ADULT  Original Submission

Incidence and Prevalence of Thoracic Aortic

Aneurysms: A Systematic Review and
Meta-analysis of Population-Based Studies
Ryan Gouveia e Melo, MD,*,†,‡ Gonçalo Silva Duarte, MD,†,§,# Alice Lopes, MD,*,‡
Mariana Alves, MD,†,§,#,¶ Daniel Caldeira, MD, PhD, FESC,†,‡,§,#,**
Ruy Fernandes e Fernandes, MD, FEBVS,*,†,‡ and Luís Mendes Pedro, MD, PhD, FEBVS*,†,‡

Thoracic aortic aneurysms (TAA) may grow asymptomatically until they rup-
ture, with a mortality over 90%. The true incidence and prevalence of this
condition is uncertain and epidemiologic data is scarce, understudied and
dispersed. Therefore, we aimed to conduct a systematic review and meta-
analysis of the incidence and prevalence of TAAs in population-based stud-
ies. We searched MEDLINE, EMBASE and CENTRAL from inception to
October 2020 for all population-based studies reporting on incidence and/or
prevalence of TAAs. Data were pooled using a random effects model. The
main outcome was the overall available worldwide incidence and prevalence
of TAAs. The secondary outcomes were to evaluate the incidence of rup-
Overall incidence of thoracic aortic aneurysms.
tured TAAs, differences in the location of these aneurysms (either ascending,
arch or descending aorta) and differences in prevalence/incidence across Central Message
different study designs. Twenty-two studies were included in the review and
A worldwide pooled incidence and prevalence
meta-analysis. The pooled incidence and prevalence of TAAs was 5.3 per
of thoracic aortic aneurysms and incidence of
100,000 individuals/year (95% confidence interval [CI]: 3.0; 8.3) and 0.16% ruptured thoracic aortic aneurysms was calcu-
(95% CI: 0.12; 0.20), respectively. The pooled incidence of ruptured aneur- lated in the systematic review and meta-
ysms was 1.6 per 100,000 individuals/year (95% CI: 1.3; 2.1). We found a analysis.
significant difference of the prevalence in autopsy-only studies, which was
0.76% (95% CI: 0.47; 1.13) and the prevalence of TAAs dropped down to Perspective Statement
0.07% (95% CI: 0.05;0.11) when these studies were excluded from the over- No paper has yet tried to reach a global esti-
all analysis. The current epidemiologic information provided serve as a base mate of incidence and prevalence of thoracic
for future public-health decisions. The lack of well-design population-base aortic aneurysms. We aimed to fill this gap in
studies and the limitations encountered serve as calling for future research the literature in order to provide; future authors
with the most accurate data on incidence and
in this field. prevalence to cite in their papers and informa-
Semin Thoracic Surg 34:1–16 © 2021 Elsevier Inc. All rights reserved. tion for future public health decisions, regard-
ing cost allocation and strategies for screening.

Abbreviations: AAA, Abdominal Aortic Aneurysm; Asc, Ascending Aorta; CASP, Critical Appraisal Skills Program; CENTRAL, Cochrane Central
Register of Controlled Trials; CI, Confidence Interval; CT, Computerized Tomography; Desc, Descending Aorta; IQR, Interquartile Ratio; NA, Non-
applicable; NR, Not Reported; PRISMA, Preferred Reporting Items for Systematic Reviews and Meta-Analyses; TAA, Thoracic Aortic Aneurysm
*Vascular Surgery Department, Hospital Santa Maria, Centro Hospitalar Universita
rio Lisboa Norte (CHULN), Lisboa, Portugal
y
Faculty of Medicine, University of Lisbon, Lisboa, Portugal
z
Cardiovascular Center of the University of Lisbon (CCUL), Lisboa, Portugal
x
Laboratory of Clinical Pharmacology and Therapeutics, Faculty of Medicine, University of Lisbon, Lisboa, Portugal
#
Instituto de Medicina Molecular, Faculty of Medicine, University of Lisbon, Lisboa, Portugal
{
Serviço de Medicina III, Hospital Pulido Valente (CHULN), Lisboa, Portugal
**Serviço de Cardiologia, Hospital Universita
rio de Santa Maria (CHULN), Lisboa, Portugal
Patient Consent: Not applicable  systematic review and meta-analysis of data at study level. No individual patient data were assessed or
available.
Funding: None to report.
Conflicts of Interest: None to report.
Address reprint requests to Ryan Gouveia e Melo, MD, Hospital de Santa Maria  Centro Hospitalar Universita
rio Lisboa Norte, EPE, Serviço de
Angiologia e Cirurgia Vascular, Avenida Professor Egas Moniz, 1649-028, Lisboa, Portugal. E-mail: ryan@campus.ul.pt

