Table of Contents  
CASE SERIES
Year : 2022  |  Volume : 9  |  Issue : 2  |  Page : 183-187

Unusual Etiologies of an Unusual Problem: Primary Aortic Mural Thrombus


Departments of General Surgery, MGM Medical College and Hospital, Navi Mumbai, Maharashtra, India

Date of Submission30-Dec-2021
Date of Acceptance21-Feb-2022
Date of Web Publication13-Jun-2022

Correspondence Address:
Vamsi Lakshman Pappu
Departments of General Surgery, MGM Medical College and Hospital, Navi Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_136_21

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  Abstract 


We report on four patients, patient 1 with left upper quadrant pain as a result of splenic infarction; the patient was subsequently found to have a thoracoabdominal aortic thrombus extending through the celiac axis. Patient 2 with breathlessness due to COVID-19 and incidental finding of aortic thrombus. Patient 3 with right lower limb gangrene due to an embolus from a partial lumen occluding thrombus in the abdominal aorta and COVID-19 positive. Patient 4 with left lower limb ischemia As this remains a relatively rare disease, there is no consensus on the nature of disease, prognostic assessments, or optimal treatment strategy.

Keywords: Anticoagulation, COVID-19, embolism, primary aortic mural thrombus, thrombectomy


How to cite this article:
Kadam S, Pappu VL, Sharma S. Unusual Etiologies of an Unusual Problem: Primary Aortic Mural Thrombus. Indian J Vasc Endovasc Surg 2022;9:183-7

How to cite this URL:
Kadam S, Pappu VL, Sharma S. Unusual Etiologies of an Unusual Problem: Primary Aortic Mural Thrombus. Indian J Vasc Endovasc Surg [serial online] 2022 [cited 2022 Jul 1];9:183-7. Available from: https://www.indjvascsurg.org/text.asp?2022/9/2/183/347255




  Introduction Top


Primary aortic mural thrombus (AMT) is a rare condition with a difficult diagnosis associated with a high rate of complications and mortality. The cause of an aortic thrombus is idiopathic in many patients, but it has been associated with atherosclerosis, aneurysms of the aorta. Other risk factors include hypercoagulable states such as smoking, endothelial disorders, neoplasms, and drugs such as steroids, oral contraceptives (OCPs), and heparin. The usual presentation is with symptoms/signs of peripheral embolization and, after further investigations, reveals an AMT. Autopsy reports quote a 0.45% incidence in the general population, with 17% showing evidence of distal embolization.[1]


  Case Report Top


Patient 1

The patient is a 41-year-old woman who came to the emergency department with a 6-month history of worsening left upper quadrant abdominal pain not associated with nausea and vomiting. She had never experienced these symptoms before. Her medical history was remarkable for an open appendicectomy 4 years ago. She is a known case of T2DM and hypertension on treatment. She gives a positive history for consumption of OCP for 12 months. She gives no history of significant weight loss. Her vital signs included a blood pressure of 130/90 mm Hg, a pulse of 120 beats/min, and a temperature of 98.2°F. Physical examination of her chest and pulses was normal. Mild tenderness was noted in the epigastrium and left upper quadrant of her abdomen with no guarding or rigidity. Her laboratory results included a leukocyte count of 14,500, a hemoglobin level of 11 g/dl, and a platelet count of 148 × 103/dl. Her electrocardiogram showed sinus tachycardia, and her 2D Echo showed no abnormalities.

A computed tomography (CT) scan [Figure 1], [Figure 2], [Figure 3] showed an infarcted spleen as well as a large thrombus in the descending thoracic aorta and abdominal aorta that extended past the celiac axis, common hepatic, left gastric, and splenic artery with significant stenosis of the splenic artery without any collateral reformation of the splenic artery with sparing of the superior mesenteric and renal arteries. A heparin infusion was begun and continued for 5 days, after which the patient was discharged on tablet rivaroxaban. The patient is called for follow-up after 3 months.
Figure 1: Computed tomography scan axial view shows infarcted spleen and large thrombus in descending aorta

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Figure 2: Three-dimensional reconstruction showing large descending aortic thrombus

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Figure 3: Computed tomography scan coronal section large descending aortic thrombus with splenic infarction

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After 3 months, repeat contrast-enhanced CT scan shows the decrease in size of the thrombus, and the patient has no complaints at present. The patient is called for follow-up after 6 months.


