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Prognostic mortality factors in advanced light chain cardiac amyloidosis: A prospective cohort study
Amira Zaroui1,2,3, Mounira Kharoubi1,2,4, Romain Gounot1,5
1French Referral Centre for Cardiac Amyloidosis, GRC Amyloid Research Institute, Amyloidosis Mondor Network, and DHU A-TVB, Henri Mondor Teaching Hospital, APHP, Creteil, France.
Insights
A new staging system, Mondor amyloidosis cardiac staging (MACS), improves risk prediction for severe cardiac AL amyloidosis patients. It uses biomarkers like hsTnT, NT-proBNP, and conjugated bilirubin to stratify mortality risk.
Area of Science:
- Cardiology
- Hematology
- Oncology
Background:
- Predicting mortality in severe cardiac AL amyloidosis is difficult due to high biomarker levels and limited methods for assessing cardiac damage.
- Accurate prognostic stratification is crucial for guiding therapeutic decisions in these high-risk patients.
Purpose of the Study:
- To identify independent predictors of mortality in de novo cardiac AL amyloidosis.
- To develop and validate a novel prognostic staging system for improved risk stratification.
Main Methods:
- Prospective, observational cohort study of 233 de novo cardiac AL amyloidosis patients over 12 years.
- Identification of mortality predictors including high-sensitivity troponin T (hsTnT), NT-proBNP, cardiac output, and conjugated bilirubin.
- Development of the Mondor amyloidosis cardiac staging (MACS) system based on biomarker cut-off values.
Main Results:
- Independent mortality predictors identified: hsTnT, NT-proBNP, cardiac output, and conjugated bilirubin.
- The MACS system stratified patients into four stages with distinct survival outcomes.
- MACS Stage 1 showed not-reached median survival, Stage 2 had 15.2 months, and Stage 3 had 6.6 months.
Conclusions:
- The novel Mondor prognostic staging system (MACS), incorporating conjugated bilirubin, significantly enhances prognostic stratification for severe cardiac AL amyloidosis.
- MACS provides a more refined assessment of mortality risk compared to existing staging systems.
Aims:
Predicting mortality in severe AL cardiac amyloidosis is challenging due to elevated biomarker levels and limited thresholds for stratifying severe cardiac damage.
Methods And Results:
This prospective, observational, cohort study included de novo, confirmed cardiac AL amyloidosis patients at the Henri Mondor National Reference Centre. The goal was to identify predictors of mortality to enhance prognostic stratification and improve informed decision-making regarding therapy. Over the 12-year study period, among the 233 patients included, 133 were NYHA III-IV and 179 Mayo 2004 III. The independent predictors for mortality identified were hsTnT, NT-proBNP, cardiac output, and conjugated bilirubin. A novel prognostic, conditional stratification, Mondor amyloidosis cardiac staging (MACS) was developed with biomarker cut-off values for Stage 1: hsTnT ≤ 107 ng/L and NT-proBNP ≤ 3867 ng/L (n = 77; 33%); for stage 2 NT-proBNP > 3867 ng/L (n = 72; 30%). For stage 3, if troponin >107 ng/L, regardless of NT-proBNP then CB 4 μmol/L, was added (n = 41; 17.5%) and stage 4: CB > 4 μmol/L (n = 43; 18.5%). The median overall survival was 8 months 95% CI [2-24]. At 1 year, 102 (44%) patients died and the Kaplan-Meier median survival with MACS Stage 1 was not reached, while stage 2 was 15.2 months (95% CI [11-18]) and stage 3, 6.6 months (95% CI [1-13]). Notably, among European stage II patients, 17.1%, n = 8 were MACS stage 3 and European stage IIIb 21.4% (n = 23) were MACS stage 4. Importantly, among European stage IIIb patients 42.2% (n = 29) were classified MACS stage 4 and 12.5% n = 9 were only MACS stage 2.
Conclusions:
The Mondor prognostic staging system, including conjugate bilirubin may significantly improve prognostic stratification for patients with severe cardiac amyloidosis.
