European Journal of Radiology

Case Report: An Incidental Case of Chronic Aortic Dissection

--Manuscript Draft--

Manuscript Number: EJR-D-22-02490

Article Type: Research Article

Section/Category: Cardiovascular Imaging

Keywords: Aortic dissection; Aneurysm; Angiogram; Chronic

Corresponding Author: Sneha Shrestha, MBBS

Kathmandu University School of Medical Sciences
Dhulikhel, NEPAL

First Author: Sumarg Simkhada

Order of Authors: Sumarg Simkhada

Sneha Shrestha, MBBS

Suyesh Raj Shrestha

Pramit Ram Shrestha

Abhyuday Kumar Yadav

Aman Raj Shrestha

Abstract: Highlights

1. A rare case of chronic aortic dissection (Stanford type A)

2. Involvement of ascending and descending thoracic aorta, aortic arch and suprarenal
part of abdominal aorta
3. A filling defect in left main pulmonary artery indicating embolism
4. Presence of left sided pleural effusion
Introduction
Chronic aortic dissection is a rare variant of aortic dissection where dissection in a
weakened aortic wall and subsequent blood flow occur over a period of 3 months or
more. The underlying mechanism for chronicity and global burden of the disease is not
well documented or understood. Further contributed by the overall rarity of aortic
dissection, literature published on the topic is lacking.
Case report
We present a case of a 83 year old lady who presented with features of Cor Pulmonale
with coexisting right hypochondrium tenderness which when further evaluated with a
USG revealed an aortic aneurysm with features of dissection. Further evaluation with a
CT scan revealed a Stanford type A, DeBakey type I aortic dissection with a false flap
and calcific changes hinting to its  chronic nature.
The patient was managed for the presenting features and is under regular outpatient
care for aortic dissection.
Discussion
Among the survivors of acute dissection/aneurysms people in their 6th and 7th decade
and having a history of familial disease, dyslipidemia, arterial hypertension and aortic
surgeries seem to be at  higher risk of chronic dissection. Although more common in
males,females seem to have worse prognosis. Various imaging modalities may be
useful in diagnosis, CT being the most reliable. Intimal tears and flaps showing flow in
the false lumen and calcification of the aortic wall are some positive radiological
findings. Thrombus formation in the slow blood flowing false lumen with subsequent
embolisation, secondary aneurysms and rupture, end organ malperfusion, pericardial
tamponade may be some dreaded complications. These may be avoided by timely
diagnosis and intervention that may extend the life expectancy by as much as 7 years.
Conclusions:
Chronic Aortic dissection is a rare and complicated entity that is not well understood.
Various complications of the disease may be the cause of severe morbidity and
mortality. Timely diagnosis and interventions such as aortic repair and/ or active
surveillance  are essential for a positive outcome.

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Assistant Professor
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Pawan Katwal, MD
drpawankatwal91@gmail.com

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Cover Letter

24/5/2022

To,
The Editor,
Annals of Medicine and Surgery

Subject: Cover letter for submission of a manuscript

Dear Sir,

This is to inform you that we would like to submit a manuscript with title “An Incidental
Case of Chronic Aortic Dissection” in your esteemed journal. We hope for a positive
response from the editorial team and we hope to be associated with the journal.

Sincerely,

Shrestha S.,
Department of Radiology,
Dhulikhel Hospital,
Kathmandu University Hospital
Conflict of Interest

Conflicts of Interest
There are no conflicts of interest.
Title Page (including paper title & Complete corresponding author
details)

An Incidental Case of Chronic Aortic Dissection

Authors :
Sumarg Simkhada, Sneha Shrestha, Suyesh Raj Shrestha, Pramit Ram Shrestha,
Abhyuday Kumar Yadav, Aman Raj Shrestha

Sumarg Simkhada, S., Simkhada, Kathmandu University School of Medical

Sciences, Dhulikhel, 45210, Nepal
Email : sumargasimkhada@gmail.com

Sneha Shrestha, S., Shrestha, Kathmandu University School of Medical Sciences,

Dhulikhel, 45210, Nepal
Email: snehashrestha091@gmail.com
ORCid: 0000-0002-5575-7882

Suyesh Raj Shrestha, S.R, Shrestha, Kathmandu University School of Medical

Sciences, Dhulikhel, 45210, Nepal
Email: suyesh311@gmail.com
ORCid: 0000-0002-5883-5049

Pramit Ram Shrestha, P.R, Shrestha, Kathmandu University School of Medical

Sciences, Dhulikhel, 45210, Nepal
Email: pramit.shrestha.prs@gmail.com
ORCid: 0000-0002-2596-1671