  Case Report Top


Patient 2

The patient is a 64-year-old male who came to the emergency department with a history of fever, cough, and breathlessness for 4 days. He had no comorbidities and no previous history of hospitalization. He was a chronic smoker consuming a pack of beedis a day. His vital signs included a blood pressure of 110/70 mm Hg, a pulse of 108 beats per minute, and a temperature of 101.7°F. Physical examination of his chest showed bilateral decreased air entry. His abdomen was soft with no tenderness, guarding, or rigidity. His laboratory results included a leukocyte count of 14,500, a hemoglobin level of 13 g/dl, and a platelet count of 331 × 103/dl. A CT [Figure 4] and [Figure 5] scan was performed to rule out pulmonary emboli, which showed no evidence for the same and incidentally revealed 30% luminal narrowing of the celiac trunk and 50% luminal narrowing of the right main renal artery. A heparin infusion was begun and continued. On his second hospitalization day, he required intubation after developing acute respiratory distress syndrome, and the patient succumbed to complications of COVID-19.
Figure 4: Computed tomography scan coronal section showing thrombus in descending thoracic aorta

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Figure 5: Computed tomography scan axial section showing thrombus in descending thoracic aorta

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  Case Report Top


Patient 3

Ten-day-old male child presented to the ED with the informant being his mother with complaints of black discoloration of his right foot for 4 days, first noticed on his toes. There was no history of trauma, fever, and umbilical artery catheterization at birth. Birth history had full-term normal vaginal delivery, small for gestational age baby, cried immediately after birth, and birth weight – 2.45 kg with no additional birth events. There was no history of NICU stay, immunized at birth. The baby was 3rd by order of birth with no congenital anomalies noted in previous siblings. On examination, the baby was alert, afebrile, HR – 138/min, BP – 90/60 mmHg, and SpO2 98% on room air. Local examination of the right lower limb revealed black discoloration of the right lower limb from toes up to mid-calf [Figure 6], CRT revealed delayed in the right thigh (7 s), the right femoral pulsations absent, the left lower limb – pink, warm, CRT – 6 s, feeble pulsations of the left femoral artery and popliteal artery, and absent pulsations of the left anterior tibial artery and posterior tibial artery. A Doppler study [Figure 7] and [Figure 8] revealed the presence of partial lumen occluding thrombus (hyperechoic) in the abdominal aorta below the level of renal arteries, absent flow in the right lower limb, and monophasic flow in the left femoral artery. A prothrombotic screen was performed, and the COVID antibody test was found to be positive. A systemic thrombolysis with injection reteplase was performed, which showed mild improvement in circulation with the propagation of clot up to the origin of superior mesenteric artery on a Doppler scan. A transverse lower abdominal incision was made, bowel loops were mobilized, and the abdominal aorta with the iliac bifurcation was observed. A transverse aortotomy incision was given [Figure 9] and a Fogarty catheter was passed distally and proximally for clot retrieval; a long segment thrombus from the distal abdominal aorta and bilateral femoral arteries was retrieved; followed by good proximal inflow and good distal backflow. Bilateral limb perfusion improved postoperatively though the gangrenous right lower limb could not be saved and a right below-knee amputation was performed for the same.
Figure 6: Right gangrenous lower limb

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Figure 7: Preoperative abdominal ultrasound demonstrating occlusive aortic thrombus. Axial image with large occlusive thrombus within abdominal aorta (yellow arrow) and a patent inferior vena cava (green arrow)

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Figure 8: Saggital image showing occlusive thrombus

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Figure 9: Transverse aortotomy closed with Prolene 6-0