1043-0679/$see front matter © 2021 Elsevier Inc. All rights reserved. 1

https://doi.org/10.1053/j.semtcvs.2021.02.029
ADULT  Original Submission
Keywords: Thoracic aortic aneurysms, Prevalence,
Epidemiologic study, Meta-analysis
INTRODUCTION
Thoracic aortic aneurysms (TAA) are known to be “silent
killers.” According to their natural history, they usually grow
asymptomatically until they rupture, which may have a mortal-
ity ranging from 94% to 100%.1,2
Being located in the thorax makes the diagnosis more chal-
lenging, which is usually made incidentally on an echocardio-
gram, chest-CT or on autopsy. This is the opposite of the
better known abdominal aortic aneurysm which is easy to
screen and currently well studied with robust population-
based studies.3
On the contrary, epidemiologic data on TAA is scarce,
understudied and dispersed.4 The real prevalence of TAAs is
uncertain, with a wide variability described among papers,2,5
expressing the need for a reliable estimate of incidence and
prevalence of these aneurysms. This information is important
for the development of healthcare policies, allocation of fund-
ing and medical care.4 In addition, information regarding inci-
dence and prevalence of TAAs and the possible analysis of
factors related to them is important for clinical understanding
of the behavior the disease in order to improve care.
We sought out to fill this gap in the literature by performing
a systematic-review and meta-analysis of all population-based
studies regarding thoracic aortic aneurysms.
METHODS
The review followed and complied with the PRISMA (Pre-
ferred Reporting Items for Systematic Reviews and Meta-Analy-
ses) guidelines6 and has been registered in the PROSPERO
public database (CRD42019121857).
Eligibility Criteria
All population-based studies reporting incidence or preva-
lence of thoracic aortic aneurysms were included. Studies
reporting ruptured and/or intact aneurysms were included, as
well as autopsy and death certificate studies if the total popula-
tion was described.
There was no date, publication type or language restrictions.
Animal studies were not included.
Papers were excluded if they reported on the same popula-
tion (in order not to duplicate events), if specific and indepen-
dent information was not given regarding thoracic aortic
aneurysms or if the population considered was not commu-
nity-based (ie, studies evaluating only patients with known
aortic stenosis or other specific conditions, for example).
Information Sources and Search Method
We performed electronic searches in the following data-
bases: EMBASE, MEDLINE and Cochrane Central Register of
Controlled Trials (CENTRAL), from inception to October
2020. References were also cross-checked for potential
2 Seminars in Thoracic and Cardiovascular Surgery  Volume 34, Number 1
Incidence,
additional studies and consulted specialists for additional
potential studies. See the supplementary file for details of the
search strategy (Supplementary Data 1). The yielded results
were cross checked, and duplicates were eliminated.
Study Selection, Data Collection Process and
Synthesis
Two reviewers (RGM and AL) independently screened the
titles and abstracts that resulted from the search. Both authors
also independently reviewed the full text papers and any dis-
agreements were resolved by consulting a third partner (GD).
After the final search result, data was extracted indepen-
dently by 2 authors (RGM and AL) using a pre-designed report
form and uploaded into an Excel sheet following confirmation
of agreement of the data.
Data extracted including the following: study site/population
(country and state/city), year(s) of the study, methods of
extracting the population-based data, total population, total
number of TAA, prevalence, incidence, location of the aneu-
rysm (ascending, arch or descending aneurysms), type of aneu-
rysm (intact or ruptured) and demographics (sex, age, and risk
factors). We based the definition of TAA on each study criteria
and thoracoabdominal aneurysms were including according to
their thoracic component. There was no restriction regarding
classification of the thoracic aneurysm and post-dissection
aneurysms were included if specified that they affected the tho-
racic aorta. In autopsy studies we considered all previously
untreated patients as cases for TAA, if the patient had had treat-
ment (either open or endovascular repair) the case was
excluded. If this was not mentioned in the study, we assumed
it to be untreated TAAs.
The main outcome of interest was to estimate the overall
available worldwide incidence and prevalence of TAAs in pop-
ulation-based studies. The incidence was defined as the num-
ber of new diagnosis/reports of TAAs per 100,000 persons per
year. The prevalence was defined as number of existing cases
during the time of the study in the population.
The secondary outcomes were to evaluate the incidence of
ruptured thoracic aortic aneurysms, differences in the location
of these aneurysms (ascending, arch or descending aorta) and
differences in prevalence/incidence across different study
designs.
Statistical analysis
Quantitative analysis was performed when appropriate using
R language environment (version 3.4.1) with the “meta” pack-
age (version 4.12-0).
The results yielded by the data extraction were expressed in
counts by 100,000 individuals/year for incidence and percen-
tages (frequency) for prevalence. When available, age-

