Journal of Cardiothoracic and Vascular Anesthesia 31 (2017) 1735–1739

Contents lists available at ScienceDirect

journal homepage: www.jcvaonline.com

E-Challenge

Acute Aortic Syndrome – More in the Spectrum

Eleonora Avenatti, MDn,1, Mark D. Iafrati, MD†, Visal Patel, MD‡,
Stephen H. Little, MDn, Natesa G. Pandian, MD§,
Stefan A. Ianchulev, MD‡
n
Department of Cardiology, DeBakey Heart and Vascular Center, Houston Methodist Hospital, Houston, TX
†
Department of Vascular Surgery, Tufts Medical Center, Boston, MA
‡
Department of Anesthesiology and Perioperative Medicine, Tufts Medical Center, Boston, MA
§
Department of Cardiovascular Imaging and Hemodynamic Laboratory, Tufts Medical Center, Boston, MA

A 68-YEAR-OLD MAN presented to an outside hospital
with complaints of fever and nonspecific “influenza-like
symptoms” over the period of a week, with worsening short-
ness of breath and severe upper back pain. Blood samples
were drawn and sent for culture. A computerized tomography
(CT) scan of the chest performed in the emergency department
raised suspicion of aortic dissection, and the patient was
transferred to the authors’ center. On initial examination he
appeared alert and awake. His blood pressure was 130/40
mmHg, heart rate 98 beats/min, respiratory rate of 18/minute,
and oxygen saturation was 93% on room air. On physical
examination, symmetric peripheral pulses were appreciated,
and no cardiac murmur was detected. Medical history was
relevant for alcoholism, hypertension, diabetes, gout, hyperlip-
idemia, and severe peripheral artery disease. The initial CT
scans from the outside hospital were considered non-diagnostic;
the cardiothoracic surgery team consulted anesthesia for evalua-
tion with transesophageal echocardiography (TEE) in the
operating room (Fig 1 and Video 1).
Challenge: What is your interpretation of the echocardio-
graphic findings, and how should this patient be managed?
TEE revealed a properly functioning aortic valve. Aortic
walls appeared to have an increased echogenicity as well as
increased thickness. The thickening looked homogenous and
1
Address reprint requests to Eleonora Avenatti, MD, Cardiovascular
Imaging Section, Department of Cardiology, DeBakey Heart & Vascular
Center, Houston Methodist Hospital, 6565 Fannin Street, Lab F583, Houston,
TX 77030.
E-mail address: eavenatti@houstonmethodist.org (E. Avenatti).
showed a concentric pattern, the latter finding being more
evident in the ascending aorta and in the short-axis view.
There was no mobile dissection flap and no flow within the
thickened walls was detected with the color Doppler. The TEE
findings were suggestive of intramural hematoma (IMH). A
follow-up CT scan confirmed these findings and noted a
“thickened aortic wall, consistent with an IMH, measuring
up to 5.7 cm in diameter and longitudinally extending from the
sinotubular junction to the aortic arch. An “ulcer-like” projec-
tion extending for up to 14 mm into the hematoma was seen,
with a base of approximately 12 mm” (Fig 2).
IMH is part of the acute aortic syndrome, along with classic
aortic dissection and penetrating ulcer.1 These different entities
are grouped together as they all share a similar clinical
presentation of chest pain and have the potential for cata-
strophic evolution into aortic rupture requiring emergent
treatment.
Clinical suspicion needs to be high in this clinical setting,
and diagnosis heavily relies on imaging. TEE, CT, and cardiac
magnetic resonance all have shown excellent performances in
this setting, with sensitivity and specificity reportedly as high
as 100%, 98%, and 98% and 98%, 95%, and 98%, respec-
tively.2 Rather than globally recommending 1 imaging modality
over another in acute aortic syndromes, it is important to
understand their relative strengths. Specific advantages of TEE
are its “bedside” availability and capability of performing a
global functional evaluation, both potentially crucial factors in
this specific clinical setting. The major limitation of TEE is the
difficulty visualizing the distal ascending aorta and proximal
aortic arch. CT scanning, on the other hand, can provide a

http://dx.doi.org/10.1053/j.jvca.2017.04.036
1053-0770/& 2017 Elsevier Inc. All rights reserved.
1736 E. Avenatti et al. / Journal of Cardiothoracic and Vascular Anesthesia 31 (2017) 1735–1739

Fig 1. Transesophageal echocardiogram at the time of admission. (A) Mid-esophageal aortic valve long-axis view demonstrating a properly functioning aortic
valve. (B) Mid-esophageal long-axis view of the ascending aorta. (C) Mid-esophageal short-axis view of the ascending aorta with color-Doppler evaluation.