Abhyuday Kumar Yadav, A.K, Yadav, Kathmandu University School of Medical

Sciences, Dhulikhel, 45210, Nepal
Email: abhyudayjnk1999@gmail.com
ORCid: 0000-0002-7905-9026
Aman Raj Shrestha, A.R, Shrestha, Kathmandu University School of Medical
Sciences, Dhulikhel, 45210, Nepal
Email : Amanrajstha123@gmail.com
Manuscript containing Abstract, Sections and References (Without Click here to view linked References
corresponding author details) CLEAN

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Case Report : An Incidental Case of Chronic Aortic
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8 Dissection
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12 Authors :
13 Sumarg Simkhada, Sneha Shrestha, Suyesh Raj Shrestha, Pramit Ram Shrestha,
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15 Abhyuday Kumar Yadav, Aman Raj Shrestha
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Sumarg Simkhada, S., Simkhada, Kathmandu University School of Medical
21 Sciences, Dhulikhel, 45210, Nepal
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23 Email : sumargasimkhada@gmail.com
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27 Sneha Shrestha, S., Shrestha, Kathmandu University School of Medical Sciences,
28 Dhulikhel, 45210, Nepal
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30 Email: snehashrestha091@gmail.com
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32 ORCid: 0000-0002-5575-7882
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36 Suyesh Raj Shrestha, S.R, Shrestha, Kathmandu University School of Medical
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38 Sciences, Dhulikhel, 45210, Nepal
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40 Email: suyesh311@gmail.com
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42 ORCid: 0000-0002-5883-5049
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46 Pramit Ram Shrestha, P.R, Shrestha, Kathmandu University School of Medical
47 Sciences, Dhulikhel, 45210, Nepal
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49 Email: pramit.shrestha.prs@gmail.com
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51 ORCid: 0000-0002-2596-1671
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55 Abhyuday Kumar Yadav, A.K, Yadav, Kathmandu University School of Medical
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57 Sciences, Dhulikhel, 45210, Nepal
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59 Email: abhyudayjnk1999@gmail.com
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ORCid: 0000-0002-7905-9026
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8 Aman Raj Shrestha, A.R, Shrestha, Kathmandu University School of Medical
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10 Sciences, Dhulikhel, 45210, Nepal
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12 Email : Amanrajstha123@gmail.com
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22 Highlights
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24 1. A rare case of chronic aortic dissection (Stanford type A)
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26 2. Involvement of ascending and descending thoracic aorta, aortic arch and
27 suprarenal part of abdominal aorta
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29 3. A filling defect in left main pulmonary artery indicating embolism
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31 4. Presence of left sided pleural effusion
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39 Keywords :
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41 Aortic dissection; Aneurysm; Angiogram; Chronic
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5 Abstract
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7 Introduction
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9 Chronic aortic dissection is a rare variant of aortic dissection where dissection in a
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weakened aortic wall and subsequent blood flow occur over a period of 3 months or
12 more. The underlying mechanism for chronicity and global burden of the disease is
13
14 not well documented or understood. Further contributed by the overall rarity of
15 aortic dissection, literature published on the topic is lacking.
16
17 Case report
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19 We present a case of a 83 year old lady who presented with features of Cor
20 Pulmonale with coexisting right hypochondrium tenderness which when further
21
22 evaluated with a USG revealed an aortic aneurysm with features of dissection.
23
24 Further evaluation with a CT scan revealed a Stanford type A, DeBakey type I aortic
25 dissection with a false flap and calcific changes hinting to its chronic nature.
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27 The patient was managed for the presenting features and is under regular outpatient
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29
care for aortic dissection.
30 Discussion
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32 Among the survivors of acute dissection/aneurysms people in their 6th and 7th
33 decade and having a history of familial disease, dyslipidemia, arterial hypertension
34
35 and aortic surgeries seem to be at higher risk of chronic dissection. Although more
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37 common in males, females seem to have worse prognosis. Various imaging
38 modalities may be useful in diagnosis, CT being the most reliable. Intimal tears and
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40 flaps showing flow in the false lumen and calcification of the aortic wall are some
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42 positive radiological findings. Thrombus formation in the slow blood flowing false
43 lumen with subsequent embolisation, secondary aneurysms and rupture, end organ
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45 malperfusion, pericardial tamponade may be some dreaded complications. These
46
47
may be avoided by timely diagnosis and intervention that may extend the life
48 expectancy by as much as 7 years.
49
50 Conclusions:
51 Chronic Aortic dissection is a rare and complicated entity that is not well understood.
52
53 Various complications of the disease may be the cause of severe morbidity and
54
55 mortality. Timely diagnosis and interventions such as aortic repair and/ or active
56 surveillance are essential for a positive outcome.
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7 Introduction
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11 Aortic dissection is a rare but life threatening disease with an incidence of 5 to 30
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cases per 1 million people per year.1 Compromised aortic integrity due to inherited
14 or acquired aortic wall instability results in a tear in intimal layer and aortic
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16 dissection subsequently.2 Further entry of blood between intima and media is the
17 underlying pathology for aortic dissection.2 Aortic dissection occurring after 3
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19 months are referred to as chronic aortic dissection which is even rare.2 There is