standardized incidence was used preferentially. The incidence
data was directly extracted from the paper or calculated accord-
ing to the data provided regarding number of events, popula-
tion at risk and years of the study. If total population was not
provided in the paper or by the authors, the numbers provided
in the country census was used.7 For the prevalence calculation
the total number of individuals screened was used as the
denominator.
The data was subjected to Freeman-Tukey transformation
(double arcsine transformation) in order to avoid negative
prevalence/incidence in the confidence interval, limiting the CI
among 0100%.8 A random-effects model was used to pool
the data in order to account for the heterogeneity of the
included studies such as the difference in designs.9 Statistical
heterogeneity was assessed using I2.10
Sensitivity analysis was performed regarding study designs
being based on: autopsy; death certificates and hospital admis-
sions; regarding the size of the population studied, diagnostic
method, date of publication, use of age-standardized data and
geographical area.
Risk of Bias
As there are no specific assessment tools to evaluate the risk
of bias in population-based studies, therefore, we adapted and
used the Critical Appraisal Skills Program (CASP) cohort study
checklist to asses for risk of bias, in which we categorized 11
items as having high, unclear or low risk of bias.11 Two authors
(RGM and AL) independently assessed every included paper.
The overall risk of bias for each study was divided as high or
low-risk, with high risk being those studies in which at least
two items were assessed at a high risk of bias, or more than
three items had a rating of unclear. “Small study effect”12 was
assessed qualitatively by visual inspection of inverted funnel
plot asymmetry. Leave-one-out meta-analysis was also per-
formed.
Consent
Patient consent was not applicable as the systematic review
and meta-analysis was performed with data at study level. No
individual patient data were assessed or available.
RESULTS
Included Studies
The electronic database yielded 3851 articles. After dupli-
cates were removed 3585 remained, of which after title and
abstract review resulted 54 papers that were evaluated in full
text. Of these, 22 were included in the qualitative and quantita-
tive synthesis (Fig. 1).
Out the 22 included in the review, 13 were population-
based studies reporting on both ruptured and intact TAAs,1,4,
1323
6 studies reported only on ruptured TAA2,2428 and 3
reported only on autopsy studies.5,29,30
Out of the 32 papers excluded the reasons for exclusion are
detailed in Figure 1.
Seminars in Thoracic and Cardiovascular Surgery  Volume 34, Number 1
ADULT  Original Submission
Study characteristics and demographic data
The details regarding the studies are expressed in Table 1
and the relevant data extracted in Table 2. Studies reported
data ranging from 1946 to 2016, encompassing a population
over 100 Million people and identified 104 519 TAAs. Coun-
tries analyzed included the US, Canada, Brazil, Japan, Canada,
Finland, Sweden, Holland, United Kingdom and Germany.
Data regarding incidence and prevalence were heteroge-
neous, with 6 studies only describing incidence (total popula-
tion or number of TAAs identified not presented), 8 only
describing prevalence and 8 studies presented both (study data
is reported in Tables 1 and 2 and the numerator and denomi-
nator data to derive the incidence and prevalence are detailed
in the Supplementary Data 24).
Only one article (McClure et al)16 reported on specific risk
factors. The method of diagnostic was different among studies
being autopsy or surgery in 4, only autopsy in 3, CT-Scans in
3, 9 did not specify the diagnosis method and based counts
either on admission/discharge papers (10) or on death certifi-
cates (2) (Table 1). Specific etiology of the aneurysms found
was only specified in 7 studies (Table 2).
Mean age of the studied TAA patients was only reported in
10 papers and the mean varied between 59 and 85 years
(Table 2), largely due to the specific design of the study, seeing
as for example the autopsy studies typically included older
patients. Patients with TAA were predominantly male (Table 2)
with a median frequency of 61% (min-max: 3983).
MAIN OUTCOMES
Overall Incidence and Prevalence of Thoracic Aortic
Aneurysms in Population-Based Studies Reporting on
all TAAs
For this calculation we excluded papers only reporting on
ruptured aneurysms.
The pooled calculated incidence of TAAs in the popula-
tion was 5.3 per 100,000 individuals/year (95% confidence
interval [CI]: 3.0; 8.3; I2 100%; 2,617,989,819 person-
years; 10 studies) (Fig. 2). The pooled calculated prevalence
was 0.16% (95% CI: 0.12; 0.20; I2 100%; 102,962,741
patients; 13 studies) for a median of 12 years (min-max:
329) analyzed (Fig. 3).
SECONDARY OUTCOMES
Incidence of Ruptured TAAs
The calculated incidence and prevalence of ruptured TAAs
was 1.6 per 100,000/year (95% CI: 0.9; 2.5; I2 100%;
7,454,689,936 persons-year; 12 studies) (Fig. 4). Six of the
studies included for this analysis only reported on ruptured
TAAs, the remainder reported on both ruptured and intact and
we were able to extract the precise number of ruptures in the
overall cohort.
3
ADULT  Original Submission
Figure 1. PRISMA flow-chart.
Prevalence of TAAs in Autopsy Studies
These studies were made using a population-base model
where they performed autopsies for every cause of death
(including more than 75% of the population). Aneurysms
included both ruptured and intact and would either be the
cause of death or a finding.
For these studies, the calculated prevalence of TAAs was
0.76% (95%CI: 0.47; 1.13. I2 92%; 72,172 patients; 3 studies),
respectively (Fig. 5).
Frequency of Different Location of TAAs
Only 8 studies provided details for the precise location of the
TAAs.
Out of all TAAs, 45.5% of patients had thoracic aortic aneur-
ysms which included the ascending aorta (95%CI: 28.7; 62.9.
I2 97%, 1297 patients) (Fig. 6, top), 21.3% included the aortic
arch (95% CI: 11.4; 32.4. I2 95%, 1280 patients) (Fig. 6, mid-
dle), and 34.6% included the descending thoracic aorta (95%
CI: 25.6; 44.2. I2 88%, 1297 patients) (Fig. 6, bottom).
4 Seminars in Thoracic and Cardiovascular Surgery  Volume 34, Number 1
Sensitivity Analysis
We opted to report summary estimates using a random
effects model in order to account for differences in study
designs. However, in order to analyze the impact of different
designs, we performed a sensitivity analysis regarding autopsy
study designs and studies using either death certificates or hos-
pital admissions as methods of assessment, the size of the pop-
ulation studied, diagnostic method, date of publication, use of
age-standardized data, geographical location (continent) and
risk of bias (Table 3).
In this analysis we observed a trend towards increase in the
incidence of TAA (5 vs 5.5 TAAs per 100,000/year, before and
after 2000, respectively) but a decrease in the prevalence
(0.09% vs 0.07%, before and after 2000, respectively, when
excluding autopsy-based studies). Moreover, when we ana-
lyzed only ruptured TAAs, incidence decreased with time (4.0
vs 1.0 ruptured TAAs per 100,000/year, before and after 2000,
respectively).
When analyzing only hospital admission studies with
administrative coding data the prevalence tended to be lower
Seminars in Thoracic and Cardiovascular Surgery  Volume 34, Number 1

