Abstract
Cardiac amyloidosis, as the extracellular deposition of amyloid proteins in the cardiac tissue, is associated with a high risk for thromboembolic events, with the highest risk being in those with wild-type transthyretin amyloidosis, followed by variant transthyretin and light-chain amyloidosis. The CHA2DS2-VASc score, as used traditionally to guide anticoagulation in atrial fibrillation, has been shown to be untrustworthy in this cohort as it underestimates the true thromboembolic risk. While direct oral anticoagulants (DOACs) have demonstrated a certain advantage in reducing thrombotic events compared to vitamin K antagonists (VKAs), their efficacy remains less than optimal due to the complex interplay of prothrombotic mechanisms in cardiac amyloidosis.
This article presents a comprehensive literature review of anticoagulation in cardiac amyloidosis, supplemented by a case report of an 80-year-old female with wild-type transthyretin amyloidosis who, under DOAC therapy, developed acute lower limb arterial occlusion. The case reinforces the multifactorial etiology of thrombosis in cardiac amyloidosis, including left atrial enlargement, blood stasis, and amyloid-induced endothelial dysfunction. Postoperative course and challenges in anticoagulant therapy demonstrate the need for patient-specific treatment strategies.
The authors underscore the distinctive thromboembolic mechanisms in transthyretin and light-chain amyloidosis and the limitations of the current therapeutic approaches. The paper concludes by calling for randomized clinical trials to create firm, evidence-based guidelines for anticoagulation therapy in this high-risk population. This research aims to close the knowledge gap and improve clinical outcomes in cardiac amyloidosis patients.
Keywords
- Cardiac Amyloidosis
- Thromboembolism
- Direct Oral Anticoagulants (DOACs)
- VitaminK Antagonists (VKAs)
- Atrial Remodeling
- Anticoagulation Therapy
- Amyloid Cardiomyopathy
- Endothelial Dysfunction