complete evaluation of the entire aorta, and usually is available
widely in different healthcare settings. Exposure to radiation and
intravenous iodinated contrast has become less clinically
relevant with technologic innovations such as multidetector
CT scanning and electrocardiogram gating.
Differentiating IMH from the other acute aortic syndromes
is important. From an echocardiographic standpoint, the
identification of the dissection flap is specific, albeit not
sensitive, for aortic dissection. The absence of a flap and/or
flow in the false lumen is considered a key feature of
intramural hematoma.1 Even though the severe atherosclerosis
of the patient made penetrating ulcer an important differential,
the findings in this patient were overall more consistent with
IMH. The location and appearance of the lesion was indeed
not typical for a penetrating ulcer. Penetrating ulcers have been
reported to be more common in the mid and distal portions of
the descending aorta3 and typically present with a ragged
profile of the lumen. The concentric thickening of aortic walls
and the preserved shape of the aortic lumen, on the other hand,
represent specific imaging features of IMH, and can be
appreciated on both the TEE and the CT scan. Ulceration
indenting the otherwise smooth aortic lumen profile can be
present, as was in the authors’ patient.
A little more than a third of IMHs will reabsorb sponta-
neously, while the most common long-term evolution is
formation of aneurysm and pseudoaneurysm. Up to 16% of
cases of IMH have been reported to progress to overt
dissection.4 Scant data are available on risk factors for such
a progression, and most of them are inferred from evolution of
IMH originating from and involving only the descending
aorta.1,4 Current guidelines indicate emergency surgery as
the preferred approach for IMH involving the ascending aorta,

Fig 2. Axial views of the ascending aorta from the chest CT on admission (helical CT with contrast, prospectively gated) with thickened ascending aortic walls and
(arrow) ulcer-like projection within the IMH.
 E. Avenatti et al. / Journal of Cardiothoracic and Vascular Anesthesia 31 (2017) 1735–1739
Fig 3. Spinal MRI. (A) Whole spine with (red circle) edema and enhancement within the endplates and intervertebral discs at the thoracic level indicating epidural
abscess. (B) Detail of thoracic vertebrae: T2/STIR signal hyperintensity and enhancement within the T7 and T8 vertebral bodies, indicating osteomyelitis.
and medical therapy for lesions involving the descending aorta
only, mirroring the recommendation for type-I and type-II
aortic dissection, respectively. Nevertheless, in the case of
ascending IMH in patients with significant comorbidities,
initial medical treatment with a careful “watchful waiting”
strategy is considered acceptable, with aggressive management
of blood pressure and optimization of pain control.1 In this
patient, considering the clinical comorbidities, medical man-
agement was chosen. Aggressive control of blood pressure and
pain, together with broad-spectrum antibiotic coverage, were
instituted.
Clinical Case – Part 2
On hospitalization day 4, the patient experienced fever
(38.61C); blood and urine samples were sent again for culture,
which eventually returned negative. Complaints of increasing
back pain prompted evaluation of the spine with a targeted
MRI. Multiple epidural abscesses spanning from T8 to T10
were identified with multifocal thoracic spine osteomyelitis
(Fig 3). Physical examination was negative for spinal cord
injury or compression. Progressive shortness of breath
prompted a follow-up transthoracic echocardiogram that
demonstrated a new pericardial effusion (Fig 4, Video 2).
The patient was hemodynamically stable. The pericardial
effusion was partially drained under echocardiographic gui-
dance, with close monitoring of the patient’s hemodynamic
condition. Drainage yielded 300 mL of serosanguinous fluid.
1737
A repeat chest CT scan was performed (Fig 5). Urine and
blood cultures from the admission day revealed growth of
gram-negatives rods that were identified as Salmonella enter-
ica spp, serovar Dublin, with multiple drug resistance profiles.
Challenge: What differentials should be considered in the
evolving scenario? Should the management change?
The follow-up transthoracic echocardiogram demonstrated a
new moderate circumferential pericardial effusion, with no
obvious signs of significant hemodynamic impact—absence of
right ventricular free wall diastolic collapse, no septal bounce,
no respiratory variation detected on pulsed-Doppler evalua-
tion. The pericardiocentesis had a diagnostic purpose more
than a therapeutic one. The serosanguinous effusion was
considered an obvious sign of disease progression. The chest
CT confirmed the evolving situation: the previously identified
ascending aortic thoracic IMH and the ulcer appeared larger.
The official report showed increased dimension (approxi-
mately 14  20  24 mm on this second scan) and increased
density of the IMH, suggesting presence of active bleeding
into the aortic wall. The inferior margin of the lesion remained
distal to the sinotubular junction, without involving the aortic
sinuses. In the broader clinical context of bacteremia with
osteomyelitis, aortitis with mycotic aneurysm became a critical
part of the differential diagnosis.
Given the increase in size and the likely mycotic origin, the
aneurysm was felt to be acutely life-threatening and the
decision was made after multidisciplinary discussion involving
infectious disease, cardiothoracic, vascular surgery, and
anesthesia to pursue a surgical approach. Endovascular stent
1738 E. Avenatti et al. / Journal of Cardiothoracic and Vascular Anesthesia 31 (2017) 1735–1739

