Authors
Valentina Silvestri, Manuel Toledano, Bénédicte Hivert, Emmanuelle Bourgeois, Benjamin Carpentier, Jean Giraud, Alexandre Willaume, François Delelis, Laurent Pascal
Published in
JACC. Case reports. Pages 109553. Aug 27, 2026. Epub Aug 27, 2026.
Abstract
Cardiac amyloid light-chain (AL) amyloidosis carries a poor prognosis and is considered largely irreversible once symptomatic. Long-term serial cardiovascular magnetic resonance (CMR) data following B-cell maturation antigen-directed therapy are lacking.
A 64-year-old man with biopsy-proven cardiac AL amyloidosis achieved sustained hematologic complete response after teclistamab following failure of 4 prior lines including daratumumab. Eleven CMR examinations over 9 years documented a biphasic trajectory: progressive infiltration through 2020 (left ventricular mass 186 g, wall thickness 18 mm, global longitudinal strain -4.8%), followed by sustained structural and functional regression through 2025 (left ventricular mass 127 g, wall thickness 13 mm, global longitudinal strain -13.7%).
This represents the longest serial CMR follow-up in cardiac AL amyloidosis reported to date, demonstrating that cine-derived parameters reliably track cardiac regression in real-world conditions. Native T1 normalized while extracellular volume fraction (ECV) paradoxically increased, suggesting persistent fibril burden beyond hematologic clearance.
Serial CMR with cine-derived parameters provides robust longitudinal monitoring of cardiac AL amyloidosis regression following novel plasma cell-directed therapy, even without systematic contrast enhancement.
PMID:
42663348
Bibliographic data and abstract were imported from PubMed on 28 Aug 2026.
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