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21 paucity of research regarding chronic aortic dissection due to its rarity making it a
22 very important topic to be discussed. The actual prevalence of patients living with
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24 chronic aortic dissection throughout the world and the expenses for long term care
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is unknown showing a huge research gap.3 This case report has been prepared with
27 an objective of giving more information about this case and reflecting the paucity of
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29 interest shown in this condition regarding the risk prediction and tools for extracting
30 advanced prognostically relevant features about the condition. This case report was
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32 prepared according to SCARE criteria.4
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36 Case Presentation
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38 An 83 year old woman presented to the emergency department of Dhulikhel
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40 Hospital with gradually increasing shortness of breath over 5 days and an
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42 intermittent cough that was productive, purulent, foul smelling and mixed with blood
43 for 4 days. She also had high grade fever, chest pain, and decreased appetite over the
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45 past few days. On examination, there was bilateral non tender pitting pedal edema
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with no overlying skin changes however facial puffiness could be appreciated. A
48 chest examination revealed wheeze and crepitations. A provisional diagnosis of Cor
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50 Pulmonale was made and she was managed accordingly.
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53 Ultrasonography of the abdomen was performed because of tenderness in the right
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55 hypochondrium which incidentally revealed an aortic aneurysm with features of
56 aortic dissection and a Multi Detector CT angiogram of aorta showed involvement
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58 of the ascending aorta, descending thoracic aorta and suprarenal part of abdominal
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60 aorta with a mural thrombosis in the false lumen. Aneurysmal dilatation measuring
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3.36cm involved the descending thoracic aorta whereas aneurysmal dilatation
6 measuring 3.20 cm was present in the suprarenal abdominal aorta. A double lumen
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8 aortic dissection which was Stanford type A, DeBakey type I and an eccentric filling
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defect within the left main pulmonary artery and its lower segmental branch
11 suggesting embolism were also noted as findings. A flap measuring 0.51cm was
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13 present with low density false lumen which was larger than true lumen(Fig 7). The
14 flap was straight and calcification was seen. Pericardial effusion and left sided
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16 pleural effusion were also observed in the CT angiogram.(Fig 1 and 5)
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19 No active interventions were made for aortic dissection credit to asymptomatic state
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21 and old age of the patient but is under active follow up.
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Discussion
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Chronic aortic dissection is a rare form that occurs 3 months from its onset.1 The
45 prevalence rate of chronic aortic dissection is 28 to 42 per 100000 survivors of acute
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47 dissection in the US.5(chronic ct aortic diss) Patients with this disease have sudden
48 onset severe tearing chest pain. Older age(50-65 years) males, dyslipidemia, arterial
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50 hypertension, aortic surgeries and a positive family history are some important risk
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52 factors.1,2 Women have a lower risk of disease, however among the diseased, they
53 present later and have worse prognosis.2 Our patient is also a lady who presented
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55 very late.
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58 Stanford system and Debakey classification are used to classify aortic dissection.
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60 According to the Stanford system and Debakey classification, our patient falls under
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DeBakey I and Stanford A type. Patients with operated Stanford type A dissection
6 are the largest group progressing to chronic dissection later and those with non
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8 operated Stanford type A very rarely have chronic aortic dissection as they don’t
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survive in the acute event.3 As our patient is an asymptomatic chronic patient and
11 falls under Stanford type A, this is indeed a very rare event.
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14 CT scan, Echocardiography, Magnetic Resonance Imaging and Invasive
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16 Aortography are some of the diagnostic modalities to diagnose aortic dissection. 6
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18 Ultrasonography shows widening aortic silhouette in abdominal aortic aneurysm
19 type. Confirmation of diagnosis is done by identifying the presence of intimal tear
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21 through CT which is the imaging modality of choice.2,3 High Resolution CT scan
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shows intimal tear and intimal flap leading blood flow into true and false lumens.
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26 Outer wall calcifications are a feature more common in true lumen but can be seen
27 in false lumen as well.7 Incidence of thrombus formation in false lumen is more
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29 common in chronic dissections than acute dissections.7 Mostly, true lumen is smaller
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31 than false lumen and over time, blood flows over false lumen causing development
32 of aortic aneurysm.1
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36 Beak sign(acute angle between the dissection flap and outer aortic wall) is usually
37 seen in acute aortic dissection and rarely in chronic as the wall is fibrosed and
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39 straight in such cases.7 False lumen is prone to thrombus formation in chronic aortic
