberto,4 and Giuseppe Filiberto Serraino,4
Catanzaro, Naples and Roma, Italy

Background: Shaggy aorta (SA) depicts the severe aortic surface degeneration, extremely
friable, and likely to cause spontaneous peripheral and visceral embolization or during catheter-
ization, aortic manipulation, surgery, or minimally invasive procedures. This study aims to pro-
vide the most accurate and up-to-date information on this disease.
Methods: Potentially eligible studies to be included were identified by searching the following
databases: CENTRAL Library, ClinicalTrials.gov, MEDLINE, and CINAHL, using a combination
of subject headings and text words to identify relevant studies: (Shaggy aorta) OR (aortic embo-
lization) OR (aortic embolism) OR (aortic thrombus) OR (aortic plaque). From a total of 29,111
abstracts, and after applying inclusion and exclusion criteria, we considered 60 studies for inclu-
sion in this review.
Results: Appropriate measurement and assessment of the aortic wall are pivotal in the modern
era, in particular when percutaneous procedures are performed, as SA has been identified as an
independent risk factor for spinal cord injury, mesenteric embolization, and cerebral infarction
after endovascular aortic repair. Furthermore, SA increases the rate of cerebral complications
during transcatheter aortic valve implantation.
Conclusions: In conclusion, prompt diagnosis of SA syndrome and appropriate guidelines on
the management of these conditions may help physicians to better assess the patient risk and to
minimize the dreadful-related complications.

6
RS and UMB contributed equally and share the first authorship. Department of Health Sciences, University of Catanzaro, Cata-
PM and GFS are contributed equally and share the last authorship. nzaro, Italy.
Funding: All authors have no source of funding. 7
Department of Radiology, Pugliese-Ciaccio Hospital of Catanzaro,
Competing Interest: All authors have no conflict of interests. Catanzaro, Italy.
1
Interuniversity Center of Phlebolymphology (CIFL). International Correspondence to: Prof. Raffaele Serra, MD, PhD, Interuniversity
Research and Educational Program in Clinical and Experimental Center of Phlebolymphology (CIFL)., International Research and
Biotechnology’’ at the Department of Medical and Surgical Sciences, Educational Program in Clinical and Experimental Biotechnology’’ at
University Magna Graecia of Catanzaro, Catanzaro, Italy. the Department of Medical and Surgical Sciences, University Magna
2
Department of Medical and Surgical Sciences, University of Cata- Graecia of Catanzaro, Viale Europa 88100 Catanzaro, Italy Viale
nzaro, Catanzaro, Italy. Europa, Localit
a Germaneto, 88100 Catanzaro. Italy; E-mail:
3
Department of Public Health, University of Naples ‘‘Federico II’’, rserra@unicz.it
Naples, Italy. Ann Vasc Surg 2020; -: 1–14
4
Department of Experimental and Clinical Medicine, University of https://doi.org/10.1016/j.avsg.2020.08.009
Catanzaro, Catanzaro, Italy. Ó 2020 Elsevier Inc. All rights reserved.
5 Manuscript received: June 16, 2020; manuscript accepted: August 5,
Sapienza’’ University of Rome, Department of Public Health and 2020; published online: - - -
Infectious Disease, Roma, Italy.

1
2 Serra et al. Annals of Vascular Surgery

INTRODUCTION assessed using the Downs and Black quality check-

list4: this system is a well-validated, reliable, and
Embolization is a common, and life-threatening methodologically strong tool.5 There were no exclu-
complication of severe aortic atherosclerosis, which sion criteria.
causes ischemic damage to the involved organs.
Lipids accumulation in the inner aortic layers, as a
result of the atherosclerotic process, with fibrous tis-
sue deposition and surface erosions, may cause RESULTS
either thrombotic (thromboembolic) or atheroscle- A total of 29,111 abstracts were retrieved from the
rotic (cholesterol crystal) embolism.1,2 Diffuse searches. There were 29,043 articles excluded for
atheromatous embolization must be distinguished duplicate publication or excluded on the basis of title
from the focal embolization of thrombotic material and abstracts. A total of 68 relevant publications
originating from cardiac chambers or peripheral an- were retrieved for further assessment.3,6e62
eurysms occluding small arteries and arterioles, and After reading the text, 8 studies were excluded
causing the ‘‘blue-toe’’ syndrome, renal insufficiency because not relevant,63e70 and 60 included in our
or mesenteric ischemia. The evidence, at visual in- review (Fig. 1). Studies present were available
spection, on imaging of an irregular and spiculated only as abstracts,6,40,48,54,71 23 were observational
atheroma is commonly called ‘‘Shaggy Aorta’’ studies,3,6,7,14,18,21,27,29e33,37e39,41,43,45,46,49,51,57,62
(SA).3 This term well depicts the severe aortic sur- 28 were case reports,8e13,15,17,19,24e26,28,34e
face degeneration, extremely friable, and likely to 36,40,44,47,50,52e56,71,72
2 were reviews,20,48 6 were
cause atheroembolism. Spontaneous peripheral comments on the topic,22,23,42,58,59,61 1 was a clin-
and visceral embolization from diffuse aortic athero- ical image report,60 and 1 was a study protocol16
sclerotic disease has been termed as the "SA Syn- (Table I). Definitions of ‘‘SA’’ vary between
drome,’’ first described by Hollier et al. in 1991.3 studies,3,33,41 making comparisons difficult. The
SA is also associated with the high risk of diffuse em- morphological characteristics of SA were not fully
bolism after catheterization, aortic manipulation, understood because direct visualization was consid-
surgery, or minimally invasive procedure such as ered too dangerous before the study by Komatsu
transcatheter aortic valve implantation (TAVI) or et al. described aortic plaques and injuries using a
endovascular aneurysm repair (EVAR).1 Since SA 6F percutaneous nonobstructive angioscopy
was first described, many changes have been made (NOA) system with a rotational pull back. Puff-like
in preventive medicine, imaging, and treatment, ruptures (responsible for atheromatous emboliza-
determining new definitions and issues also in the tion because debris scatters from these plaques),
related field of acute aortic syndromes, and growing chandelier-like ruptures (suggesting the presence
up emerging considerations.2 The aim of this study of cholesterol crystals), and erosions were described
was to provide the most accurate and up-to-date as the characteristics of vulnerable plaques.47 The
literature review on this issue. incidence of SA in patients with thoracoabdominal
aneurysms (TAA) was found between 11% and
MATERIALS AND METHODS 14.3%.41,57 Even in experienced hands, during
open TAA repair, the incidence of mortality or major
Potentially eligible studies to be included in the re- complications is about 30% in the presence of irreg-
view were identified by searching the Cochrane ularly shaped atherosclerotic aortic wall.57 Interest-
Central Register of Controlled Trials (CENTRAL; ingly, Nemoto et al. showed, in a pilot study, that
Internet), ClinicalTrials.gov, MEDLINE (PubMed statins could reduce extensive thoracic atheromas
1946 to May 2020), EMBASE (Ovid 1975 to May and lower lipid concentrations in abdominal aortic
2020), and the Cumulative Index to Nursing and Al- aneurysm (AAA) patients who underwent
lied Health Literature (CINAHL) Plus (1979 to May contrast-enhanced computed tomography (CT).32
2020), using a combination of subject headings These results were not confirmed by Maeda et al.62
and text words to identify relevant studies. The Yokawa et al.57 evaluated the impact of diffuse
Medline search strategy showed below has been aortic atherosclerosis-related thrombosis on the out-
adapted as appropriate for other databases: (Shaggy comes of open thoracoabdominal aortic aneurysm
aorta) OR (aortic embolization) OR (aortic embo- (TAAA) repair. A multivariate analysis demon-
lism) OR (aortic thrombus) OR (aortic plaque). strated that SA was a significant risk factor for com-
The last search was run on May 15, 2020. In addition posite outcome such as operative mortality, spinal
to searching databases, we searched in trial registries cord injury, and acute renal failure, concluding
and checked the reference lists. All studies were that preoperative CT scan assessment of the aorta
Volume -, - 2020 Shaggy aorta syndrome 3