Table 1. Study Characteristics

Author (Year) Geographic Location Year(s) How Was the Data Patients Reported Diagnostic Definition of TAA
(Country/city or County) Obtained Methodology
Ingoldy et al United Kingdom - 1974-1983 NR Only Ruptured TAAs NR "Rupture of thoracic (..)
(1986) Swansea aneurysms"
Drott et al Sweden - Gotenborg 1952-1988 NR Only Ruptured TAAs Autopsy or Surgery "Rupture of aorta above
(1992) the diaphragm"
Johansson et Sweden - Stockholm 1980 - 1989 National bureau of Only Ruptured TAAs Autopsy or Surgery "Rupture of aorta above
al (1995) Statistics and the diaphragm"
Computer Hospital
Registries
Clouse et al USA - Olmsted County, 1980-1994 Rochester Only Ruptured TAAs NR "spontaneous
(2004) Minnesota Epidemiology Project decompression of (..)
degenerative
aneurysm
extraluminally"
Abdulameer USA - all states 1999-2016 Retrospective review of Only Ruptured TAAs NR (based counts on NR
et al (2018) the Multiple Causes of mentions on death
Death records, certificate)
maintained by the U.S.
National Center for
Health Statistics
(NCHS), using the
Centers for Disease
Control and
Prevention (CDC)
Wide-ranging OnLine
Data for
Epidemiologic
Research (WONDER)
platform
Yamaguchi et Japan 2012-2015 Japanese Registry of All All TAAs ICD-10 codes NR

ADULT  Original Submission

al (2019) cardiac and vascular
DiseasesDiagnostic
Procedure
Combination (JROAD-
DPC) database
Bickerstaff et USA - Rochester, 1951-1980 Rochester All TAAs Autopsy, surgery or NR
al (1982) Minnesota Epidemiology Project roentgenography
Samy et al United Kingdom - 1980-1989 Registrar General of All TAAs NR (based counts on NR
(1993) Glasgow Scotland and Scottish diagnosis described in
Mortality Record Form the forms)
1
(continued on next page)
5
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ADULT  Original Submission

Table 1. (continued )
Author (Year) Geographic Location Year(s) How Was the Data Patients Reported Diagnostic Definition of TAA
(Country/city or County) Obtained Methodology
Lilienfeld et al USA - Minneapolis, St. 1979-1984 Collection of all All TAAs NR (based counts on NR
(1993) Paul metropolitan area discharge information diagnosis in the
from acute care discharge or
hospitals (with the admission papers)
exception of
discharges from the
Veterans Affairs
Medical Center) in the
seven-county
Minneapolis- St. Paul
metropolitan area
Clouse et al USA - Olmsted County, 1980-1994 Rochester All TAAs Radiographic studies, “Focal aortic dilation
(1998) Minnesota Epidemiology Project Surgery or Autopsies (1.5 times larger than
normal local aorta)
was identified and
Seminars in Thoracic and Cardiovascular Surgery  Volume 34, Number 1

confirmed by
radiographic studies,
operation, or autopsy”
Itani et al Japan - Chiba 1995-2002 Screening project for All TAAs CT-Scan “Discontinuous and
(2002) lung cancer and limited aortic dilatation
tuberculosis in which a diameter of
more than 50mm in
the ascending aorta,
40 mm in the
descending aorta.”
Olsson et al Sweden - nationwide 1987-2002 National Bureau of All TAAs NR (based counts on “Dilatation of the aorta
(2006) Statistics the Hospitals including all wall
Discharge Registry) layers, with a diameter
exceeding 5 cm or
50% increase;
thoracic aortic rupture
was defined as
contained or free
extravasation of blood
from the aorta to
tissues or cavities”
Santo et al ~o Paulo
Brazil - Sa 1985-2009 Death Certificates All TAAs NR (based counts on NR
(2012) (Sistema Estadual de mention in death
Analise de Dados certificate)
(SEADE)), analyzing
primary and
secondary diagnosis
(continued on next page)
Seminars in Thoracic and Cardiovascular Surgery  Volume 34, Number 1