Fig 4. Follow-up transthoracic echocardiogram. Right, standard apical 4-chamber view. Left, standard subcostal view.

placement was regarded as the best viable option. With
fluoroscopy guidance and with the aid of TEE for monitoring
for complications, endovascular aortic repair (EVAR) was
performed, with the placement of an ascending aortic endo-
graft via femoral access (Fig 6).
The postoperative course was complicated by sepsis,
volume overload, and delirium. The patient was discharged
eventually to a physical rehabilitation facility on postoperative
day 15 on long-term antibiotics. On follow-up, the patient was
doing well 12 months post-procedure.
Discussion
Infective aortitis is a rare cause of aortic aneurysm (0.6%-
1.3%), encompassing far higher morbidity and mortality
compared with uncomplicated IMH.5 In early series, mortality
was close to 100% in patients maintained on medical therapy
alone.6 Surgical approach with resection of the damaged tissue
and subsequent revascularization, either with extra-anatomic
bypass or direct reconstruction, long has been the standard of
treatment. This approach, however, is of limited applicability,
considering the high prevalence of significant comorbidities in
the treated patients, a factor that explains an intrinsic mortality
still as high as 40%.7 Moreover, aortitis caused by Salmonella
has a specific higher progression risk, showing a subacute
presentation and subsequent fast progression to a mycotic
aneurysm, with a greater tendency toward early rupture
compared with gram-positive infections.5,8
Cardiovascular involvement in the setting of Salmonella
species infection is quite rare compared with the global burden
of uncomplicated salmonellosis. Indeed, fewer than 5% of
infected patients develop bacteremia,6,9 and of those only
about 10% will show cardiovascular involvement6,10 in the
form of endocarditis, device-associated infection, or aortitis.
Nevertheless, up to one-third of aortitis can be related to
Salmonella spp, and specific risk factors have been identified.
Those include both host-related factors, such as immunosup-
pression, age 4 50 years, atherosclerotic disease,6,11,12 and
pathogen-specific features. Salmonella is characterized by a
greater tendency to attach to and to seed into damaged
endothelium,13 possibly due to its specific virulence factors,
namely fimbriae and flagella, and their interaction with altered
endothelial structures.
In the present case, the patient was older than 60 and had very
advanced peripheral vascular disease, an optimal medium for
seeding of the infection. Unfortunately, the primary source of
infection could not be identified. The patient had a negative HIV
test, but his poorly controlled diabetes mellitus could have been