Fig. 1. Flow diagram of study inclusion.

could predict high-risk patients for open TAAA represent a valid option in patients with SA.72 In
repair. Takano et al.44 described the use of an inter- ascending aortic aneurysm, complicated with
mittent clamping of the visceral and carotid arteries atherosclerotic lesions of the descending aorta, a
under an extracorporeal circulation circuit without hybrid approach using frozen elephant trunk tech-
a blood flow pump, in a patient with multiple aortic nique could be a valid treatment to reduce the em-
aneurysms and SA treated by conventional open bolism risk.54 Uchida et al.28 reported a modified
repair for an AAA, and endoluminal stent grafting arch-first technique in a patient with arch aneurysm
for a thoracic aortic aneurysm. In his experience, complicated by SA, involving arch replacement with
this led to the avoidance of embolization. SA repre- a beating heart after reconstruction of supra-aortic
sents also a significant determinant of neurologic vessels while maintaining normal blood pressure.
morbidity during open aortic arch surgery.45 To pre- SA has been identified as an independent risk fac-
vent the risk of stroke during aortic arch repair, tor for spinal cord injury49 or mesenteric emboliza-
novel procedures of selective cerebral perfusion tion46 after thoracic endovascular aortic repair
have been developed, showing an acceptable risk (TEVAR). SA represents an independent risk factors
for elderly patients with SA.14,18,21,51,71 Single- for cerebral infarction after EVAR,37,39 also after left
stage hybrid arch repair, which involved ascending subclavian artery coverage.38 Bowel ischemia also
aorta replacement and debranching of arch vessels, represents a life-threatening complication of
consecutively with endovascular repair could EVAR. In fact, the evidence of irregular aortic
Table I. Characteristics of included studies

4 Serra et al.
Study Diagnostic
(author Study Number of imaging
year) Country period Study design patients Age, years technique Treatment Inclusion criteria Exclusion criteria Outcomes Main findings

Hollier USA 1962e1989 RC 88 69 (48e83) Arteriography Surgical treatment: Clinical findings n.a. Mortality Mortality
3
1991 Suprarenal compatible with Renal failure Renal failure
endarterectomy ‘‘shaggy’’ aorta: Visceral Visceral
or suprarenal diffuse embolization embolization
graft ± lumbar microembolization
sympathectomy of the lower trunk,
Infrarenal with livedo
graft + lumbar reticularis of the
sympathectomy buttocks, thighs,
Axillobifemoral legs, and feet, and
bypass with frequently with
external iliac multiple ischemic
ligation, artery toes; diffuse renal or
ligation and lum- visceral
bar microembolization;
sympathectomy diffuse peripheral
Medical treatment embolization)
Nypaver USA 1985e1992 Retrospective 43 67 (48e91) Arteriography Surgical treatment: Abdominal aortic Isolated renal or Major Mortality
1993 7 NRCT Prosthetic graft replacement with visceral complication/ Renal failure
reconstruction supraceliac aortic revascularization ischemia
of the abdominal cross-clamping Emergency involving the
aorta operation kidney, liver,
Aortic endar- Aneurysms bowel, or spinal
terectomy, right extending to or cord
renal involving the Renal
endarterectomy, origins of the dysfunction
left renal celiac axis or Vital organ
endarterectomy, superior ischemic
celiac endarterec- mesenteric artery complications
tomy, SMA
endarterectomy

8
Bols 1994 Belgium n.a. Case series 5 n.a. Arteriography Medical therapy n.a. n.a. Mortality Mortality
with oral Peripheral Amputations
anticoagulation; embolization Skin lesions
surgical vascular Skin lesions

Annals of Vascular Surgery

replacement
 Volume
Zempo Japan n.a. Case report 1 78 CTA TPEG n.a. n.a. Mortality for Cerebral infarction
9
2001 diffuse DIC
embolization: MOF
Cerebral

