Cardiac amyloidosis is an often overlooked and thus misdiagnosed form of a restrictive cardiomyopathy in which delayed diagnosis is associated with significant morbidity and mortality. Immunoglobulin light chain (AL) cardiac amyloidosis is the most deadly from of cardiac amyloidosis but significant progress has been made in treatment with the first FDA approved therapy for this condition - daratumumab.

In this interview, Mathew Maurer, MD and Stephen J. Nicholls, MBBS, PhD, FACC, with Yuvraj Chowdhury, MD, discuss Pathophysiology and Therapeutic Approaches to Cardiac Amyloidosis--Focus on AL.

References:

1. Kastritis E, Palladini G, Minnema MC, et al. Daratumumab-Based Treatment for Immunoglobulin Light-Chain Amyloidosis. N Engl J Med 2021;385:46-58.
2. Muchtar E, Dispenzieri A, Gertz MA. Treatment of AL Amyloidosis: Mayo Stratification of Myeloma and Risk-Adapted Therapy (mSMART) Consensus Statement 2020 Update. Mayo Clin Proc 2021;96:1546-1577.
3. Maurer MS, Elliott P, Comenzo R, Semigran M, and Rapezzi C. Addressing Common Questions Encountered in the Diagnosis and Management of Cardiac Amyloidosis. Circ 2017;135:1357-1377.
4. Griffin JM, Rosenblum H, and Maurer MS. Pathophysiology and Therapeutic Approaches to Cardiac Amyloidosis. Circ Res 2021;128:1554–1575.
5. Bonnefous L, Kharoubi M, Bézard M. Assessing Cardiac Amyloidosis Subtypes by Unsupervised Phenotype Clustering Analysis. J Am Coll Cardiol 2021;78:2177–2192.
6. Ruberg F. Phenotype Mapping in Cardiac Amyloidosis. J Am Coll Cardiol 2021;78:2193–2195.
7. Witteles R and Ky B. Cardiac Amyloidosis: The “Tipping Point” Has Been Reached. J Am Coll Cardiol CardioOnc 2021;3:617–618.
8. Bianchi G, Zhang Y, and Comenzo RL. AL Amyloidosis: Current Chemotherapy and Immune Therapy Treatment Strategies: JACC: CardioOncology State-of-the-Art Review. J Am Coll Cardiol CardioOnc 2021;3:467–487.

Clinical Topics: Heart Failure and Cardiomyopathies

Keywords: ACCELLite, Amyloidosis, Immunoglobulin Light Chains

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