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University of Lynchburg DMSc Doctoral Project Assignment Repository

University of Lynchburg DMSc Doctoral Project Assignment Repository

Specialty

Cardiology

Abstract

Objective: This review examines recent developments in the diagnosis and treatment of cardiac amyloidosis and evaluates their impact on morbidity and mortality

Methods: A review of the current literature was done focusing on recent advancements and algorithmic approaches to diagnosis, including echocardiography with global longitudinal strain, cardiac magnetic resonance imaging, bone scintigraphy with 99m-technetium pyrophosphate, and serum and urine biomarkers. Therapeutic strategies such as proteasome inhibitors were evaluated for their use in AL amyloidosis. Therapeutic strategies such as transthyretin stabilizers and gene silencers were assessed for their use in ATTR amyloidosis.

Results: Diagnostic algorithms and advances in cardiac imaging have improved the ability to detect cardiac amyloidosis earlier, along with differentiating AL and ATTR subtypes, thus reducing reliance on cardiac biopsy. Proteasome inhibitor based regimens have significantly improved survival in AL amyloidosis. Transthyretin stabilizers and gene silencers have been shown to slow disease progression and significantly reduce mortality in ATTR amyloidosis.

Conclusion: The recent advances in noninvasive diagnosis and disease-modifying therapy have taken cardiac amyloidosis and transitioned it from a fatal and underrecognized condition into a treatable cardiomyopathy. Earlier recognition remains the key determinant of outcome.

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