-, -
infarction
Renal

2020
dysfunction
Liver dysfunction
Bowel ischemia
DIC
MOF
Hayashida Japan n.a. Case report 1 59 Arteriography; Thrombectomy and n.a. n.a. Mortality for Mortality
2004 10 CTA unfractionated diffuse Inferior mesenteric
heparin embolization artery occlusion
administration Renal failure
Bowel ischemia
Lower extremity
ischemia
Sanada Japan n.a. Case report 1 75 Arteriography; Hybrid treatment Use of a homemade n.a. Peripheral Procedure-related
11
2005 CTA (aortic debranching, intra-aortic filter embolization embolic events
TEVAR) device during Neurological deficits
TEVAR
Illuminati Italy n.a. Case report 1 65 CTA Hybrid treatment ‘‘Blue toe’’ syndrome n.a. Peripheral Embolism at FU
2007 12 (TEVAR + embolization
aortobifemoral 26-month FU
bypass)
Tanaka Japan n.a. Case report 1 76 CTA Thrombectomy, Acute thrombosis of n.a. Renal failure Peripheral arterial
13)
2010 aortobifemoral abdominal aortic Other organ vascularization
bypass aneurysm dysfunction Renal failure
Okada Japan 2002e2010 Retrospective 321 69.8 ± 13.3 CTA Total arch replacement Aortic arch disease n.a. Mortality Mortality
14
2012 NRCT Permanent Permanent
neurologic neurologic
dysfunction dysfunction
Aorta-related Sepsis
events at 3-year Gastrointestinal tract
and 5-year FU necrosis
Pulmonary failure
Hemorrhage
PAU
Hori 2012 Japan n.a. Case report 1 68 CTA TEVAR Symptomatic n.a. Peripheral Symptoms
15
descending aortic embolization improving
aneurysm Renal failure
Hoshina_a Japan n.a. Case report 1 70 CTA Abdominal aortic Abdominal aortic n.a. DIC Thrombocytopenia

Shaggy aorta syndrome 5

2012 16 replacement aneurysm and DIC
coagulation
disorders
Igarashi Japan n.a. Case report 1 82 CTA Hybrid treatment Aortic aneurysm n.a. Neurologic No complications
2013_a (ascending aorta complications
17
replacement, aortic Peripheral
debranching, embolization
TEVAR) 30-day FU
(Continued)
Table I. Continued

6 Serra et al.
Study Diagnostic
(author Study Number of imaging
year) Country period Study design patients Age, years technique Treatment Inclusion criteria Exclusion criteria Outcomes Main findings

Okita_a Japan 2002e2012 Retrospective 423 69.2 ± 13.1 n.a. Total arch Aortic arch disease n.a. Mortality Mortality
18
2013 NRCT replacement Permanent Permanent
neurologic neurologic
dysfunction dysfunction
Peripheral Sepsis
embolization Bowel ischemia
Aorta-related Pulmonary failure
events at FU Hemorrhage
PAU
Jinno 2013 Japan n.a. Case report 1 75 CTA Surgical aortic Symptomatic TAAA n.a. Postoperative Symptoms
19
replacement respiratory improving
complication No complications
Takagi Japan 2005e2010 RC 63 73 ± 9 CTA Total arch replacement Nondissecting arch n.a. Mortality Mortality
21
2013 aneurysm Cerebral Stroke
Preoperative CTA embolization Re-exploration for
evaluation bleeding
Renal failure
Respiratory failure
Hoshina_b Japan n.a. Case report 1 62 CTA Intermittent clamp Thoracic aortic n.a. Peripheral Pseudomembranous
2013 24 technique for all aneurysm and embolization enterocolitis
visceral arteries ascending colon Liver dysfunction Peripheral
during TEVAR; carcinoma Renal embolization
right dysfunction
hemicolectomy Bowel ischemia
Igarashi Japan n.a. Case report 1 83 CTA Hybrid treatment TAAA; emboli n.a. Mortality Renal failure
2013_b (debranching, filtration Peripheral
25
TEVAR) embolization
Organ
dysfunction
Endoleaks
Paraplegia
Wada T Japan 2008e2009 Retrospective 122 EVAR CTA Surgical aortic Abdominal aortic n.a. Mortality Mortality
26
2015 NRCT (74.4 ± 8.1) replacement; aneurysm Peripheral Cerebral infarction
Open repair EVAR embolization Ischemic colitis
(69.7 ± 9.4) HLOS Lower limb ischemia
Endoleaks Myocardial
infarction

Annals of Vascular Surgery

Wound infection
Endoleak (type 1,
type 2, type 3)
Wada H Japan n.a. Case report 1 76 CTA Surgical ascending Aortic arch disease n.a. Peripheral Transient delirium
2014 27 aorta, arch and embolization Right hemiparesis
descending aorta Neurologic caused by small
replacement, dysfunction cerebral infarction
debranching, Symptoms
coronary bypass improving
 Volume
Uchida Japan 2002e2012 RC 438 69.1 ± 13.4 n.a. Total arch replacement Aortic arch disease n.a. Mortality Mortality
28
2013 Permanent Permanent
neurologic neurologic
dysfunction dysfunction

-, -
Peripheral Sepsis
embolization Bowel ischemia

2020
Aorta-related Pulmonary failure
events at FU Hemorrhage
PAU
Okita_b France 2009e2011 Prospectively 235 74 (38e93) n.a. Open surgical Abdominal aortic Ruptured AAA Mortality Mortality
29
2013 NRCT replacement, aneurysm treated Nonruptured AAA Surgical risk Vascular
EVAR, or LAS with open surgery, operated in Vascular complications
EVAR, or LAS emergency complications Myocardial
Patients not Endoleaks infarction
suitable for open Infections MOF
surgery, EVAR, or Endoleaks
LAS Infections
Coscas Japan 2006e2009 RC 539 76 (36e95) CTA EVAR Abdominal aortic Fenestrated or Mortality Mortality
30
2014 aneurysm branched Endoleaks Endoleaks
Elective EVAR endovascular Spontaneous Spontaneous graft
grafting graft thrombosis thrombosis
Isolated iliac artery FU Peripheral
aneurysms Peripheral embolization
Custom-made embolization (cerebral, limb,
grafts bowel)
Toya 2014 Japan 2002e2011 RC 29 Statin group: CTA Medical therapy SA patients with n.a. Atheromatous Lipid lowering
31
78.4 ± 6.1 with statins abdominal aortic area Atheromatous area
Nonstatin group: aneurysm Serum lipid variation
77.1 ± 6.3 concentrations
FU
Peripheral
embolization
Nemoto UK 2008e2013 RC 99 Mesenteric CTA Fenestrated EVAR Patients with n.a. Mortality Mortality
2013 32 ischemia group: paravisceral Mesenteric Mesenteric ischemia
78 ± 12 aneurysms ischemia Renal dysfunction
Nonmesenteric Renal Endoleaks
ischemia group: dysfunction
73 ± 18 Endoleak
FU
Patel 2014 Japan 2005e2010 RC 8 72.4 ± 8.0 CTA Surgical aortic TAAA n.a. Paraplegia No complications
33
replacement Mortality
(Continued)

