ArticleOpen heart2023
Prognosis of light chain amyloidosis: a multivariable analysis for survival prediction in patients with cardiac involvement proven by endomyocardial biopsy.
Article in Open heart, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed.
- Incremental prognostic utility of congestion markers in cardiac transthyretin amyloidosis.Clinical research in cardiology : official journal of the German Cardiac Society · 2026Article
- Rare case of young cardiac amyloidosis: difficult diagnostic pathway in routine clinical care.European heart journal. Case reports · 2026Article
- Clinical characteristics and prognosis of immunoglobulin light chain amyloidosis patients.Frontiers in medicine · 2026Article
- Multimodality Imaging in Cardiac Amyloidosis.Journal of clinical medicine · 2025Review
- Prevalence of Autonomic Dysfunction and Correlation with Markers of Disease Severity in Cardiac Amyloidosis.Journal of clinical medicine · 2025Article
- Echocardiographic risk stratification in light chain and transthyretin amyloidosis: a meta-analysis.European heart journal open · 2025Article
- The Prognostic Value of Platelet-to-Lymphocyte Ratio and Tricuspid Regurgitation Velocity in Patients with Light-Chain Myocardial Amyloidosis.Journal of inflammation research · 2025Article
- Functional Status and Quality of Life in Light-Chain Amyloidosis: Advanced Imaging, Longitudinal Changes, and Outcomes.JACC. Heart failure · 2024Article
- Liver stiffness as a prognostic parameter and tool for risk stratification in advanced cardiac transthyretin amyloidosis.Clinical research in cardiology : official journal of the German Cardiac Society · 2024Article
- Prognostic mortality factors in advanced light chain cardiac amyloidosis: A prospective cohort study.ESC heart failure · 2024Observational
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Abstract
backgroundCardiac involvement is a main determinant of mortality in light chain (AL) amyloidosis but data on survival of patients with cardiac AL amyloidosis proven by endomyocardial biopsy (EMB) are sparse.
methodsThis study analysed clinical, laboratory, electrocardiography and echocardiographic parameters for their prognostic value in the assessment of patients with AL amyloidosis and cardiac involvement. Patients with AL amyloidosis who had their first visit to the amyloidosis centre at the University Hospital Heidelberg between 2006 and 2017 (n=1628) were filtered for cardiac involvement proven by EMB. In the final cohort, mortality-associated markers were analysed by univariate and multivariable Cox regression. Cut-off values for each parameter were calculated using the survival time.
resultsOne-hundred and seventy-four patients could be identified. Median overall survival time was 1.5 years and median follow-up time was 5.2 years. At the end of the investigation period, 115 patients had died. In multivariable analysis, New York Heart Association-functional class >II (HR 1.65; 95% CI 1.09 to 2.50; p=0.019), left ventricular global longitudinal strain (HR 1.12; 95% CI 1.03 to 1.22; p=0.007), left ventricular end-systolic volume (HR 1.02; 95% CI 1.01 to 1.03; p=0.001), systolic pulmonary artery pressure (HR 0.98; 95% CI 0.96 to 0.99; p=0.027), N-terminal pro-B-type natriuretic peptide (HR 1.57; 95% CI 1.17 to 2.11; p=0.003) and difference in free light chains (HR 1.30; 95% CI 1.05 to 1.62; p=0.017) were independently predictive.
conclusionAmong all patients with AL amyloidosis those with cardiac involvement represent a high-risk population with limited therapy options. Therefore, accurate risk stratification is necessary to identify cardiac amyloidosis patients with favourable prognosis. Incorporation of modern imaging techniques into existing or newly developed scoring systems is a promising option that might enable the implementation of risk-adapted therapeutic strategies.
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