Cardiac amyloidosis therapy reference (2026)

Cardiac amyloidosis therapy in 2026 organises around the underlying type.

AL amyloidosis (light chain amyloidosis): treatment is directed at the underlying plasma cell clone producing the abnormal light chains. Daratumumab plus cyclophosphamide-bortezomib-dexamethasone (Dara-CyBorD) is first-line standard. High-dose chemotherapy plus autologous stem cell transplant remains an option in selected fit patients. Isatuximab plus VRd combinations are reading out. Emerging anti-amyloid antibody programs (birtamimab targeting deposited amyloid for clearance, others) are in late-stage trials. Supportive care for cardiac, renal, and other organ involvement.

ATTR amyloidosis (covered in detail in round 8): TTR stabilisers (tafamidis, acoramidis); TTR silencers (vutrisiran with ATTR-CM approval; eplontersen with ATTR-PN approval and CARDIO-TTRansform cardiac readout pending; patisiran is ATTR-PN-only, with FDA rejecting its ATTR-CM application in 2023); and emerging CRISPR-based one-time TTR knock-out programs (NTLA-2001).

The diagnostic-pathway question is shared. Bone scintigraphy combined with monoclonal protein workup distinguishes ATTR from AL amyloidosis. Cardiac MRI with extracellular volume quantification, echocardiography with strain imaging, biomarkers (NT-proBNP, troponin), and (when needed) endomyocardial biopsy are part of the workup. Diagnostic awareness in heart failure clinics and amyloid centres has grown substantially in the past several years and continues to expand.

The care-pathway integration. Multidisciplinary amyloid clinics (cardiology with hematology with nephrology with neurology with appropriate other specialties) deliver the most comprehensive care. Access to such clinics has expanded but remains uneven.