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Published on: August 24, 2019
Amyloidosis and Thoracic Aortic Disease: A Scoping Review
Vasiliki Androutsopoulou1, Konstantinos Spanos2, Ioanna Giannouka3
1Department of Cardiothoracic Surgery, University Hospital of Larissa, Faculty of Medicine, School of Health Sciences, University of Thessaly, 41222 Larissa, Greece.
Amyloidosis can affect the thoracic aorta, causing aneurysms and dissections. Early recognition and multidisciplinary care are crucial for managing these rare but serious complications.
Area of Science:
- Cardiology
- Vascular Surgery
- Hematology
Background:
- Amyloidosis involves protein fibril deposition, commonly light-chain (AL) or transthyretin-derived (ATTR) amyloidosis.
- Cardiac involvement is recognized, but thoracic aortic complications like aneurysms and dissections are rare and under-reported.
Purpose of the Study:
- To review the pathophysiology, diagnosis, and treatment of thoracic aortic diseases related to amyloidosis.
Main Methods:
- Scoping review following PRISMA-ScR guidelines.
- Extensive literature search (2000-2025) in three databases using keywords: amyloidosis, thoracic aorta, aneurysm, dissection, aortopathy.
- Included studies on pathophysiology, diagnosis, clinical manifestations, treatment, and prognosis.
Main Results:
- Twenty-nine studies were included, primarily case reports and autopsy studies.
- Amyloid infiltration disrupts aortic wall architecture, leading to degeneration, impaired perfusion, and arterial stiffness, contributing to aneurysms and lesions.
- Management involves targeted therapies (e.g., CyBorD, Daratumumab) and supportive care; acute large-vessel management follows standard protocols, but prognosis is influenced by systemic disease.
Conclusions:
- Thoracic aortic amyloidosis requires standard surveillance and surgical criteria, with increased operative risk.
- Multidisciplinary care, early detection, and individualized risk stratification are vital for improving outcomes, especially with concurrent cardiac involvement.
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