Abstract
Coarctation of the aorta and aortic thrombus coexistence is extremely rare. This case series presents 2 adult patients with diverse presentations of coarctation of the aorta complicated by aortic thrombus characterized by cardiac computed tomography angiography.
Key words: adult, aortic coarctation, aortic thrombus, computed tomography angiography
Graphical Abstract
Coarctation of the aorta (CoA) accounts for 6% to 8% of congenital heart diseases (CHDs),1 with favorable long-term survival rates in patients who reach adulthood.2 Despite this, long-term morbidity is common, primarily due to aortic complications and hypertension.2
Aortic rupture/dissection, premature coronary/cerebral artery disease, infective endocarditis, intracranial hemorrhage, left heart failure, and associated CHDs could complicate CoA.2 Aortic thrombus (AoT) is usually associated with an underlying aortic pathology,3,4 although CoA and AoT coexistence is extremely rare.5,6
In this case series, we present 2 adult cases of CoA complicated with AoT, highlighting the crucial role of cardiac computed tomography angiography (CTA) in the diagnosis, management, and follow-up of these conditions.
Case 1
A 27-year-old man was admitted with a history of 2 months of progressive dyspnea and malaise. The patient denied a personal and family history of heart disease. The vital signs at admission showed high blood pressure (240/120 mm Hg); heart rate (64 beats/minute), respiratory rate (16 breaths/minute), and Spo2 (94% on room air) were unremarkable. A physical examination revealed a mid-systolic murmur in the aortic area with a systolic gradient of 40 mm Hg between the superior and inferior extremities. The electrocardiogram revealed left ventricular hypertrophy (Figure 1A), and the chest radiograph showed rib notching (Figure 1B). Blood test results were unremarkable. Transthoracic echocardiography (TTE) demonstrated a normal functional tricuspid aortic valve but not a well-defined aortic arch and descending aorta because of poor acoustic window. Aortic CTA showed CoA with an AoT (Figure 2). Associated disease-related hypercoagulable states were ruled out. Infusion of unfractionated heparin and antihypertensive drugs were prescribed, and the patient underwent a stent aortoplasty without incident (Figure 3). The patient was discharged in good health 4 days after the procedure; at the 2-year follow-up, the patient remained symptom-free.
Figure 1.
Patient 1 With Coarctation of the Aorta Complicated by Aortic Thrombus
(A) Initial electrocardiogram: left ventricular hypertrophy. (B) Admission chest x-ray film shows rib notching.
Figure 2.
Patient 1 With Coarctation of the Aorta Complicated by Aortic Thrombus
Aortic CTA showing CoA is 24 mm from the left subclavian vein with an interrupted segment of 11.5 mm in length due to AoT, as well as dilated mammary, epigastric, and intercostal arteries, with multiple thick collateral arteries on the anterior and posterior walls of the thorax. (A, B) Maximum intensity projection imaging. (C) 3D volume rendered image on the anterior view. (D) 3D volume rendered image on the lateral view. 3D = 3-dimensional; AoT = aortic thrombus; CoA = coarctation of the aorta; CTA = computed tomography angiography.
Figure 3.
Patient 1: Stent Aortoplasty
Case 2
A 52-year-old man with a history of hypertension was admitted because of progressive dyspnea. He reported an episode of intense chest pain 24 hours ago, accompanied by diaphoresis. The patient denied a personal and family history of heart disease. The vital signs at admission showed a low Spo2 level (87% on room air); blood pressure (128/81 mm Hg), heart rate (90 beats/minute), and respiratory rate (20 breaths/minute) were unremarkable. The physical examination showed diminished pulses in the inferior extremities and pulmonary crackles. The electrocardiogram revealed a complete left bundle branch block (Figure 4A), and the chest radiograph showed pulmonary congestion (Figure 4B). Blood tests yielded high troponin (4,368 ng/dL) and N-terminal pro–B-type natriuretic peptide levels (6,220 pg/mL), resulting in a diagnosis of acute coronary syndrome complicated by acute heart failure. Prophylactic TTE-guided intra-aortic balloon pump placement was performed, but none passed through the aortic arch. The patient underwent an emergency aortic CTA, which showed a CoA with an AoT (Figure 5). Considering these findings, the patient underwent an emergency stent aortoplasty (Figure 6), after an uneventful intra-aortic balloon pump placement; urgent coronary angiography identified triple-vessel coronary disease, and the stents were successfully implanted. A follow-up aortic CTA showed that the aortic stents were intact and permeable (Figure 7). The patient was discharged in good health 13 days after the procedure; at the 1-year follow-up, the patient remained symptom-free.
Figure 4.
