Predictors of Mortality in Light Chain Cardiac Amyloidosis with Heart Failure

Abstract

Cardiac involvement in systemic amyloidosis (AL) occurs in ~50% of all AL patients. However once symptomatic heart failure develops, therapeutic options are limited thereby conferring a poor overall prognosis. The median survival is <6 months when AL patients are untreated for the underlying plasma cell dyscrasia. We thus sought to identify risk factors of increased mortality in treatment - naïve , AL cardiac amyloidosis with heart failure. Patients with biopsy-proven AL cardiac amyloid, who presented with heart failure and did not received prior AL treatment, were enrolled between 2004–2014, at the initial visit to the Amyloidosis Center at Boston University Medical Center. Routine laboratory tests, physical examination and echocardiography data were collected. There were 165 predominantly white (76.4%), and male (61%) patients, with a mean age of 61.6 ± 9.5 years. Median survival was 10.9 months (95% CI 6.2–14.7). By multivariate analysis increased relative wall thickness (RWT) [HR 6.70; 95% CI 2.45–18.30), older age (HR 1.04; 95% CI 1.01–1.06), higher New York Heart Association (NYHA) functional class (HR 1.50; 95% CI 1.02–2.2), log brain natriuretic peptide (BNP) levels (HR 1.45; 95% CI 1.15–1.81) and C-reactive protein (CRP) levels (HR 1.02; 95% CI 1.00–1.04) were significant predictors for increased mortality. In conclusion, in treatment-naïve, AL cardiac amyloidosis patients with heart failure symptoms who lack these high-risk features may have a better outcome. These findings might allow for better risk stratification although outcomes are still poor.

Chronic Diarrhea as the Presenting Feature of Amyloidosis with Multiple Myeloma: A Case Report Diagnosed by a Myocardial Biopsy

“…It has been reported that a favorable response rate to bortezomib and dexamethasone as therapy for MM ranges from 66-90% ( 23 ). However, in patients with AL amyloidosis, once symptoms of heart failure occur, the prognosis is dismal with a median survival of <6 months if the patients remain untreated ( 24 ). Moreover, the severe diarrhea did not improve after chemotherapy initiation in the present case.…”

Body Mass Index and Treatment Response in Patients with Cardiac Light-Chain Amyloidosis

“…Patients with very advanced cardiac amyloidosis have been reported to have a poor prognosis, with median survival of approximately 6 months and thus, risk stratification remains important for management [27]. In a prior study of 165 patients with AL amyloidosis, increased cardiac wall thickness, older age, higher NYHA class, BNP, and CRP levels were prognostic indicators for increased mortality [28]. Progressive cachexia has been associated with increased morbidity and mortality in cancer patients and in heart failure patients and thus it was hypothesized that patients with increased BMI would be associated with lower mortality as fat reserve may permit a patient to better withstand the toxic side effects of chemotherapy and the metabolic stress of the disease [29][30][31].…”

A simple echocardiographic score to rule out cardiac amyloidosis

“…In agreement with the better feasibility of the AMYLI score, the AL and IWT score could be retrospectively calculated only in 61% and 57% of patients in the original cohort, respectively, and in 75% and 70% of patients in the validation cohort, respectively,. In particular, despite being independently associated to patients' prognosis in AL amyloidosis, 10 RWT represents a quite simplistic parameter of LV geometry, and far more complicated parameters might be needed to track all the different hypertrophic patterns of CA. 11,12 Contrary to the AL and IWT scores, we also not assigned a coefficient to the 2 variables selected, and we tested the same score (RWT*E/e′) in the whole population as well as the haematology and hypertrophy cohort.…”