Shaggy aorta syndrome 7

Table I. Continued

8 Serra et al.
Study Diagnostic
(author Study Number of imaging
year) Country period Study design patients Age, years technique Treatment Inclusion criteria Exclusion criteria Outcomes Main findings

Takahashi USA 1962e1989 RC 88 69 (48e83) Arteriography Surgical treatment: Clinical findings n.a. Mortality Mortality
2014 34 Suprarenal compatible with Renal failure Visceral
endarterectomy ‘‘shaggy’’ aorta: Visceral embolization
or suprarenal diffuse embolization
graft ± lumbar microembolization
sympathectomy of the lower trunk,
Infrarenal with livedo
graft + lumbar reticularis of the
sympathectomy buttocks, thighs,
Axillobifemoral legs, and feet, and
bypass with frequently with
external iliac multiple ischemic
ligation, artery toes; diffuse renal or
ligation and lum- visceral
bar microembolization;
sympathectomy diffuse peripheral
Medical treatment embolization)
Murakami Japan 2014 Case Report 1 68 CTA EVAR SA patient n.a Peripheral Stroke embolism
2015 35 embolism
Sawazaki Japan 2015 Surgical 2 n.a. CTA Surgical epiaortic Arch aneurysm in SA n.a. Peripheral Neurologic
2015 36 technique vessels isolation patients embolism complications
technique
Morimoto Japan 2007e2012 Case Series 6 73.5 (70e87) CTA Hybrid Repair Subclavian Artery n.a. Mortality Mortality
2015 37 Aneurysm Peripheral
associated with embolism
atherosclerosis e Endoleak; graft
patency
Baba 2015 Japan 2006e2014 RC 178 75.1 (mean) CTA TEVAR Distal Arch Aneurysm n.a. 30-day mortality Cerebral infarction
38
Perioperative Postoperative
complications endoleak
HLOS
Kanaoka Japan 2006e2013 RC 439 74 (mean) CTA TEVAR Fusiform descending Surgery for acute or 30-day mortality Cerebral infarction
2016 39 TAA > 55 mm or chronic aortic Comorbidities
saccular dissection or
TAA > 60 mm or emergent surgery
saccular aneurysm
unfit for open

Annals of Vascular Surgery

surgery
Kwon Korea 2009e2012 RC 447 69 SD 8 Contrast EVAR, OSR Elective AAA repair Ruptured AAA 30-day mortality Mortality
41
2016 (nonshaggy enhanced
patients) CT
71 SD7 (shaggy
patients)
 Volume
Toya 2016 Japan 2011e2014 RC 135 77 (57e93) CTA EVAR Elective Abdominal Acute myocardial or Mortality Serum creatinine
43
Aortic Aneurysm cerebral infarction Chronic kidney
(>5 cm diameter) or recent blue toe disease
or saccular syndrome

-, -
morphology
Takano Japan 2016 Case Report 1 74 CTA Endoluminal therapy Saccular TAA n.a. Perioperative Embolization

2020
2016 44 complications
Imasaka Japan 2008e2015 RC 200 71 SD 12 MRA Aortic surgery with Elective aortic arch n.a. In-hospital Stroke
2017 45 selective antegrade surgery mortality, stroke
cerebral perfusion Neurological
and moderate morbidity,
hypothermic myocardial
circulatory arrest infarction, renal
failure
Reexploration for
bleeding
Acosta Finland 2011e2015 Case Control 9 69 (50e81) CTA EVAR, endovascular Aortic aneurysm; n.a. -Mesenteric Smoking cessation,
46
2017 Study repair for aortoiliac aortoiliac occlusive embolization, patient selection
occlusive disease disease In-hospital and procedure
mortality, planning as risk
Biochemical factors
markers
Komatsu Japan 2017 Case Report 1 70 CTA, NOA NOA SA n.a. Embolism, renal NOA is safe
2017 47 function
Inflammatory
markers
Hiraoka Japan 2008e2014 RC 175 75.7 SD 5.6 (SCI CTA TEVAR Aortic Aneurysms, Reintervention for Neurological SA, COPD, aortic
49
2018 group) aortic dissection, prior TEVAR complications coverage length as
71.8SD10.1 (No pseudo aneurysms, risk factors for SCI
SCI group) traumatic and
mycotic aneurysms
Murono Japan 2018 Case Report 1 77 CTA Laparoscopic small SA n.a. Embolism Cholesterol crystals
2018 50 bowel resection
Kasama Japan 2010e2016 RC 99 80 SD 3 Contrast- Surgical aortic arch Aortic Aneurysms Type A aortic 30-day mortality, ICP is safe in SA
2019 51 enhanced repair with ICP dissection neurological
CT complications
renal failure,
mediastinitis
52
Yu 2019 Japan 2018 Case Report 1 69 CTA Surgical total arch Saccular Aortic n.a. Perioperative Postoperative
replacement Aneurysm with complications complications
MAS
Ryomoto Japan 2010e2017 RC 32 77 (mean) CTA TEVAR supported by Aortic arch disease Zone 0 proximal Postoperative Perioperative stroke
2019 53 mini-CPB landing respiratory

Shaggy aorta syndrome 9

support, stroke,
mortality
Mori 2019 Japan 2018 Case Report 1 84 CTA, MRI TAVI Aortic valve stenosis n.a. Neurologic SCI
55
complications
Nomura Japan 2018 Case Report 1 89 CTA TAVI Aortic valve stenosis n.a. Perioperative Cerebral embolism
2019 56 complications
(Continued)
Table I. Continued

10 Serra et al.
Study Diagnostic
(author Study Number of imaging
year) Country period Study design patients Age, years technique Treatment Inclusion criteria Exclusion criteria Outcomes Main findings