Patient 2 With Coarctation of the Aorta Complicated by Aortic Thrombus
(A) Initial electrocardiogram: complete left bundle branch block. (B) Admission chest x-ray film showing pulmonary congestion.
Figure 5.
Patient 2 With Coarctation of the Aorta Complicated by Aortic Thrombus
Aortic CTA showing CoA is 11.5 mm from the left subclavian vein with an interrupted segment due to AoT, as well as dilated mammary, epigastric, and intercostal arteries with multiple thick collateral arteries. (A, B) Maximum intensity projection imaging. (C) 3D volume rendered image on the anterior view. (D) 3D volume rendered image on the lateral view. Abbreviations as in Figure 2.
Figure 6.
Patient 2: Stent Aortoplasty
Figure 7.
Patient 2 With Coarctation of the Aorta Complicated by Aortic Thrombus
Aortic computed tomography angiography demonstrated an intact, permeable aortic stent. (A, B) Maximum intensity projection imaging. (C) Three-dimensional (3D) volume rendered image on the anterior view. (D) 3D volume rendered image on the lateral view.
Discussion
High pressure and rapid flow inside the aorta limit AoT development and growth.4 The majority of AoT cases are associated with severe aortic atherosclerosis, aneurysms, aortic dissection, and aortitis.4,7 Thrombotic material adhering to the aortic wall is usually associated with atherosclerosis or aneurysms.8
Blood stagnation distal to the stenosed segment may contribute to arterial thrombosis.6 Abnormal-shaped portions of the aorta, where turbulence, eddying, and other abnormal flow states exist, could impact the wall shear stress on the intima and result in local thrombosis. In addition, erratic flow distal to the narrowing part of the aorta strengthens lipid aggregation, which may lead to thrombosis.4 An AoT arising in the context of underlying aortic pathology is classified as secondary, whereas primary AoT develops in a normal or minimally atherosclerotic aorta and is usually pedunculated and protrudes into the aortic lumen.3
The main predisposing factors for AoT include: 1) hypercoagulable states, either genetically or acquired, such as malignancy; and 2) risk factors for vasculopathy, such as smoking, hypertension, obesity, diabetes, and dyslipidemia.9 Primary AoTs are generally idiopathic, although some are associated with hypercoagulable states.3 Further risk factors and causes of AoT include steroid use, trauma, drug abuse, heparin-induced thrombosis, rheumatism, primary endothelial disorders, iatrogenic causes, and vasculitis.8,10
CoA severity determines signs and symptoms; adult patients are diagnosed at arterial hypertension work-ups. In most cases, CoA remains undetected until adulthood, with symptoms associated with pressure gradients in the upper and lower extremities (abdominal angina and claudication).1,2
Radiofemoral pulse delays and weak pulses can also indicate significant CoA. Patients with clinical suspicion must undergo blood pressure measurements in both the lower and upper extremities; a systolic gradient ≥20 mm Hg indicates significant CoA.1,2
In untreated AoT, the natural course is either asymptomatic or symptomatic systemic embolization, multiorgan failure, and eventually death.3,9 In approximately 45% of cases, the initial presentation is claudication symptoms, such as extremity pain, ischemic rest pain, skin discoloration, gangrene, and necrosis. Chest pain, back pain, and dyspnea are less common initial presentations.9
As a rule, blood pressure assessment should be conducted in both arms and lower extremities to exclude a blood pressure differential caused by acute aortic syndrome; aortic murmurs should also be assessed, which may indicate bicuspid aortic valve or aortic regurgitation, which frequently accompany aortic dissections.3
The diagnostic work-up of aortic disease should include a combination of imaging modalities to thoroughly evaluate the heart and entire aorta.7 In determining the initial cardiac imaging modality, consideration should be given to the patient's history and clinical presentation, as well as to the institution's expertise and experience in performing each diagnostic imaging technique.3 AoT diagnosis is often confirmed by transesophageal echocardiography or CCTA; however, cardiac magnetic resonance imaging and angiography can also contribute to the diagnosis.3,7,9 Despite the widespread use of transesophageal echocardiography for the diagnosis of AoT, some parts of the aorta, such as the ascending aorta and upper descending aorta, cannot be visualized and may lead to misdiagnosis of AoT.4
In patients with CoA, cardiac CTA is one of the preferred noninvasive techniques for detecting complications and for surgical planning.2 In terms of anatomical assessment of the entire aorta, cardiac CTA is an excellent imaging technique because it is a noninvasive, 3-dimensional, ionizing-based imaging modality that allows for the acquisition of cardiac images with high spatial resolution. In addition, it is particularly appropriate for acutely ill and functionally impaired patients who cannot remain in the supine position for extended periods, as well as for intubated or sedated patients, neonates, and uncooperative children1 Cardiac CTA is widely available in emergency departments at all hours and can be performed within a short timeframe.3
