A Changing Landscape of Mortality for Systemic Light Chain Amyloidosis

Barrett, Christopher D.; Dobos, Katharine; Liedtke, Michaela; Tuzovic, Mirela; Haddad, François; Kobayashi, Yukari; Lafayette, Richard A.; Fowler, Michael B.; Arai, Sally; Schrier, Stanley L.; Witteles, Ronald

doi:10.1016/j.jchf.2019.07.007

Cited by 39 publications

(32 citation statements)

References 24 publications

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“…-2016 period questioned the prognostic validity of the currently adopted cut-off of 180 mg/L for dFLC, stating that baseline dFLC provided prognostic information only at lower values, namely ≥50 mg/L. 93 Dittrich et al 92 recently compared the performance of Mayo2004, Mayo3b and Mayo2012 in patients with atrial fibrillation or reduced GFR (<50 mL/min/1.73 m 2 ). The Authors reported that Mayo3b is the best risk stratification score in these clinical settings.…”

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confidence: 99%

Use of biomarkers to diagnose and manage cardiac amyloidosis

Castiglione

Franzini

Aimo

et al. 2021

European J of Heart Fail

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Amyloidoses are characterized by the tissue accumulation of misfolded proteins into insoluble fibrils. The two most common types of systemic amyloidosis result from the deposition of immunoglobulin light chains (AL) and wild-type or variant transthyretin (ATTRwt/ATTRv). Cardiac involvement is the main determinant of outcome in both AL and ATTR, and cardiac amyloidosis (CA) is increasingly recognized as a cause of heart failure. In CA, circulating biomarkers are important diagnostic tools, allow to refine risk stratification at baseline and during follow-up, help to tailor the therapeutic strategy and monitor the response to treatment. Among amyloid precursors, free light chains are established biomarkers in AL amyloidosis, while the plasma transthyretin assay is currently being investigated as a tool for supporting the diagnosis of ATTRv amyloidosis, predicting outcome and monitor response to novel tetramer stabilizers or small interfering RNA drugs in ATTR CA. Natriuretic peptides (NPs) and troponins are consistently elevated in patients with AL and ATTR CA. Plasma NPs, troponins and free light chains hold prognostic significance in AL amyloidosis, and are evaluated for therapy decision-making and follow-up, while the value of NPs and troponins in ATTR is less well established. Biomarkers can be usefully integrated with clinical and imaging variables at all levels of the clinical algorithm of systemic amyloidosis, from screening to diagnosis and prognosis, and treatment tailoring.

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confidence: 99%

Use of biomarkers to diagnose and manage cardiac amyloidosis

Castiglione

Franzini

Aimo

et al. 2021

European J of Heart Fail

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“…In addition, our cohort was in a rather advanced stage of HF, potentially further limiting our outcome analyses. However, the outcome of our cardiac ATTRwt and AL cohorts resemble contemporary CA cohorts from other centers [28,29]. Due to the fact that our study cohort consists only of cardiac ATTRwt and AL patients, the generalizability of our data to other forms of CA, especially hereditary ATTR, is limited.…”

Section: Limitationsmentioning

confidence: 99%

Hemodynamic Profiles and Their Prognostic Relevance in Cardiac Amyloidosis

Duca

Snidat

Binder-Rodriguez

et al. 2020

JCM

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This study sought to characterize cardiac amyloidosis (CA) patients with respect to hemodynamic parameters and asses their prognostic impact in different CA cohorts. Intracardiac and pulmonary arterial pressures (PAPs) are among the strongest predictors of outcomes in patients with heart failure (HF). Despite that, the hemodynamic profiles of patients with CA and their relation to prognosis have rarely been investigated. Invasive hemodynamic, clinical, and laboratory assessment, as well as cardiac magnetic resonance imaging were performed in our CA cohort. A total of 61 patients, 35 (57.4%) with wild-type transthyretin amyloidosis (ATTRwt) and 26 (42.6%) with light-chain amyloidosis (AL) were enrolled. ATTRwt patients had lower N-terminal prohormone of brain natriuretic peptide values and were less frequently in New York Heart Association class ≥ III. Intracardiac and PAPs were elevated, but hemodynamic parameters did not differ between CA groups. Whereas in ATTRwt, the median mean PAP (hazard ratio (HR): 1.130, p = 0.040) and pulmonary vascular resistance (HR: 1.010, p = 0.046) were independent predictors of outcome, no hemodynamic parameter was associated with outcome in the AL group. Cardiac ATTRwt and AL patients feature elevated intracardiac and PAPs and show similar hemodynamic profiles. However, hemodynamic parameters are of greater prognostic relevance in ATTRwt, potentially providing a new therapeutic target.

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“…Cardiac involvement in amyloidosis confers significant morbidity and mortality 13 . This, however, is frequently attributed to rapid progression of AL‐CM 1,6,13,14 despite substantial improvements in light chain‐reducing therapies for AL‐CM during the last two decades 15 . ATTR‐CM patients have a better prognosis than do AL amyloid patients despite the fact that ATTR presents with thicker myocardial walls 1,16 .…”

Section: Introductionmentioning

confidence: 99%

Heart failure from ATTRwt amyloid cardiomyopathy is associated with poor prognosis

et al. 2020

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Aims Amyloid cardiomyopathy is an underappreciated cause of morbidity and mortality. Recent evidence suggests that ATTR wild-type cardiomyopathy (ATTRwt-CM) is probably much more common than widely appreciated. So far, no data are available on comparison of mortality from ATTRwt-CM and other heart failure aetiologies. Methods and results This was a retrospective, observational, cohort study of 2251 patients and their data collected prospectively from May 2000 to June 2018. Long-term mortality was the main outcome measure. Underlying cardiomyopathies were classified as amyloid CM (6.1%) [ATTRwt 3.0%; light-chain amyloidosis (AL) 3.1%], dilated CM (dCMP) (46.4%), ischaemic heart disease (IHD) (24.4%), hypertensive heart disease (HHD) (14.6%), hypertrophic CM (HCM) (5.1%), and valvular heart disease (VHD) (3.4%). Median duration of follow-up was 7.1 years (interquartile range 3.4-11.3). Five-year overall survival in the whole cohort was 80.1%. In multivariate analysis, individuals with amyloid CM were 3.74 times [95% confidence interval (CI) 2.72-5.14; P < 0.001] more likely to die of any reason than were individuals with dCMP. Mortality was higher in AL-CM compared with ATTRwt-CM [hazard ratio (HR) 2.88; 95% CI 1.48-5.58; P = 0.002]. Mortality rates in patients with ATTRwt-CM were higher than in patients with dCMP (

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A Changing Landscape of Mortality for Systemic Light Chain Amyloidosis

Cited by 39 publications

References 24 publications

Use of biomarkers to diagnose and manage cardiac amyloidosis

Use of biomarkers to diagnose and manage cardiac amyloidosis

Hemodynamic Profiles and Their Prognostic Relevance in Cardiac Amyloidosis

Heart failure from ATTRwt amyloid cardiomyopathy is associated with poor prognosis

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