Prognostic mortality factors in advanced light chain cardiac amyloidosis: A prospective cohort study

• Published in: ESC Heart Failure Vol. 11; no. 3; pp. 1707 - 1719
• Main Authors: Zaroui, Amira, Kharoubi, Mounira, Gounot, Romain, Oghina, Silvia, Degoutte, Charlotte, Bezard, Melanie, Galat, Arnault, Guendouz, Soulef, Roulin, Louise, Audard, Vincent, Leroy, Vincent, Teiger, Emmanuel, Poullot, Elsa, Molinier‐Frenkel, Valérie, Le Bras, Fabien, Belhadj, Karim, Bastard, Jean‐Philippe, Fellahi, Soraya, Shourick, Jason, Lemonier, Francois, Damy, Thibaud
• Format: Journal Article
• Language: English
• Published: England John Wiley & Sons, Inc 01.06.2024; John Wiley and Sons Inc; Wiley
• Subjects: Aged; AL amyloidosis; Amyloidosis; Biomarkers - blood; Biopsy; Cardiomyopathies - blood; Cardiomyopathies - diagnosis; Cardiomyopathies - mortality; Cohort analysis; Ethics; Female; Flow velocity; Follow-Up Studies; Heart transplants; Humans; Immunoglobulin Light-chain Amyloidosis - blood; Immunoglobulin Light-chain Amyloidosis - diagnosis; Immunoglobulin Light-chain Amyloidosis - mortality; Light; Magnetic resonance imaging; Male; Medical prognosis; Middle Aged; Mortality; Natriuretic Peptide, Brain - blood; Original; Patients; Peptide Fragments - blood; Predictive biomarkers; Prognosis; Prospective Studies; Staging score; Survival Rate - trends; Ultrasonic imaging; AL amyloidosis; Prognosis; Staging score; Mortality; Predictive biomarkers
• ISSN: 2055-5822; 2055-5822
• DOI: 10.1002/ehf2.14671

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.