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  Case Report Top


Patient 4

A patient is a 30-year-old man who came to the emergency department with a 7-day history of worsening pain in the left leg with difficulty in walking. He had never experienced these symptoms before. His medical history was remarkable for a left foot plating following a road traffic accident 10 years ago. He has no known comorbidities. He is a tobacco chewer and chronic alcoholic consuming around 90 ml daily for the last 12 years. He has no history of significant weight loss. His vital signs included a blood pressure of 132/90 mm Hg, a pulse of 86 beats/min, and a temperature of 97.5°F. Physical examination of his chest and pulses was normal. His laboratory results included a leukocyte count of 7540, a hemoglobin level of 11 g/dl, and a platelet count of 252 × 103/dl. A CT scan [Figure 10] showed a partial lumen occluding thrombus in infrarenal aorta extending into the proximal aorta, complete lumen occluding thrombus in the left anterior tibial artery extending into the deep peroneal artery. A heparin infusion was begun and continued for 5 days, after which the patient was taken for embolectomy. The patient tolerated the procedure well and was discharged the following week.
Figure 10: Computed tomography scan axial section showing thrombus in infrarenal aorta extending into the proximal aorta

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  Discussion Top


AMTs is a rare disease that can cause distal embolism but can also be detected incidentally in asymptomatic cases. Although AMT is associated with aortic disease such as aortic aneurysm, dissection, or atherosclerosis, a thrombus in a healthy aorta is unusual since the aortic blood flow is too fast for clotting and the formation of a thrombus.[2],[3].Other causes include malignancy, drugs such as oral contraceptive pills and heparin, infection, trauma, hypercoagulable triggers such as protein C, protein S, antithrombin III deficiency, and autoimmune diseases such as SLE and APLA.[4] Thus, the etiology of aortic thrombosis is not well understood [Figure 11]. Clinical presentation varies from asymptomatic incidental findings to signs of peripheral embolization. Our first case was found to have only one risk factor that was chronic consumption of OCP that led to long segment aortic thrombus and was treated with anticoagulation alone. Our last case was idiopathic and negative for prothrombotic workup treated with anticoagulation and embolectomy. A complete workup for hypercoagulability and accurate clinical assessment is imperative before initiating treatment. The various methods of treatment include anticoagulant therapy, local or systemic thrombolytic therapy, thrombectomy, and surgical resection. Medical management mainly involves systemic anticoagulation with heparin, usually as the first-line treatment, but optimal duration and intensity of anticoagulation are not well established. Early initiation of anticoagulation is associated with thrombus resolution in most of the cases. Surgical intervention is indicated in the presence of a mobile thrombus, persistence of thrombus, or re-embolization. Although there are multiple treatment options, there are no approved guidelines and there is no evidence on which method is better; hence, treatment largely depends on patient presentation, location, and extent of the thrombus.[5]
Figure 11: Pie chart of causes of PAMT encountered in our study

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COVID-19 was declared as a pandemic in March 2020, with symptoms such as fever, dry cough, headache, anosmia, myalgia to severe systemic involvement, including pneumonia, viral myocarditis, and various other coagulation abnormalities leading to stroke and myocardial infarction. Venous thromboembolism is now well recognized as a common complication of severe COVID-19 disease.[6] Our next two cases add to the growing list of potential sites and consequences of thrombosis in COVID-19. Together, these varied consequences add to our understanding of the pathophysiology of thromboembolism in COVID-19 disease. Inflammation leads to microvascular and macrovascular endothelial damage, which then leads to disturbances in the coagulation cascade.[7] There is also inhibition of fibrinolysis in this condition, often resulting in extensive thrombosis. The interleukin 6-related inflammatory storm stimulates the liver to produce fibrinogen and thrombopoietin and also disrupts the endothelium of the vessels leading to activation of the extrinsic pathway of coagulation, causing catastrophic thrombotic events. Neonatal aortic thrombosis is a rare condition that occurs in 0.2% of newborns admitted to the neonatal intensive care unit.[8] Symptomatic aortic thrombosis represents a significant subset of these neonates and has an incidence of 0.1–1.1 per 100,000 newborns.[9] The clinical presentation is variable and ranges from irritability to acute limb ischemia, intestinal malperfusion, and renal failure. Nearly 80% of the case are associated with umbilical artery catheterization, but other risk factors include dehydration, sepsis, polycythemia, a low cardiac output state, perinatal asphyxia, extreme prematurity, maternal diabetes, and inherited thrombophilias. Doppler ultrasonography remains the first-line modality to make the diagnosis. Treatment options include anticoagulation, thrombolytic therapy, and surgical thrombectomy. 90% of the published cases report the use of nonoperative treatment strategies, but treatment largely depends on patient presentation and expert opinion.[10]