Yokawa Japan 1999e2018 RC 251 62 SD 14 CTA Surgical TAAA repair Aortic disease CT not performed Mortality, SCI, AKI, CAD
57
2019 reexploration,
transfusion,
stroke, AKI,
HLOS
Maeda Japan 20 RC 301 74.6 SD 8.6 CTA TEVAR SA, prior aortic graft Post dissection, Embolic Score to predict
62
2019 replacement, prior ruptured and complications, postoperative
endovascular repair concomitant AKI embolic
for AAA aneurysm; re complications
intervention for
TEVAR
Oishi 2019 Japan 2019 Case Report 2 83 (66e80) 3D CTA Single stage hybrid Aortic aneurysm n.a. Perioperative No embolic
72
arch Repair complications complications
(ascending aorta
replacement,
debranching of arch
vessels, TEVAR)

AKI, acute kidney injury; CAD, coronary artery disease; COPD, chronic obstructive pulmonary disease; CTA, computed tomography angiography; DIC, disseminated intravascular coagulopathy;
FU, follow-up; HLOS, hospital length of stay; ICP, isolated cerebral perfusion; LAS, laparoscopic aortic surgery; MAS, middle aortic syndrome; mini-CPB, mini-cardiopulmonary bypass; MOF,
multiple organ failure; MRA, magnetic resonance angiography; n.a., not available; OSR, open surgical repair; PAU, penetrating aortic ulcer; RC, retrospective cohort study; SCI, spinal cord
ischemia group; TPEG, transluminally placed endovascular graft.

Annals of Vascular Surgery

Volume -, - 2020 Shaggy aorta syndrome 11

surface and increased thrombus volume in the para-

visceral segment predicts the occurrence of mesen-
teric and renal ischemia in patients treated with
fenestrated endografts.33,60
During endovascular aortic aneurisms repair, the
presence of an SA, absence of oral beta-blocker
administration and an increased preoperative creati-
nine level are independent predictors of early postop-
erative chronic kidney disease progression,43
associate with 30-day morbidity and mortality and
obviously independent predictors of embolic compli-
cations associated with the procedure.31 Poor long-
term survival was also expected in patients with
SA,41 suggesting the surgical threshold should be
pushed back among AAA patients presenting with
this aortic disease.30 It was suggested that EVAR in
SA should not be performed unless there is a consider-
able reason,27 and it was also described as an effective
method of trapping emboli in a filter involving the
femoral artery and vein in an external shunt to pre-
vent shower embolism.26 A filtration-type embolus
protection device with a nitinol basket and polyester
fabric could be introduced through a 12F sheath and Fig. 2. Extensive, irregular, and spiculated atheroma of
opened in the supraceliac aorta during the stent- the aorta at CT imaging.
graft procedure, as described by Sanada et al.11
Although some studies suggest that endovascular
approach is contraindicated for SA due to risk of and prompt diagnosis with new imaging measure-
embolization, EVAR, it may be considered as an op- ments will help to better assess the patient risk. CT
tion for aneurysm complications, like in aortoen- plays a central role in the diagnosis, risk stratifica-
teric fistula, as reported by Hori et al.15 tion, and management of aortic diseases,2 and, in
An effective and less invasive treatment for aortic Figures 2 and 3, impressive images of CT scans,
stenosis in surgical high- and medium-risk patients belonging to our department’s collection, are
is TAVI. Stroke and paraplegia55 are potential com- showed. For the majority of acute aortic syndromes
plications associated with TAVI, and SA increases such as aortic dissection and intramural hematoma,
the risk for cerebral complication. In this context, CT scan represents the preferred imaging modality
Nomura et al.56 and Sawazaki et al.36 adopted a with a very high sensitivity and specificity.73 Unfor-
modified artery isolation technique without using tunately, the accuracy of CT scan in ulcerating pla-
conventional protection devices through bilateral ques and thrombi is not well documented.74
axillary artery perfusion resulting effective for the Detailed information about the aortic wall could be
prevention of cerebral embolism. acquired from the magnetic resonance imaging
(MRI) and transesophageal echocardiography,
even if limited to some aortic segments. The evolu-
DISCUSSION tion of these imaging techniques could visualize
and measure blood flow patterns by a time-
Meticulous measurements and assessment of the resolved 3D flow-sensitive MRI,75 or adding impor-
aortic wall are crucial in the modern era where tant insights regarding aortic geometry, by a 3D ul-
percutaneous procedures represent the standard trasound imaging and contrast-enhanced
treatment for most of the aortic aneurysms and ultrasound. In open aortic surgery, epiaortic ultraso-
acute aortic syndromes. Sometimes, the aortic axis nography may reveal mobile thrombi and minimal
also represents the way to get to cardiac structures plaque, giving to the surgeon a good place to put
(i.e., aortic valve for TAVI, atrial, or ventricular the clamp.13 Interestingly, many types of advanced
septum for ventricular septal defect or atrial septal atherosclerotic plaques, not visualized by traditional
defect closure), and therefore accurate preoperative imaging were clearly demonstrated using NOA.47
evaluation of the aorta is critical.46 To minimize the NOA can now offer a qualitative and quantitative
complications related to SA syndrome, awareness evaluation of the aortic plaques. Once diagnosed,
12 Serra et al. Annals of Vascular Surgery

cardiologists, should be made on the basis of the

associated risks and benefits in patients with docu-
mented SA.62,78 What can we do to our next patient
undergoing open or endovascular treatment with
SA? Antegrade perfusion through axillary arteries
is preferable to retrograde perfusion using peripheral
cannulation in femoral arteries. Aortic manipulation
should be always avoided; epiaortic ultrasonogra-
phy could be useful when preoperative cannulation
or clamping site was not investigated as in emer-
gency cases. Moreover, segmental aortic clamping
is a not well-investigated risk factor of embolic com-
plications in severe atheromatous aorta; in this case,
circulatory arrest with hypothermia could represent
a valid alternative to reduce complications in SAs.
The next step should be the drafting of new guide-
lines on diagnosis and grading of SA on the basis of
the multicenter experiences, with an external vali-
dation of available scoring methods to derive the
best evidence on what system should be adopted.61

