Cardiac Biomarker Complete Response in AL Amyloidosis: Characteristics, Cardiac Recovery, and Survival of 63 Patients-Article: Cardiac Biomarker Complete Response in AL Amyloidosis: Characteristics, Cardiac Recovery, and Survival of 63 Patients

Article: Cardiac Biomarker Complete Response in AL Amyloidosis: Characteristics, Cardiac Recovery, and Survival of 63 Patients

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This single-center retrospective study examined 63 patients with AL amyloidosis and cardiac involvement who achieved “cardiac biomarker complete response” (CR), defined as sustained NT-proBNP ≤350 pg/mL or BNP ≤80 pg/mL for at least 12 months. Out of 1,342 evaluable patients, only 4.7% reached this deep cardiac biochemical response, though rates increased in the later treatment era.<br /><br />Patients were relatively young (median age 57) and mostly male. Most had early-to-intermediate cardiac disease at diagnosis, and 76% achieved hematologic CR before cardiac biomarker CR. The median time to cardiac biomarker CR was about 21 months. At the time of CR, NT-proBNP had fallen by nearly 87% from baseline, and troponin levels also declined substantially. Echocardiographic measures improved, including strain, septal thickness, and filling parameters, but did not fully normalize in all patients, showing that biochemical recovery does not always mean complete structural cardiac recovery.<br /><br />During long-term follow-up, cardiac progression occurred in 14% of patients, and 44% required additional clone-directed therapy, indicating that relapse remains possible even after cardiac biomarker CR. Temporary rises in natriuretic peptides were common and did not necessarily signal true progression. Despite these issues, survival was excellent: 5-year overall survival was 98% and 10-year survival was 92%, similar to a matched general U.S. population.<br /><br />The study concludes that cardiac biomarker CR represents the deepest biochemical cardiac response in AL amyloidosis and is associated with outstanding long-term survival, but persistent cardiac abnormalities and the need for lifelong monitoring remain important. Early diagnosis and timely, effective clone-directed therapy appear crucial for achieving this favorable outcome.

Keywords

AL amyloidosis

cardiac involvement

cardiac biomarker complete response

NT-proBNP

BNP

hematologic complete response

troponin

echocardiography

overall survival

clone-directed therapy