  Conclusion Top


The isolated development of an AMT in an otherwise normal aorta is a phenomenon seen infrequently. Prompt recognition of this entity and ascertaining its underlying etiopathogenesis is crucial, as it can change the clinical outcomes to a greater extent. All possible etiologies such as infectious, inflammatory, malignant, hypercoagulable, autoimmune, COVID-19 disease, and familial aortic ailments must be excluded. Although multiple treatment options are available, there are no expert-approved guidelines for the same. While some studies suggest an aggressive approach to prevent recurrence and risk of peripheral embolization, others prefer a less invasive conservative approach. Anticoagulation remains as an effective means for controlling and preventing further progression of mural thrombi with longer duration in some patients till the cause is identified and rectified. We suggest having a standardized investigation pathway for similar patients and a dedicated registry of these patients to understand the pathophysiology and formulate an excellent plan of management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Machleder HI, Takiff H, Lois JF, Holburt E. Aortic mural thrombus: An occult source of arterial thromboembolism. J Vasc Surg 1986;4:473-8.  Back to cited text no. 1
    
2.
Sohn V, Arthurs Z, Andersen C, Starnes B. Aortic thrombus due to essential thrombocytosis: Strategies for medical and surgical management. Ann Vasc Surg 2008;22:676-80.  Back to cited text no. 2
    
3.
Fayad ZY, Semaan E, Fahoum B, Briggs M, Tortolani A, D'Ayala M. Aortic mural thrombus in the normal or minimally atherosclerotic aorta. Ann Vasc Surg 2013;27:282-90.  Back to cited text no. 3
    
4.
Verma H, Meda N, Vora S, George RK, Tripathi RK. Contemporary management of symptomatic primary aortic mural thrombus. J Vasc Surg 2014;60:1524-34.  Back to cited text no. 4
    
5.
Rai A, Shantata JK, Sagar J. A rare case of mural thrombus in normal descending thoracic aorta with literature review. Surg Med Open Access J 2020;3:SMOAJ.000572.  Back to cited text no. 5
    
6.
Ferner RE, Marcel Levi RS, Aronson JK. Thrombosis in COVID-19: Clinical Outcomes, Biochemical and Pathological Changes, and Treatments. Centre for Evidence-Base Medicine, University of Oxford; 04 June, 2020. Available from: https://www.cebm.net/covid-19/thrombosis-in-covid-19-clinical-outcomes-biochemical-and-pathological-changes-and-treatments/. [Last accessed on 2022 Feb 17].  Back to cited text no. 6
    
7.
Anwar S, Acharya S, Shabih S, Khabut A. Acute limb ischemia in COVID-19 disease: A mysterious coagulopathy. Cureus 2020;12:e9167.  Back to cited text no. 7
    
8.
Nag UP, Greenberg RG, Leraas HJ, Roberts C, Kamyszek R, Tracy E, et al. Risk factors for thrombosis in the neonatal Intensive Care Unit: Analysis of a large national database. Blood 2018;130 Suppl 1:3351.  Back to cited text no. 8
    
9.
Nosan G, Groselj-Grenc M, Paro-Panjan D. Thrombosis in newborns: Experience from 31 cases. Signa Vitae 2012;7:29-32.  Back to cited text no. 9
    
10.
Dieffenbach BV, Nath BD, Tracy ET, Kim HB. Management of symptomatic neonatal aortic thrombosis: When is surgery indicated? J Pediatric Surg Case Rep 2019;47:101247.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]



 

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