Although there are specific separate treatment guidelines for CoA2 and AoT3, owing to the rarity of CoA complicated by AoT, there are currently no standardized therapeutic protocols or clinical guidelines.5,6 Depending on the patient's condition and the center's experience, patient treatment strategies may include medical management with anticoagulation along with invasive procedures, such as endovascular approaches or open surgery.4,9,10
After surgical and percutaneous aortic interventions, cardiac CTA plays an important role in identifying residual or recurrent stenosis and detecting complications associated with endovascular stents, including leaks, stent migration, fracture, stent thrombosis, pseudoaneurysm formation, and dissection.1
This case series illustrates 2 adult patients with a relatively common CHD in different clinical settings, both of whom had AoT, an infrequently coexisting disease. As previously discussed, we believe blood stagnation due to CoA contributed to AoT development. Despite the differences in clinical settings, both patients underwent a comprehensive physical examination and an initial TTE. However, because of the inconclusive echocardiography findings, a further cardiac imaging modality was required to make a definitive diagnosis of a suspected aortopathy. Aortic CTA is a valuable tool because it provides accurate visualization of its anatomy at high resolution with rapid acquisition, allowing volumetric reconstruction to enhance structural visualization. Multidisciplinary approaches are crucial for the timely and successful treatment of atypical CoA presentations.
Conclusions
CoA complicated by AoT is a rare and severe condition requiring a multidisciplinary diagnostic approach. Cardiac CTA is an integral part of aortic disease evaluation because it provides precise visualization of the aortic anatomy and thrombus, supports appropriate treatment planning, and serves as a valuable diagnostic tool in complex cases.
Take-Home Messages
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Coarctation of the aorta complicated by an aortic thrombus is a rare and severe condition requiring multidisciplinary diagnostic evaluation.
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Cardiac computed tomography is considered the most effective noninvasive method for detecting aortopathies.
Funding Support and Author Disclosures
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Footnotes
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
References
- 1.Leo I., Sabatino J., Avesani M., et al. Non-invasive imaging assessment in patients with aortic coarctation: a contemporary review. J Clin Med. 2023;13(1):28. doi: 10.3390/jcm13010028. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Baumgartner H., De Backer J., Babu-Narayan S.V., et al. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J. 2021;42(6):563–645. doi: 10.1093/eurheartj/ehaa554. [DOI] [PubMed] [Google Scholar]
- 3.Isselbacher E.M., Preventza O., Hamilton Black J., III, et al. 2022 ACC/AHA guideline for the diagnosis and management of aortic disease: a report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2022;80(24):e223–e393. doi: 10.1016/j.jacc.2022.08.004. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 4.Yang S., Yu J., Zeng W., et al. Aortic floating thrombus detected by computed tomography angiography incidentally: Five cases and a literature review. J Thorac Cardiovasc Surg. 2017;153(4):791–803. doi: 10.1016/j.jtcvs.2016.12.015. [DOI] [PubMed] [Google Scholar]
- 5.Pozzi M., Taylor J., de Leval M.R. Coarctation of the aorta associated with calcific aortic thrombosis in a neonate. Eur J Cardiothorac Surg. 1991;5(10):552–553. doi: 10.1016/1010-7940(91)90111-v. [DOI] [PubMed] [Google Scholar]
- 6.Darwazah A.K., Ibraheem S. Aortic coarctation associated with thrombosis mimicking interrupted arch. Asian Cardiovasc Thorac Ann. 2016;24(8):825–826. doi: 10.1177/0218492316653295. [DOI] [PubMed] [Google Scholar]
- 7.Fayad Z.Y., 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(3):282–290. doi: 10.1016/j.avsg.2012.03.011. [DOI] [PubMed] [Google Scholar]
- 8.Caron F., Anand S.S. Antithrombotic therapy in aortic diseases: a narrative review. Vasc Med. 2017;22(1):57–65. doi: 10.1177/1358863X16675229. [DOI] [PubMed] [Google Scholar]
- 9.Nguyen Q., Ma X., Vervoort D., Luc J.G.Y. Management strategies for descending thoracic aortic thrombus: a review of the literature. Innovations (Phila) 2022;17(4):283–296. doi: 10.1177/15569845221107011. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 10.Meyermann K., Trani J., Caputo F.J., Lombardi J.V. Descending thoracic aortic mural thrombus presentation and treatment strategies. J Vasc Surg. 2017;66(3):931–936. doi: 10.1016/j.jvs.2017.05.109. [DOI] [PubMed] [Google Scholar]