Table 1. (continued )
Author (Year) Geographic Location Year(s) How Was the Data Patients Reported Diagnostic Definition of TAA
(Country/city or County) Obtained Methodology
Von Allmen et United Kingdom- 1999-2010 Hospital Episodes All TAAs in people over 50 NR (based counts on NR
al (2013) England and Wales Statistics (HES) diagnosis in the
(England) and Health discharge or
Solutions Wales admission papers)
PEDW Statistics
(Wales)
Kalsch et al Bochum, Essen, and 2000-2003 Random sample of the All TAAs in people 45-74 Electron beam Aortic diameter >5cm
(2013) Mu€lheim/Ruhr, Heinz Nixdorf Recall computer tomography
Germany study
McClure et al Canada - Ontario 2002-2014 Institute for Clinical All TAAs NR (based counts on NR
(2018) Evaluative Sciences diagnosis in the
(patient-level data) discharge or
admission papers)
Geisbuch et al Germany 2005-2014 Extracted from All TAAs NR (based counts on NR
(2918) diagnostic related diagnosis in the
group (DRG) statistics discharge or
provided by the admission papers)
research data centers
of the German Federal
Statistical Office
Bons et al Holland - Rotterdam 2003-2006 Random sample from All TAAs in people over 55 CT-Scan Ascending Aorta larger
(2019) the Rotterdam Study, than 5mm and
a prospective descending aorta
population-based larger than that 45mm
cohort study (older
than 55)
Lodewyks et Manitoba, Canada 1994-2016 Manitoba Center for All TAAs patients >18 ICD-9 and ICD-10 NR
al, 2020 Health Policy and hospital codes
Manitoba Thoracic

ADULT  Original Submission

Aortic Disease clinical
database
Tala et al Finland - Helsinki 1946-1966 Department of All TAAs Autopsy NR
(1967) pathology University
of Helsinki
Young et al Germany - Hannover 1977-1981 Unselected series of All TAAs Autopsy NR
(1987) autopsies
(continued on next page)
7
ADULT  Original Submission

access to some extra-

autopsy which did not
aneurysm detected at
(0.03% vs 0.16%). When autopsy studies were excluded from

directly cause death.

Rupture was defined
as blood penetration
“Asymptomatic TAA

aortic wall, gaining

the analysis the prevalence was lower (0.07% vs 0.16%).
was defined as an

of all layers of the

aortic part of the

thoracic cavity”
Definition of TAA

We also analyzed data according to geographical area by

dividing the studies according to the continent were the popu-
lation-based data was retrieved. Asia17,28 and South America14
only had one study each, regarding either incidence or preva-
lence of TAAs. Comparing Europe and North America, and
when autopsy studies were excluded (which were only per-
formed in Europe), North America tended to have a higher
incidence (7.3 vs 4.8 TAAs per 100,000 individuals/year) and
prevalence (0.12% vs 0.07%) of TAAs. On the contrary,
regarding ruptured TAAs, the incidence was slightly higher in
Methodology

Europe comparing to North America (2.2 vs 1.8 per 100,000

Diagnostic

individuals/year).
Autopsy

In orange studies only reporting ruptured TAA. In green population-based studies reporting on all types of TAAs. In yellow autopsy studies.

Risk of Bias
Overall, the risk of bias was considered to be high. We
found a significant risk of bias in the measurement of the
outcome.1,2,4,1316,1922,2426,28 Since TAAs are mainly
asymptomatic, the use of hospital medical records to calculate
Patients Reported

incidence of such a disease will inevitably underscore the real

value. Moreover, patients might have a ruptured TAA and die
on site, thus never reaching the hospital, if one is to use hospi-
tal admissions to measure such an outcome, these patients are
All TAAs

not accounted for. The only way to accurately measure the

incidence/prevalence of TAAs would be to screen the entire
population.
In the paper from Bons et al,18 the use of a cohort of patients
(accounts for 85% of
All autopsies in Malmo

over 55 might lead to a selection bias and overestimate the real

How Was the Data

prevalence of the disease in the general population. Additional

the population)

source of bias was the absence of adjusting for key risk factors,
which occurred in all studies expect in the study bt McClure et
Obtained

al,16 such as age, smoking, and hypertension (see Supplemen-

tary Data 5). We found a “small study effect” (Supplementary
Data 6) where smaller studies showed higher proportions.

DISCUSSION
1958-1985

There are two main findings in this systematic review: (1)

Year(s)

the pooled incidence and prevalence of TAAs is 5.3 per

100,000 individuals/year (95% CI: 3.0; 8.3) and 0.16% (95%
TAA, Thoracic aortic aneurysm; NR, Not Reported.

CI: 0.12; 0.20), respectively; (2) the pooled incidence of rup-

(Country/city or County)

tured TAAs was 1.6 per 100,000 individuals/year (95%CI: 1.3;

Geographic Location

2.1) (Fig. 7).

Sweden - Malmo

After sensitivity analysis by excluding the autopsy-based

studies, the prevalence of TAAs dropped down to 0.07% (95%
CI: 0.05; 0.11), and after we analyzed the different types of
studies separately, we found the prevalence of TAAs in the
autopsy-only studies to be 0.76% (95% CI: 0.47; 1.12). This
leads us to 2 possible explanations, either there is an overesti-
Table 1. (continued )

mation of TAAs in autopsy studies or there is an underestima-

tion in all other methodologies used.
Svensjo et al
Author (Year)

The differences found between both study designs are strik-

(1996)

ing. The autopsy studies, by inherently being design as such,

evaluated an older population, more prone to the development
of degenerative aneurysms, which might explain the