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dissection cases due to atheromatous changes in the newly formed intima. 7 Also,
42 flaps in chronic aortic dissection are devoid of curvature and appear rather straight
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44 due to the fibrosis and neointima formation causing thick, rigid and straight flaps.7,8
45 Thrombus formation in false lumen occurs in acute cases primarily due to
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47 thrombogenic mediators and blood stasis in false lumen while thrombus in chronic
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49 cases is due to stasis related to aneurysmal enlargement and atheromatous changes
50 in neointima.7 In this case, false lumen, flap calcification and thrombus in the false
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52 lumen was observed.
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55 A study showed that the patients who underwent aortic repair or died had a
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57 significant increase in size of false lumen over time compared to those without
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intervention where the size didn’t change demonstrating that patency of false lumen
60 is an independent risk factor for dissection related aortic rupture, surgery or death.9
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6 Pleural effusion is a complication of acute aortic dissection. Some studies suggest
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8 that leukocytosis, high body temperature, thrombocytosis resulted in pleural effusion
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while some say it occurred secondary to atelectasis. The reason for pleural effusion
11 in chronic aortic dissection as in this case is unclear, however Schattner et al,
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13 Veyssier-Belot et al, Giladi et al and Murray et al reported occurrence of pleural
14 effusion in some of their patients with chronic aortic dissection. 10 There were also
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16 studies that showed presence of pericardial effusion in the patients with aortic
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18 dissection which aligns with the result in our patient as well.11,12
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21 Therapeutic administration of beta blockers, lifelong surveillance and repair of aortic
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dissection with aneurysm is the treatment protocol for chronic aortic dissections.3,5
24 Surgical repair is opted when complications like rupture, malperfusion syndromes,
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26 aneurysmal transformation arise.5 Most of the patients with chronic type develop
27 aortic dilatation in proximal descending aorta so they should be operated with open
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29 aortic replacement or endovascular therapy depending on age, anatomy and other
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31 associated disorders. The need for surgical repair in patients with chronic type A
32 aortic dissection depends upon the aortic diameter, which according to the current
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34 guidelines is 55 mm.13 Stent-assisted balloon-induced intimal disruption and
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36 relamination of aortic dissection (STABILISE) technique has been used successfully
37 for complicated chronic aortic dissection. 14 Our patient, due to her age(83 yrs) and
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39 uncomplicated aortic dissection is kept under medications and for frequent follow
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up.
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44 Aortic rupture, pericardial tamponade, aortic regurgitation, end-organ malperfusion,
45 and acute heart failure are some complications connected to aortic dissection out of
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47 which aortic rupture is the most feared complication in 3-18%. 3,15 Aortic aneurysms
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49 are commonly seen in chronic aortic dissection and are the primary reason for
50 reoperations as well.3 Average survival of seven years is expected for the people
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52 with aortic dissection.3
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7 Conclusion
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9 An aortic dissection presenting as a chronic complaint is a rare phenomenon.
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11 ( straight and thick flap pointed to intimal calcification—chronic
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13 Chronic aortic dissections who are asymptomatic can be managed by beta blockers
14 and lifelong surveillance. Surgical correction in a chronic A type aortic dissection is
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16 needed when the aortic diameter is 55 mm or more. Aortic dissection is a fatal
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18 condition which can get worse due to various complications, hence prompt workup
19 and treatment is important.
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Declaration of patient consent
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8 Written informed consent was obtained from the patient for publication of this case
9 report and accompanying images. A copy of the written consent is available for
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11 review by the Editor-in-Chief of this journal on request.
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Sources of funding
17 None
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22 Ethical approval
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27 As the case report contains information of the retrospective period, we had obtained
28 exempt for ethical approval from the Institutional ethical committee.
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Conflicts of Interest
36 There are no conflicts of interest.
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41 Provenance and peer review
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43 Not commissioned, externally peer-reviewed
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32 Figure 1 : Chronic dissection with involvement of arch of aorta with left sided pleural
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42 Figure 2 : Chronic dissection completely involving descending thoracic aorta and
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29 5. Giugliano G, Spadera L, De Laurentis M, et al. Chronic aortic dissection: still a challenge. Acta
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Yadav
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Patient Care
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Dr. Sumarg Simkhada,
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