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Cardiac Amyloidosis: Multimodal Imaging of Disease Activity and Response to Treatment
Rishi K Patel1, Marianna Fontana1, Frederick L Ruberg2
1National Amyloidosis Centre, University College London, Royal Free Campus, United Kingdom (R.K.P., M.F.).
Insights
Cardiac amyloidosis (CA) involves protein buildup in the heart. Advanced imaging and therapies are crucial for diagnosing, prognosing, and treating transthyretin and light chain amyloidosis, improving patient outcomes.
Area of Science:
- Cardiology
- Medical Imaging
- Biochemistry
Background:
- Cardiac amyloidosis (CA) is a progressive condition caused by misfolded protein deposition in the heart muscle.
- Clinical presentation and severity of CA vary significantly among patients.
- Understanding CA pathogenesis is vital for improving patient outcomes.
Purpose of the Study:
- To review current therapies for transthyretin and light chain amyloidosis.
- To outline the role of imaging modalities in diagnosing and managing CA.
- To explore how advances in therapeutics and imaging impact future research.
Main Methods:
- Review of contemporary therapies for transthyretin and light chain amyloidosis.
- Analysis of echocardiography, cardiac magnetic resonance, and bone scintigraphy in CA assessment.
- Examination of the interplay between therapeutic and imaging advancements.
Main Results:
- Multimodal imaging aids in diagnosing CA, informing prognosis, and monitoring treatment response.
- Echocardiography, CMR, and bone scintigraphy assess functional and cellular aspects of CA.
- Concurrent advances in therapies and imaging offer new research avenues.
Conclusions:
- Multimodal imaging is essential for personalized treatment strategies in CA.
- Improved understanding of CA pathogenesis through integrated approaches can enhance patient care.
- Targeted therapies combined with advanced imaging promise better outcomes for CA patients.
Abstract:
Cardiac amyloidosis (CA) is a disease characterized by the deposition of misfolded protein deposits in the myocardial interstitium. Although advanced CA confers significant morbidity and mortality, the magnitude of deposition and ensuing clinical manifestations vary greatly. Thus, an improved understanding of disease pathogenesis at both cellular and functional levels would afford critical insights that may improve outcomes. This review will summarize contemporary therapies for the 2 major types of CA, transthyretin and light chain amyloidosis, and outline the capacity of imaging modalities to both diagnose CA, inform prognosis, and follow response to available therapies. We explore the current landscape of echocardiography, cardiac magnetic resonance, and bone scintigraphy in the assessment of functional and cellular parameters of dysfunction in CA throughout disease pathogenesis. Finally, we examine the impact of concurrent advances in both therapeutics and imaging on future research questions that improve our understanding of underlying disease mechanisms. Multimodal imaging in CA affords an indispensable tool to offer individualized treatment plans and improve outcomes in patients with CA.
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