Refining Risk Stratification in Light-Chain Cardiac Amyloidosis: A Single-Center Ambispective Cohort Study

Background: Recent advances in disease awareness and diagnostic strategies have led to earlier diagnosis and improved survival in patients with light-chain cardiac amyloidosis (AL-CA). However, currently used prognostic staging systems are based on older cohorts and rely mainly on cardiac biomarkers, without incorporating patient-related clinical parameters. Aims: We sought to evaluate contemporary survival outcomes in patients with AL cardiac amyloidosis and to identify clinically available predictors that may improve current risk stratification. Methods: This was a single-center ambispective cohort study including consecutive patients diagnosed with AL cardiac amyloidosis between 2014 and 2024. Results: Ninety-six consecutive patients with AL-CA diagnosed between 2014 and 2024 were included. The mean age was 59.6 ± 11.2 years, and 38 patients (39.6%) were women. During follow-up, 51 patients (53.1%) died. Multivariable Cox regression identified lower systolic blood pressure (SBP) (HR 1.30, 95% CI 1.10–1.54; p = 0.002), troponin levels >3× the upper reference limit (HR 2.67, 95% CI 1.29–5.55; p = 0.008), and advanced New York Heart Association (NYHA) functional class (HR 1.92, 95% CI 1.14–3.24; p = 0.014) as independent predictors of mortality. Adding SBP to the Mayo 2012 staging system was associated with a modest improvement in prognostic discrimination (C-index: 0.685 vs. 0.723; ΔC-index = 0.038) and a modest reduction in prediction error. Conclusions: Lower SBP, marked troponin elevation, and advanced NYHA functional class are independent predictors of mortality in AL-CA. SBP may provide modest incremental prognostic information beyond the Mayo 2012 staging system; however, its added predictive value requires validation in larger independent cohorts.

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Journal
Diagnostics
Published
2026-09-24
DOI
https://doi.org/10.3390/diagnostics16193111
Primary Topic
Amyloidosis: Diagnosis, Treatment, Outcomes
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article
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article

Refining Risk Stratification in Light-Chain Cardiac Amyloidosis: A Single-Center Ambispective Cohort Study

İrem Müge Akbulut, İrem Dinçer, Şeyhmus Atan, Mehmet Emre Özerdem et al.
Diagnostics
Amyloidosis: Diagnosis, Treatment, Outcomes
article

Refining Risk Stratification in Light-Chain Cardiac Amyloidosis: A Single-Center Ambispective Cohort Study

İrem Müge Akbulut, İrem Dinçer, Şeyhmus Atan, Mehmet Emre Özerdem, Sibel Turhan, Güldane Cengiz Seval, Halil Gulyigit, Meral Beksac, Menekse Gerede Uludag
article en

Abstract

Background: Recent advances in disease awareness and diagnostic strategies have led to earlier diagnosis and improved survival in patients with light-chain cardiac amyloidosis (AL-CA). However, currently used prognostic staging systems are based on older cohorts and rely mainly on cardiac biomarkers, without incorporating patient-related clinical parameters. Aims: We sought to evaluate contemporary survival outcomes in patients with AL cardiac amyloidosis and to identify clinically available predictors that may improve current risk stratification. Methods: This was a single-center ambispective cohort study including consecutive patients diagnosed with AL cardiac amyloidosis between 2014 and 2024. Results: Ninety-six consecutive patients with AL-CA diagnosed between 2014 and 2024 were included. The mean age was 59.6 ± 11.2 years, and 38 patients (39.6%) were women. During follow-up, 51 patients (53.1%) died. Multivariable Cox regression identified lower systolic blood pressure (SBP) (HR 1.30, 95% CI 1.10–1.54; p = 0.002), troponin levels >3× the upper reference limit (HR 2.67, 95% CI 1.29–5.55; p = 0.008), and advanced New York Heart Association (NYHA) functional class (HR 1.92, 95% CI 1.14–3.24; p = 0.014) as independent predictors of mortality. Adding SBP to the Mayo 2012 staging system was associated with a modest improvement in prognostic discrimination (C-index: 0.685 vs. 0.723; ΔC-index = 0.038) and a modest reduction in prediction error. Conclusions: Lower SBP, marked troponin elevation, and advanced NYHA functional class are independent predictors of mortality in AL-CA. SBP may provide modest incremental prognostic information beyond the Mayo 2012 staging system; however, its added predictive value requires validation in larger independent cohorts.

DiagnosticsVol. 16(19)
Izmir University (TR), Ankara University (TR), Orthopedic Specialty Hospital (US), Sağlık Bilimleri Üniversitesi (TR), Liv Hospital (TR)
Gender equality
Openalex Percentile: Top 19%
Amyloidosis: Diagnosis, Treatment, Outcomes
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