Cardiac Biomarker Complete Response in AL Amyloidosis | JACC: CardioOncology

0:00Hello, my name is Michael Corey. I am an advanced heart failure cardiologist at Duke University Health System in Journal of Carolina. Today I will be discussing the original research manuscript entitled Cardiac by a Marker Complete Response in ALM oidosis, characteristics cardiac recovery and survival of 63 patients by doctors Mukhtar, Gertz, and colleagues. This is an important retrospective observational study that characterizes the clinical features, treatment outcomes, and survival rates of ALM oidosis patients who achieved a complete cardiac biomarker response to plasma cell directed therapies. ALM oidosis is a plasma cell disorder where cardiac involvement largely determines prognosis. Misfolded immunoglobulin light change formed toxic aggregates that deposit in the heart causing restrictive cardiomyopathy, heart failure, conduction abnormalities, and arrhythmias. In this study, the investigators analyze patients from a single center between 2004 and 2023. Patients with confirmed cardiac involvement

1:01were included in this study if they had elevated natural arctic peptides defined by anti-probe and p greater than 650 or bnp greater than 150, and cardiac biomarker complete response was defined as maintaining an anti-probe bnp less than or equal to 350 or bnp less than or equal to 80 for at least 12 months. Among 1342 AL cardiac amyloidosis patients with elevated natural arctic peptides, 63 or about 5% achieved cardiac biomarker complete response over 20 years. The rate of complete response nearly doubled in the latter half of the study, coinciding with increased use of portesimib and deritumemab based therapies and less frequent stem cell transplantation. Median time to cardiac biomarker complete response was 20 months, and 96% of patients achieved complete response by 60 months. At complete response, median anti-probe and p dropped 86% from baseline, and tropotom levels decreased substantially. Echo cardiography

2:03showed reduced wall thickness and improved myocardial systolic and diastolic performance in disease. Patients with complete response were typically younger, had capa isotope, isotype AL, better kidney function, and were diagnosed earlier. Survival rates were exceptional in the cardiac biomarker complete response cohort. Median survival was over 9 years from treatment start, and estimated 5 and 10 year survival rates were 98% and 92%. Most impressively, overall survival matched the general population after adjusting for age, sex, and treatment initiation year. It is notable though that 14% of patients experienced anti-probe and p progression after 7 and a half years marked by declining ejection fraction, atrial fibrillation, and worsening valve of their disease. The clear takeaway from this study is that patients with cardiac biomarker complete response have survival rates similar to the general population, which is a significant milestone in AL cardiac amlidosis treatment, and highlights the value of early detection and timely plasma cell

3:07directed therapy. However, even patients with cardiac biomarker complete response require ongoing heart failure of care and are susceptible to future cardiac complications. In order to build on these remarkable survival advancements, the multidisciplinary care, particularly between hematologists and cardiologists, that has been at its foundation needs to continue now into long-term survival.