8 Seminars in Thoracic and Cardiovascular Surgery  Volume 34, Number 1

Seminars in Thoracic and Cardiovascular Surgery  Volume 34, Number 1

Table 2. Study Extracted Data

Author (Year) Total Number of Calculated Age (Mean- Male Sex (%) Number of Location (asc/ Size of TAA Etiology of TAA
Population Patients With Incidence (per SD) Ruptured arch/desc) (Mean-SD)
TAAs 100,000) Aneurysms
Ingoldy et al 248 000 78 2-4 NR NR 78 NR NR NR
(1986)
Drott et al 363 000 (1952) 590 4,2(1960); 4 71,2 (0,5) 54 590 384 / 59 / 147 NR NR
(1992) - 431 000 (1988)
(1988)
Johansson et 1.5 M (1980); 82 (1980); 76 5 70 (NR) for 45 (1980); 82 (1980); 76 81 / 23 / 45 NR Overall 61
al (1995) 1.6M (1989) (1989) men and 72 49 (1989) (1989) postdissection; no
(NR) women syphilitic TAAs
Clouse et al 100,000 28 3,5 NR NR 28 NR NR All degenerative
(2004)
Abdulameer 281.4 M 13788 0,31 NR NR 13788 NR NR NR
et al (2018)
Yamaguchi et 127,985,133 3095 0.6 NR NR 3095 NR NR NR
al (2019)
Bickerstaff et 45,000 72 5,3 NR (range 47- 39 53 37 / 8 / 27 NR Postdissection in 37
al (1982) 93; Median cases; degenerative in
65 (men); 77 15; Aortitis in 4; Cystic
(women)) medial necrosis in 3
and syphilis in 2
Samy et al 781,300 (1980) 169 2,3 NR NR NR NR NR NR
(1993) - 695,430
(1989)
Lilienfeld et al 2,113,533 NR 3,8 NR NR NR NR NR NR
(1993)
Clouse et al 100,000 133 10,4 69 (NR) 49 28 52 (asc and/or 49 (0,2) NR
(1998) arch) / 42
Itani et al 6971 11 NR 60,3 (12,1) 82 NR 3 / NR / 8 48,8 (10,1) NR
(2002)

ADULT  Original Submission

Olsson et al 8,7 M 6614 4,8 NR NR 2235 NR NR NR
(2006)
Santo et al 545,908 5825 0,79 NR 59 3439 NR NR NR
(2012)
Von Allmen et 432,997,984 27256 4,4 (1999); 9 NR NR 5702 NR NR NR
al (2013) (2010)
Kalsch et al 4129 12 NR 59.4 (7.8) NR NR NR NR NR
(2013)
McClure et al 13,7 M 9392 7,56 67 (NR) 64 NR NR NR 140 were due to
(2018) connective tissue
disease. Other
(continued on next page)
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ADULT  Original Submission

Table 2. (continued )
Author (Year) Total Number of Calculated Age (Mean- Male Sex (%) Number of Location (asc/ Size of TAA Etiology of TAA
Population Patients With Incidence (per SD) Ruptured arch/desc) (Mean-SD)
TAAs 100,000) Aneurysms
etiologies not
mentioned
Geisbuch et 81,405,718 48098 5.9 69 (12) 65.2 5848 NR NR NR
al, 2018
Bons et al 2505 6 NR NR 83 NR 4 / NR / 2 NR NR
(2019)
Lodewyks et 1,278,365 139 10.9 70 63 NR NR NR NR
al (2020) (2016)
Tala et al 10,392 98 NR NR 65 48 53 / 23 / 22 NR 66 were non-syphilitic
(1967) and 32 were syphilitic
(25 of these in the Acs
Aorta). 35 were post-
dissection
Young et al 3375 30 NR NR NR NR NR NR 12 were post-
Seminars in Thoracic and Cardiovascular Surgery  Volume 34, Number 1

(1987) dissection; 4 were

syphilitic
Svensjo et al 58,405 312 Asymptomatic Asymptomatic: 53 63 36 / 83 / 119 NR 10% had aortitis. 44
(1996) 489 per 100 77,7 (male), cases were post-
000; Rupture 85,3 (female); dissection
437 per 100 Rupture: 79,1
000 (male); 79,8
(female);
In orange studies only reporting ruptured TAA. In green population-based studies reporting on all types of TAAs. In yellow autopsy studies.
Asc, Ascending Aorta; Desc, Descending Aorta; NR, Not Reported; TAA, Thoracic aortic aneurysm.
 ADULT  Original Submission

Figure 2. Random-effects forest plot of incidence rate of thoracic aortic aneurysms (TAA). Pooled point estimate expressed as indi-
vidual per year with newly diagnosed TAAs (incidence).

Figure 3. Random-effects forest plot of prevalence of thoracic aortic aneurysms (TAA). Pooled point estimate expressed as fre-
quency of individuals with TAA in the population (prevalence).

Figure 4. Random-effects forest plot of incidence of ruptured thoracic aortic aneurysms (TAA). Pooled point estimate expressed as
individuals per year with ruptured TAAs (incidence).

Seminars in Thoracic and Cardiovascular Surgery  Volume 34, Number 1 11

ADULT  Original Submission

Figure 5. Random-effects forest plot of prevalence of thoracic aortic aneurysms (TAA) in autopsy studies. Pooled point estimate
expressed as frequency of individuals with TAA in the population screened (prevalence).