REFERENCES
1. Katsanos AH, Giannopoulos S, Kosmidou M, et al. Complex
atheromatous plaques in the descending aorta and the risk
of stroke: a systematic review and meta-analysis. Stroke
Fig. 3. Shaggy aorta at CT scan in which atheromatous 2014;45:1764.
spiculae are particularly evident. 2. Erbel R, Aboyans V, Boileau C, et al. 2014 ESC Guidelines on
the diagnosis and treatment of aortic diseases: document
covering acute and chronic aortic diseases of the thoracic and
abdominal aorta of the adult. The Task Force for the Diagnosis
SA has been known to be a significant risk factor for and Treatment of Aortic Diseases of the European Society of
embolic complications following various vascular Cardiology (ESC) [published correction appears in Eur Heart
J. 2015 Nov 1;36(41):2779]. Eur Heart J 2014;35:2873e926.
procedures.1,2 Maeda et al.62 developed a scoring 3. Hollier LH, Kazmier FJ, Ochsner J, et al. Shaggy" aorta syn-
system defining and quantifying SA, in a cohort of drome with atheromatous embolization to visceral vessels.
301 EVAR patients to predict embolic complications. Ann Vasc Surg 1991;5:439e44.
In this single-center, retrospective study, the entire 4. Downs SH, Black N. The feasibility of creating a checklist for
aorta was considered showing that patients with the assessment of the methodological quality both of rando-
mised and non-randomised studies of health care interven-
thrombus in the aorta segment from the sinotubular tions. J Epidemiol Community Health 1998;52:377e84.
junction to the celiac artery had a higher incidence 5. National Collaborating Centre for Methods and Tools. Qual-
of thromboembolic events, suggesting to carefully ity Checklist for Health Care Intervention Studies. Hamilton,
evaluate TEVAR for patients with high shaggy ON: McMaster University, 2008 (Updated 01 June, 2010).
Retrieved from: http://www.nccmt.ca/resources/search/9.
scores. One shaggy point was given for the presence
Accessed July 15, 2020.
of: (1) ulcer-like thrombus, (2) maximum thrombus 6. Kazmier FJ, Hollier LH. The "Shaggy" aorta. Heart Dis Stroke
thickness >5 mm, and (3) mural thrombus that oc- 1993;2:131e5.
cupies more than 2-thirds of the aortic circumfer- 7. Nypaver TJ, Shepard AD, Reddy DJ, et al. Supraceliac aortic
ence. The cutoff value of the shaggy score was 3 cross-clamping: determinants of outcome in elective abdom-
points. Previous studies used a transesophageal inal aortic reconstruction. J Vasc Surg 1993;17:868e76.
8. Bols A, Nevelsteen A, Verhaeghe R. Atheromatous emboli-
echocardiography assessment to evaluate mobile zation precipitated by oral anticoagulants. Int Angiol
aortic plaques with the limitation of the diagnostic 1994;13:271e4.
procedure.76,77 Even if the European Society for 9. Zempo N, Sakano H, Ikenaga S, et al. Fatal diffuse atheroma-
Vascular Surgery (ESVS) states that TEVAR should tous embolization following endovascular grafting for an
be considered for descending TAA > 6.0 cm and abdominal aortic aneurysm: report of a case. Surg Today
2001;31:269e73.
with favorable anatomy, an overall ad hoc assess- 10. Hayashida N, Murayama H, Pearce Y, et al. Shaggy aorta
ment, which includes several medical figures such syndrome after acute arterial macroembolism: report of a
as radiologist, vascular and cardiac surgeons, case. Surg Today 2004;34:354e6.
Volume -, - 2020 Shaggy aorta syndrome 13