Fig 5. Axial views of the ascending aorta from the follow-up chest CT (helical CT with contrast, prospectively gated). Arrow identifies the enlarged ulcer-like
projection within the ascending aortic walls.
 E. Avenatti et al. / Journal of Cardiothoracic and Vascular Anesthesia 31 (2017) 1735–1739
Fig 6. Endovascular repair of the mycotic aortic aneurysm; fluoroscopic view
of the ascending aortic graft just deployed.
responsible for systemic immunosuppression and accounted for his
higher susceptibility to an infection as described previously.13 He
was considered from the beginning a high risk for traditional
surgical intervention, with an STS score greater than 10% for
surgical mortality and significant calcification and severe atheroma
of the aortic arch, making a good landing zone for a traditional
aortic graft technically difficult. The initial working diagnosis of
IMH was compatible with a more conservative approach. How-
ever, the evolving clinical scenario with the identification of
multiple active foci of infection, the positive cultures for Salmo-
nella spp, the appearance of the pericardial effusion, and the
worsening of the CT scan findings despite the active antibiotic
treatment prompted a re-evaluation. Non-standard surgical
approaches in the treatment of mycotic aneurysm have been used,
building on the experience in degenerative aortic aneurysm. EVAR
in the more proximal aortic section, such as in the authors’ patient,
is a recent achievement in the interventional field.14 EVAR has
been used in the setting of infectious aortic aneurysm15 in the last
2 decades, with reported good outcomes. In a 2007 review, Kan et
al reported a 30-day mortality of 10.4%, with survival rates highly
dependent on the persistence of the infection (94% v 40% for
persistently infected patients).16 A recent multicenter European
study reviewed the experience on 123 patients and 130 mycotic
aneurysms, showing feasibility of endovascular aortic repair and its
value as a durable treatment option, with short-term survival as
high as 91%. Interestingly, in those cases, Salmonella-positive
patients showed a good long-term outcome, after facing a high
perioperative mortality, especially in the setting of active infection
and overt aortic rupture.17 In the authors’ patient, neither of these
factors was present, as the patient underwent the procedure after a
period of broad-spectrum antibiotic therapy, with negative blood
cultures pre-intervention and in stable hemodynamic conditions.
Conclusion
Infective aortitis and mycotic aneurysm, although rare, need
to be included in the differential diagnosis of acute aortic
syndromes, considering their high mortality burden. Diagnosis
1739
of such conditions requires high suspicion index and their
management heavily relies on multidisciplinary and patient-
tailored approach. Ability to turn to a multimodality imaging
approach and implement interdisciplinary collaboration,
coupled with the use of cutting-edge technologies— EVAR
in the setting of mycotic aneurysm is a very recent addition to
the interventionalists’ toolbox—may grant successful manage-
ment of this potential catastrophic condition.
Appendix A. Supplementary material
Supplementary data are available in the online version of
this article at http://dx.doi.org/10.1053/j.jvca.2017.04.036
References
1 Goldstein SA, Evangelista A, Abbara S, et al. Multimodality imaging of
diseases of the thoracic aorta in adult: From the American Society of
Echocardiography and the European Association of Cardiovascular Ima-
ging: Endorsed by the Society of Cardiovascular Computed Tomography
and Society of Cardiovascular Magnetic Resonance. J Am Soc Echocar-
diogr 2015;28:119–82.
2 Shiga T, Wajima Z, Apfel CC, et al. Diagnostic accuracy of transesopha-
geal echocardiography, helical computed tomography, and magnetic
resonance imaging for suspected thoracic aortic dissection: Systematic
review and meta-analysis. Arch Intern Med 2006;166:1350–6.
3 Coady MA, Rizzo JA, Hammond GL, et al. Penetrating ulcer of the
thoracic aorta: What is it? How do we recognize it? How do we manage it?
J Vasc Surg 1998;27:1006–16.
4 Evangelista A, Mukherjee D, Mehta RH, et al. Acute intramural hematoma
of the aorta: A mystery in evolution. Circulation 2005;111:1063–70.
5 Foote EA, Postier RG, Greenfield RA, et al. Infectious aortitis. Curr Treat
Options Cardiovasc Med 2005;7:89–97.
6 Cohen PS, O'Brien TF, Schoenbaum SC, et al. The risk of endothelial infection
in adults with salmonella bacteremia. Ann Intern Med 1978;89:931–2.
7 Kyriakides C, Kan Y, Kerle M, et al. 11-year experience with anatomical
and extra-anatomical repair of mycotic aortic aneurysms. Eur J Vasc
Endovasc Surg 2004;27:585–9.
8 Jarrett F, Darling RC, Mundth ED, et al. Experience with infected
aneurysms of the abdominal aorta. Arch Surg 1975;110:1281–6.
9 Hohmann EL. Nontyphoidal salmonellosis. Clin Infect Dis 2001;32:263–9.
10 Fernandez Guerrero ML, Aguado JM, Arribas A, et al. The spectrum of
cardiovascular infections due to salmonella enterica. Medicine (Baltimore)
2004;83:123–38.
11 Benenson S, Raveh D, Schlesinger Y, et al. The risk of vascular infection
in adult patients with nontyphi salmonella bacteremia. Am J Med
2001;110:60–3.
12 Hsu RB, Tsay YG, Chen RJ, et al. Risk factors for primary bacteremia and
endovascular infection in patients without acquired immunodeficiency syn-
drome who have nontyphoid salmonellosis. Clin Infect Dis 2003;36:829–34.
13 Soravia-Dunand VA, Loo VG, Salit IE. Aortitis due to salmonella: Report
of 10 cases and comprehensive review of the literature. Clin Infect Dis
1999;29:862–8.
14 Semba CP, Sakai T, Slonim SM, et al. Mycotic aneurysms of the thoracic
aorta: Repair with use of endovascular stent-grafts. J Vasc Interv Radiol
1998;9:33–40.
15 Kobuta H. Endovascular stent graft repair of the ascending aorta – final frontier
in the endovascular treatment of the aorta. J Thorac Dis 2016;8:E1358–60.
16 Kan CD, Lee HL, Yang YJ. Outcome after endovascular stent graft
treatment for mycotic aortic aneurysm: A systematic review. J Vasc Surg
2007;46:906–12.
17 Sörelius K, Mani K, Björck M, et al. Endovascular treatment of mycotic
aortic aneurysms: A European multicenter study. Circulation 2014;130:
2136–42.