Figure 6. Top: Random-effects forest plot of the frequency of ascending thoracic aortic aneurysms (TAA); middle: Random-effects
forest plot of the frequency of Arch TAAs; bottom: Random-effects forest plot of the frequency of Descending TAAs.

12 Seminars in Thoracic and Cardiovascular Surgery  Volume 34, Number 1

 ADULT  Original Submission

Table 3. Sensitivity analysis.

Incidence (per 100 000/year) Prevalence (%)
Overall TAAs
Including all studies 5.3 (95% CI: 3.0; 8.3) 0.16 (95% CI: 0.12; 0.20)
Including CT studies only NA 0.21 (95% CI: 0.13; 0.31)
Including only hospital admission- 5.5 (95% CI: 4.8; 6.2) 0.03 (95% CI: 0.01; 0.06)
based studies
Recent data (>2000) 5.5 (95% CI: 2.5; 9.5) 0.07 (95% CI: 0.03;0.11)
Past data (<2000) 5.0 (95% CI: 2.8; 7.8) 0.34 (95% CI: 0.13; 0.67)
Past data (<2000) without including NA 0.09 (95% CI: 0.02; 0.23)
autopsy-studies
Excluding autopsy-based studies NA 0.07 (95% CI: 0.05; 0.11)
Excluding autopsy-based and NA 0.18 (95% CI: 0.11; 0.26)
admission-based studies
Excluding autopsy-based and NA 0.05 (95%CI 0.03; 0.09)
papers with less than 10,000
people in the population
Excluding death certificate 5.8 (95%CI: 5.2; 6.5) NA
Excluding hospital admission-based 4.6 (95%CI: 0.3; 13.8) 0.40 (95% CI: 0.19; 0.70)
studies
Only age-standardized data 6.0 (95% CI: 2.8; 10.4) NA
North America 7.3 (95% CI: 5.0; 10.0) 0.12 (95% 0.060.19)
South America 0.79 (only one study)14 0.012 (only one study)14
Asia NA 0.16 (only one study)17
Europe 4.8 (95% CI: 4.15.4) 0.25 (95% CI: 0.160.35)
0.07 (95% CI: 0.020.15)
 if excluding autopsy-only studies
Excluding papers with high ROB in NA 0.15 (95% CI: 0.11; 0.19)
the recruitment of the study
population
Excluding papers with high ROB in NA 0.48 (95% CI: 0.27; 0.72)
the measurement of the outcome
Rupture TAAs
Including all studies 1.6 (95% CI: 1.3; 2.1) NA
Excluding papers using death 2.1 (95% CI: 1.6; 2.6) NA
certificates
Recent data (>2000) 1.0 (95% CI: 0.1; 1.3) NA
Past data (<2000) 4.0 (95% CI: 3.0; 5.2) NA
North America 1.8 (95% CI: 0.3; 4.7) NA
Europe 2.2 (95% CI: 1.6; 3.0) NA
South America 0.33 (only one study)12 NA
Asia 0.6 (only one study)28 NA
NA, Nonapplicable; ROB, Risk of Bias; TAAs, Thoracic Aortic Aneurysms.

differences.5,29,30 However, since thoracic aortic aneurysms are
majorly asymptomatic and difficult to screen, an underreport-
ing of these conditions in the population-based studies has to
be considered. If that is the case, the most accurate estimate on
prevalence might be achieved by merging both types of studies
 0.16%.
In fact, we found a significant risk of bias in the mea-
surement of the outcome. Since thoracic aortic aneurysms
are mainly asymptomatic, most patients either have their
diagnosis made incidentally on a chest examination or
when they rupture.
Considering the natural history of the disease, the timing of
treatment of a TAA is highly relevant for a patient’s
prognosis.4,31 The difference in 30-day mortality, for example,
if treated for an intact TAA in an elective setting is 7.6%, com-
pared to 35% if treated for rupture.4 In addition, most patients
with ruptured TAAs do not reach the hospital alive,5,18 with an
overall mortality of 94%100%.1,2 The amount of preventable
deaths would be significant if a timely diagnosis were to be
made.
Considering the estimate of 5.3 new cases per 100,000 indi-
viduals/year and an overall prevalence of 0.16% at any given
time in the population, the actual number of patients affected
by this condition is relevant.
A screening program is probably not possible to implement
currently since for an accurate diagnosis a chest-CT is usually

Seminars in Thoracic and Cardiovascular Surgery  Volume 34, Number 1 13

ADULT  Original Submission

Figure 7. Graphical abstract summarizing the main outcomes: Overall incidence of thoracic aortic aneurysms (TAA); Overal preva-
lence of TAAs and Incidence of ruptured TAAs. Take-home messages: (1) The pooled incidence and prevalence of thoracic aortic
aneurysms (TAAs) was 5.3 per 100,000 individuals/year and 0.16% respectively. (2) The pooled incidence of ruptured TAAs was
1.6 per 100,000 individuals/year. (3) We found a significant difference of the prevalence in autopsy studies, which was 0.76% and
the prevalence of TAAs dropped down to 0.07% when these studies were excluded from the overall analysis. (4) The lack of well-
design population-base studies and the limitations encountered serve as calling for future research in this field.