11. Sanada J, Matsui O, Ohtake H, et al. Distal embolus protec- aneurysm with atheromatous plaques. Ann Vasc Dis
tion with an intra-aortic filter during stent-graft repair of a 2013;6:226e9.
severely atherosclerotic thoracic aortic aneurysm. J Endo- 29. Okita Y, Okada K, Omura A, et al. Total arch replacement
vasc Ther 2005;12:642e6. using selective antegrade cerebral perfusion as the neuro-
12. Illuminati G, Bresadola L, D’Urso A, et al. Simultaneous protection strategy. Ann Cardiothorac Surg 2013;2:
stent grafting of the descending thoracic aorta and aortofe- 169e74.
moral bypass for "shaggy aorta" syndrome. Can J Surg 30. Coscas R, Maumias T, Capdevila C, et al. Mini-invasive
2007;50:E1e2. Published 2007 Oct 10. treatment of abdominal aortic aneurysms: current roles of
13. Tanaka S, Tanaka K, Morioka K, et al. Thrombosed abdom- endovascular, laparoscopic, and open techniques. Ann
inal aortic aneurysm associated with an extensively Vasc Surg 2014;28:123e31.
"shaggy" aorta repaired anatomically via a thoracoabdomi- 31. Toya N, Baba T, Kanaoka Y, et al. Embolic complications af-
nal approach with supraceliac aortic clamping: report of a ter endovascular repair of abdominal aortic aneurysms. Surg
case. Surg Today 2010;40:866e70. Today 2014;44:1893e9.
14. Okada K, Omura A, Kano H, et al. Recent advancements of 32. Nemoto M, Hoshina K, Takayama T, et al. Statins reduce
total aortic arch replacement. J Thorac Cardiovasc Surg extensive aortic atheromas in patients with abdominal aortic
2012;144:139e45. aneurysms. Ann Vasc Dis 2013;6:711e7.
15. Hori D, Yuri K, Noguchi K, et al. Case report: successful 33. Patel SD, Constantinou J, Hamilton H, et al. Editor’s choice
endovascular treatment of saccular thoracic aneurysm e a shaggy aorta is associated with mesenteric embolisation
with "shaggy aorta. Ann Thorac Cardiovasc Surg 2012;18: in patients undergoing fenestrated endografts to treat para-
536e9. visceral aortic aneurysms. Eur J Vasc Endovasc Surg
16. Hoshina K, Kaneko M, Hosaka A, et al. Lessons learned from 2014;47:374e9.
a case of abdominal aortic aneurysm accompanied by unsta- 34. Takahashi S, Orihashi K, Imai K, et al. Transintercostal-
ble coagulopathy. Case Rep Vasc Med 2012;2012:265860. evoked spinal cord potential in thoracic aortic surgery.
17. Igarashi T, Takase S, Satokawa H, et al. Open-stent grafting Ann Vasc Surg 2014;28:1775e81.
with a GORE TAG thoracic endoprosthesis for an extended 35. Murakami T, Kawatani Y, Nakamura Y, et al. Transapical
severely atheromatous thoracic aortic aneurysm: report of endovascular aortic aneurysm repair in a patient with
a case. Surg Today 2013;43:103e5. shaggy aorta syndrome. Ann Vasc Surg 2015;29:838.e7e9.
18. Okita Y, Okada K, Omura A, et al. Total arch replacement 36. Sawazaki M, Tomari S, Zaikokuji K, et al. An improved
using antegrade cerebral perfusion. J Thorac Cardiovasc isolation technique for shaggy brachiocephalic artery and
Surg 2013;145(3 Suppl):S63e71. aortic arch replacement. Gen Thorac Cardiovasc Surg
19. Jinno T, Sangawa K. Thoracoabdominal aortic aneurysm 2015;63:533e5.
repair with preservation of the diaphragm and intercostal 37. Morimoto K, Matsuda H, Fukuda T, et al. Hybrid repair of
muscles. Kyobu Geka 2013;66:125e8. proximal subclavian artery aneurysm. Ann Vasc Dis
20. Fukuda I, Daitoku K, Minakawa M, et al. Shaggy and calci- 2015;8:87e92.
fied aorta: surgical implications. Gen Thorac Cardiovasc Surg 38. Baba T, Ohki T, Kanaoka Y, et al. Clinical outcomes of left
2013;61:301e13. subclavian artery coverage on morbidity and mortality dur-
21. Takagi Y, Ando M, Akita K, et al. Arch replacement using ing thoracic endovascular aortic repair for distal arch aneu-
antegrade selective cerebral perfusion for shaggy aorta. rysms. World J Surg 2015;39:2812e22.
Asian Cardiovasc Thorac Ann 2013;21:31e6. 39. Kanaoka Y, Ohki T, Maeda K, et al. Multivariate analysis of
22. Leone JL. Aorta "espiculoanfractuosa" y razonamiento clı́n- risk factors of cerebral infarction in 439 patients undergoing
ico ["Shaggy" aorta and clinical reasoning]. Medicina (B thoracic endovascular aneurysm repair. Medicine (Balti-
Aires) 2013;73:191e2. more) 2016;95:e3335.
23. De Feo LG, Suarez JP, Landi P. Mas sobre aorta espı́culo 40. Kozlov BN, Panfilov DS, Saushkin VV, et al. Vozmozhnosti
anfractuosa [More on "shaggy" aorta]. Medicina (B Aires) gibridnogo stent-grafta «E-vita open plus» v khirurgii
2013;73:192e3. grudnoĭ aorty [Possibilities of "E-vita open plus" hybrid stent
24. Hoshina K, Nemoto M, Hashimoto T, et al. Study design of graft in surgery of thoracic aorta]. Angiol Sosud Khir
procedure study. A randomized comparison of the dose- 2016;22:66e73.
dependent effects of pitavastatin in patients with abdominal 41. Kwon H, Han Y, Noh M, et al. Impact of shaggy aorta in pa-
aortic aneurysm with massive aortic atheroma: prevention tients with abdominal aortic aneurysm following open or
of cholesterol embolization during endovascular and open endovascular aneurysm repair. Eur J Vasc Endovasc Surg
aneurysm repair with pitavastatin (procedure) study. Ann 2016;52:613e9.
Vasc Dis 2013;6:62e6. 42. Lindblad B. Commentary on ’impact of shaggy aorta in pa-
25. Igarashi T, Takase S, Satokawa H, et al. Thoracic endovascu- tients with abdominal aortic aneurysm following open or
lar aortic repair with visceral arteries intermittent clamp endovascular aneurysm repair’. Eur J Vasc Endovasc Surg
technique for descending thoracic aortic aneurysm with 2016;52:620.
shaggy aorta. Ann Vasc Surg 2013;27:974.e11e4. 43. Toya N, Ohki T, Momokawa Y, et al. Risk factors for early
26. Wada T, Anai H, Shuto T, et al. A new method for protection renal dysfunction following endovascular aortic aneurysm
from shower embolism during TEVAR on a shaggy aorta. repair and its effect on the postoperative outcome. Surg
Gen Thorac Cardiovasc Surg 2015;63:105e8. Today 2016;46:1362e9.
27. Wada H, Nishimura M, Matsumura H, et al. Risk factors in 44. Takano T, Takase S, Kagoshima A, et al. Prevention of
the treatment of abdominal aortic aneurysms in the endo- embolization in concomitant endovascular therapy for
vascular ERA. Ann Thorac Cardiovasc Surg 2014;20: thoracic and abdominal aneurysms with severely atheroma-
299e303. tous aorta. Ann Vasc Dis 2016;9:345e8.
28. Uchida N, Katayama K, Takahashi S, et al. Modified arch- 45. Imasaka KI, Tayama E, Tomita Y. The impact of carotid or
first technique performed on a beating heart for an arch intracranial atherosclerosis on perioperative stroke in
14 Serra et al. Annals of Vascular Surgery

