Background: Cardiac biomarker complete response (CR) is a new concept in light chain (AL) cardiac amyloidosis (CA).
Objective: To characterize patients with AL CA who achieved cardiac biomarker CR, including clinical presentation, treatment, cardiac recovery, and survival.
Methods: This single-center retrospective cohort study included patients diagnosed with AL CA between 2004 and 2023 who met cardiac biomarker response criteria (baseline N-terminal pro–B-type natriuretic peptide [NT-proBNP] >650 pg/mL or B-type natriuretic peptide [BNP] >150 pg/mL). Among these, we identified patients who achieved cardiac biomarker CR, defined as NT-proBNP ≤350 pg/mL or BNP ≤80 pg/mL sustained for at least 12 months. Median follow-up was 9.5 years.
Results: Sixty-three patients achieved cardiac biomarker CR (4.7% of evaluable cases [63/1,342]). This proportion increased in the second period compared with the first (6.4% [38/591] vs 3.3% [25/751]; P < 0.001). Median age was 57 years (Q1–Q3: 49–66), and 63.5% were male. Median baseline difference between involved and uninvolved free light chains was 429 mg/L (Q1–Q3: 143–708), median pretreatment NT-proBNP was 1,977 pg/mL, and 57.1% were cardiac stage II. Hematologic CR preceded cardiac biomarker CR in 76% of patients. Median time to cardiac biomarker CR was 20.6 months. At cardiac biomarker CR, median NT-proBNP was 265 pg/mL (86% reduction from baseline). Echocardiographic parameters improved by the time of cardiac biomarker CR but did not fully normalize in all patients. Cardiac progression occurred in 14% of patients, and 44.4% required subsequent clone-directed therapy. Eight patients died, two from non–AL-related causes. Survival was comparable to that of a matched general U.S. population (P = 0.35).
Conclusions: Cardiac biomarker CR represents the deepest level of cardiac biochemical recovery in AL amyloidosis and is associated with survival similar to the general population. Despite biochemical recovery, structural cardiac abnormalities may persist, underscoring the importance of early diagnosis and timely therapy.
JACC CardioOncology Editor-in-Chief and CME Editor
Bonnie Ky, MD, MSCE, FACC
Authors
Eli Muchtar, MD
Morie A. Gertz, MD
Important Dates
Date of Release: April 21, 2026
Term of Approval/Date of CME/MOC Expiration: April 20, 2027