necessary. However, opportunistic screening might be possi-
ble. Some of these aneurysms might be diagnosed with trans-
thoracic echocardiograms, since in most cases, a general
measure of the ascending and descending aortic diameter can
be made. In our review, we found these 2 locations to be the
more common occurring in 45.5% and 34.6%, respectively, in
patients with TAAs. Another way to timely diagnose these
patients is to perform a full aortic imaging when aneurysms in
other locations are found, since we know nowadays that aortic
aneurysms are a systemic disease32,33 which can occur in a syn-
chronous and metachronous manner.34,35
Comparing studies prior to 2000 and more recent ones we
found both an increase in incidence and a decrease in preva-
lence (Table 3). Controversy exists, however, regarding this
observation, since studies have described variations in both
ways.4,13,16 Studies that have described an increase in the inci-
dence of TAAs have attributed this trend due to the increase in
disease awareness and improvement in diagnosis, with better
imaging,16,21 and studies that have described a decrease in inci-
dence have attributed it to the worldwide decline in smoking
habits.13,36 In our study, an increase in incidence with a
decrease in prevalence might occur due to an improvement in
disease awareness and diagnostic tools (leading to increased
incidence) and a higher percentage of patients being treated,
especially with endovascular techniques which have expanded
the possibility of treatment to more patients31 (leading to a
decrease in prevalence). This higher awareness and diagnosis
of TAAs, might also explain, in part, the decrease in the inci-
dence of ruptured TAAs observed before and after 2000, with
more patients being offered timely treatment.
We found that North America had, simultaneously a higher
incidence of TAAs and lower incidence of rTAA. This might
reflect a higher disease awareness leading to a timelier diagno-
sis and thus lowering the rupture incidence by increasing elec-
tive aneurysm repair. This is only theoretical, as more
information is needed to draw further conclusions.
Future well designed population-based studies and well-
kept registries, reflecting the current incidence, prevalence and
behavior of TAAs, are necessary to bring a sharper and correct
answer to our questions. In addition, further studies are
needed to understand the true impact in quality-adjusted life
years for this disease and if population screening strategies,

14 Seminars in Thoracic and Cardiovascular Surgery  Volume 34, Number 1


either primary or opportunistic, would be cost-effective and
beneficial, as they have been reported for abdominal aortic
aneurysms.3,37 The significant difference between the preva-
lence found in autopsy and clinical studies is striking, as it may
mean that a significant amount of TAAs are undiagnosed with
possible devastating impact to patients. This should lead to fur-
ther study of both their impact and the feasibility and benefit of
TAA screening.
Information regarding risk factors, sex, ethnic, family and
genetic differences, is also important to analyze in these studies
in order to better understand this pathology. In our review this
was not possible due to lack of reporting in the studies.
Also, etiology of thoracic aortic aneurysms also needs to be
further investigated. In our study, due to heterogeneous report-
ing we were unable to pool this data, but we found a high fre-
quency of postdissection TAAs in the studies (Table 2). This
might have important prognostic implications and should be
more thoroughly investigated. Genetically triggered aneurysms
would also be important to differentiate, which was not possi-
ble given the available data.
This paper has some limitations. The population-based
studies encountered had different study designs and meth-
ods of reporting. Data of prevalence and incidence were
not available across all studies. Most of studies occurred
over 10 years ago which might give us a biased view since
the diagnostic methods were not as accurate and sensitive
as nowadays. Standardized methods of diagnosis and defi-
nition of TAA were also not present and differed across
studies. In autopsy studies, there was no mention regarding
previous repair of the aneurysms found, although we
assumed them to be untreated, it might not have been the
case and an overestimation of their prevalence might exist
in these studies. The differences in study designs, diagnos-
tic methods and the population included might explain, at
least, partially, the statistical heterogeneity observed and
also explains the wide variations demonstrated in our sensi-
tivity analysis (Table 3). Also, standardized measurements
of the aorta, as aortic index or height or body surface area
adjustments, were not available across the primary studies.
In addition, we found a “small study effect” (Supplementary
Data 6) where smaller studies showed higher proportions
which leads to further heterogeneity of the results.
Furthermore, the lack of information regarding aneurysm
size at the time of diagnosis also renders further conclusions
regarding the true risk of rupture of the aneurysms found.
CONCLUSION
This systematic review and meta-analysis reached an esti-
mate regarding the incidence and prevalence of TAAs, both
intact and ruptured and across different study designs.
Despite significant limitations, due to the information avail-
able, the current epidemiologic information provided might
serve as a base for future public-health decisions. The lack
of well
Seminars in Thoracic and Cardiovascular Surgery  Volume 34, Number 1
ADULT  Original Submission
AUTHORS CONTRIBUTIONS
RGM and GSD conceived the idea for the protocol and made
the main contribution to planning and preparation of timelines
for completion. RGM and AL analyzed all papers, extracted the
data and analyzed the risk of bias. GSD and RGM performed
the statistical analysis. DC and MA analyzed the data and con-
firmed the statistical analysis. RGM designed the tables and
wrote the first draft of the manuscript, which was then
reviewed and amended by MA, DC, RFF and LMP. All authors
then approved the final written manuscript. RGM is the guar-
antor for the work.
SUPPLEMENTARY MATERIAL
Scanning this QR code will take you to the article title page
to access supplementary information.
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