patients undergoing open aortic arch surgery. J Thorac Car- ahead of print, 2019 Dec 26]. Eur J Vasc Endovasc Surg
diovasc Surg 2017;153:1045e53. 2019;S1078-5884:32628-0.
46. Acosta S, K€arkk€ainen J. Causes and consequences of mesen- 63. Goto T, Fukuda I, Konno Y, et al. Clinical evaluation of a
teric embolization after endovascular aorto-iliac interven- new dispersive aortic cannula [published online ahead of
tion - a nested case control study. Anaesthesiol Intensive print, 2020 May 27]. Perfusion 2020. https://doi.org/10.
Ther 2017;49:122e9. 1177/0267659120923879. 267659120923879.
47. Komatsu S, Takahashi S, Toyama Y, et al. Exploring inside a 64. Murakami T, Sada R, Takahashi Y, et al. Arterial inflow line
shaggy aorta using non-obstructive angioscopy. BMJ Case equipped with a side arm for circulatory support and cath-
Rep 2017;2017. bcr2017219449. Published 2017 May 22. eter insertion during transcatheter aortic valve implantation
48. Okada K. Brain protection for shaggy aorta during aortic for limited vascular access. Gen Thorac Cardiovasc Surg
arch surgery. Kyobu Geka 2017;70:571e7. 2019;67:328e31.
49. Hiraoka T, Komiya T, Tsuneyoshi H, et al. Risk factors for 65. Ikeda S, Hagihara M, Kitagawa A, et al. Renal dysfunction
spinal cord ischaemia after thoracic endovascular aortic after abdominal or thoracic endovascular aortic aneurysm
repair. Interact Cardiovasc Thorac Surg 2018;27:54e9. repair: incidence and risk factors. Jpn J Radiol 2017;35:
50. Murono K, Kawai K, Hata K, et al. A case of anastomotic ste- 562e7.
nosis of the small intestine caused by cholesterol crystal em- 66. Uchida N. How to prevent spinal cord injury during endo-
bolism. Surg Case Rep 2018;4:29. Published 2018 Apr 4. vascular repair of thoracic aortic disease. Gen Thorac Cardi-
51. Kasama K, Uchida K, Karube N, et al. Impact of isolated ce- ovasc Surg 2014;62:391e7.
rebral perfusion technique for aortic arch aneurysm repair 67. Adachi K, Yamaguchi A, Yuri K, et al. Brain protection dur-
in elderly patients. Ann Thorac Surg 2019;107:533e8. ing operation for aortic. Arch Aneurysms 2013;66:952e7.
52. Yu Z, Minakawa M, Kondo N, et al. Total arch replacement 68. Ho AC, Hong CL, Yang MW, et al. Stroke after intraaortic
for aortic arch aneurysm with coexisting middle aortic syn- balloon counterpulsation associated with mobile atheroma
drome. Int J Surg Case Rep 2019;54:79e82. in thoracic aorta diagnosed using transesophageal echocar-
53. Ryomoto M, Tanaka H, Mitsuno M, et al. A novel approach diography. Chang Gung Med J 2002;25:612e6.
to prevent perioperative stroke in patients undergoing 69. Tingleff J, Egeblad H, Gøtzsche CO, et al. Perivalvular cav-
debranching thoracic endovascular aortic repair with a ities in endocarditis: abscesses versus pseudoaneurysms? A
mini-cardiopulmonary bypass support. Ann Vasc Surg transesophageal Doppler echocardiographic study in 118 pa-
2019;59:143e9. tients with endocarditis. Am Heart J 1995;130:93e100.
54. Panfilov DS, Kozlov BN, Saushkin VV, et al. Hybrid treat- 70. Pedersen JF, Berning J, Haunsø S. Single and multiple beam
ment of the ascending aortic aneurysm and "shaggy aorta" echocardiography in aortic valve endocarditis. Report of
Syndrome. Kardiologiia 2019;59(5S):65e8. three cases. Acta Med Scand 1978;204:315e9.
55. Mori K, Wada T, Shuto T, et al. Postoperative paraplegia af- 71. Joseph G, Pillai R, Shukla V, et al. Cerebral protection using
ter transapical transcatheter aortic valve implantation. J percutaneous normothermic bilateral antegrade cerebral
Cardiol Cases 2019;20:23e6. Published 2019 Mar 28. perfusion during total arch TEVAR in a patient with shaggy
56. Nomura Y, Kawashima M, Nagao K, et al. Preventing brain aorta [published online ahead of print, 2020 Apr 22]. J
embolism by using a modified isolation technique in trans- Endovasc Ther 2020. https://doi.org/10.1177/1526602
catheter aortic valve implantation for a patient with shaggy 820915940. 1526602820915940.
and porcelain aorta. J Cardiol Cases 2019;20:65e8. Pub- 72. Oishi Y, Kimura S, Sonoda H, et al. Single-stage hybrid arch
lished 2019 May 1. repair for patients with shaggy aorta. Aorta (Stamford)
57. Yokawa K, Ikeno Y, Henmi S, et al. Impact of shaggy aorta 2019;7:176e8.
on outcomes of open thoracoabdominal aortic aneurysm 73. Agarwal PP, Chughtai A, Matzinger FR, et al. Multidetector
repair [published online ahead of print, 2019 Sep 5]. J CTof thoracic aortic aneurysms. Radiographics 2009;29:
Thorac Cardiovasc Surg 2019;S0022-5223:31704-0. 537e52.
58. Kouchoukos NT. Commentary: beware the shaggy aorta 74. Quint LE, Francis IR, Williams DM, et al. Evaluation of
during thoracoabdominal aortic aneurysm repair! [pub- thoracic aortic disease with the use of helical CT and multi-
lished online ahead of print, 2019 Sep 19]. J Thorac Cardio- planar reconstructions: comparison with surgical findings.
vasc Surg 2019;S0022-5223:31849-5. Radiology 1996;201:37e41.
59. Asai T. Commentary: shaggy aorta in thoracoabdominal 75. Barker AJ, Markl M, Burk J, et al. Bicuspid aortic valve is
aortic aneurysm repair, an insidiously growing threat [pub- associated with altered wall shear stress in the ascending
lished online ahead of print, 2019 Sep 19]. J Thorac Cardio- aorta. Circ Cardiovasc Imaging 2012;5:457e66.
vasc Surg 2019;S0022-5223:31848-3. 76. Ribakove GH, Katz ES, Galloway AC, et al. Surgical implica-
60. Safaya A, Bhuta K, Rajdeo H. Shaggy aorta syndrome: recur- tions of transesophageal echocardiography to grade the
rent mesenteric embolus. Oxf Med Case Rep 2019;2019: atheromatous aortic arch. Ann Thorac Surg 1992;53:
490e1. Published 2019 Dec 9. 758e63.
61. Lepidi S, D’Oria M. The relationship between shaggy aorta 77. Montgomery DH, Ververis JJ, McGorisk G, et al. Natural his-
and embolic complications after thoracic endovascular tory of severe atheromatous disease of the thoracic aorta: a
aneurysm repair: can we smooth the rough covering? [pub- transesophageal echocardiographic study. J Am Coll Cardiol
lished online ahead of print, 2019 Dec 21]. Eur J Vasc Endo- 1996;27:95e101.
vasc Surg 2019;S1078-5884:32542-0. 78. Riambau V, B€ ockler D, Brunkwall J, et al. Editor’s choice e
62. Maeda K, Ohki T, Kanaoka Y, et al. A novel shaggy aorta management of descending thoracic aorta diseases: clinical
scoring system to predict embolic complications following Practice guidelines of the European Society for Vascular Sur-
thoracic endovascular aneurysm repair [published online gery (ESVS). Eur J Vasc Endovasc Surg 2017;53:4e52.