Key result
Teclistamab is linked to sustained AL amyloidosis regression, reducing LV mass by ~59 g.
Why the study?
Long-term serial cardiovascular magnetic resonance data following B-cell maturation antigen-directed therapy in cardiac AL amyloidosis are lacking.
Does teclistamab therapy and subsequent hematologic complete response lead to structural and functional regression in severe cardiac AL amyloidosis as monitored by serial CMR?
Case Report (n=1)
Does teclistamab therapy and subsequent hematologic complete response lead to structural and functional regression in severe cardiac AL amyloidosis as monitored by serial CMR?
Long-term serial CMR demonstrates that novel plasma cell-directed therapies like teclistamab can induce sustained structural and functional regression of previously irreversible cardiac AL amyloidosis.
May support durable cardiac response to teclistamab in AL amyloidosis; leaves open confirmation in prospective trials.
BACKGROUND: 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. CASE SUMMARY: 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%). DISCUSSION: 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. TAKE-HOME MESSAGE: 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.
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Silvestri et al. (2026) conducted a case report in Cardiac AL amyloidosis (n=1). Teclistamab therapy and serial CMR monitoring was evaluated on Cardiac structural and functional regression (left ventricular mass, wall thickness, and global longitudinal strain). Serial CMR monitoring over 9 years documented sustained structural and functional regression of cardiac AL amyloidosis after teclistamab, with LV mass decreasing from 186 g to 127 g.
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