Observational studyEuropean heart journal2022
Characteristics and natural history of early-stage cardiac transthyretin amyloidosis.
Observational study in European heart journal, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
29 citing papers in PubMed, 1 synthesis or guideline pooled it, 59 citations in OpenAlex.
- Real-world effectiveness of targeted therapies in ATTR cardiomyopathy: A meta-analysis integrating population-based data.ESC heart failure · 2025Pooled it
- ACO-SWITCH: Rationale and Design of a Phase 4 Study Evaluating Serum Transthyretin in ATTR-CM After Switching from Tafamidis to Acoramidis.Cardiology and therapy · 2026Article
- Contemporary real-world treatment of cardiac transthyretin amyloidosis with tafamidis: a long-term multicentre study.ESC heart failure · 2026Article
- Four Key Challenges After a Decade of Consecutive Paradigm Shifts in Wild-Type Transthyretin Amyloid Cardiomyopathy: From Rarely Found to Never Missed.Circulation reports · 2026Review
- Basal inferoseptal longitudinal strain deformation may indicate early cardiac involvement in wild-type carpal ATTR.ESC heart failure · 2026Article
- Standardizing the Definition of Early Diagnosis in Wild-Type Transthyretin Amyloidosis.JACC. Advances · 2026Article
- Diagnostic, Therapeutic, and Prognostic Implications of Carpal Tunnel Syndrome and Spinal Stenosis in Wild-type ATTR-Cardiomyopathy.JACC. Advances · 2026Article
- Navigating the emerging landscape of asymptomatic ATTR-CM: challenges, opportunities and the path ahead.Future cardiology · 2025Review
- Tracking a patient with no technetium-99m-pyrophosphate myocardial uptake at surgical valve replacement for aortic stenosis.Journal of geriatric cardiology : JGC · 2025Article
- Intercellular propagation of RIPK1/RIPK3 amyloid fibrils.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- Echocardiographic risk stratification in light chain and transthyretin amyloidosis: a meta-analysis.European heart journal open · 2025Article
- Right ventricular to pulmonary artery uncoupling is an early predictor of poor outcome in wild-type transthyretin amyloid cardiomyopathy.The international journal of cardiovascular imaging · 2025Article
- Article
- Clinical Phenotype and Prognosis of Asymptomatic Patients With Transthyretin Cardiac Amyloid Infiltration.JAMA cardiology · 2025Article
- Atrial cardiomyopathy in cardiac amyloidosis: clinical imaging and manifestations.NPJ cardiovascular health · 2025Article
- Early diagnosis, disease stage and prognosis in wild-type transthyretin amyloid cardiomyopathy: The DIAMOND study.ESC heart failure · 2025Article
- Recovery of right ventricular function in patients with transthyretin cardiac amyloidosis after one-year tafamidis administration.The international journal of cardiovascular imaging · 2025Article
- Changes of clinical characteristics, distribution of red flags and prognosis in contemporary patients with wild-type transthyretin amyloidosis cardiomyopathy.Annals of medicine · 2024Article
- Exercise Hemodynamics and Mitochondrial Oxidative Capacity in Disease Stages of Wild-Type Transthyretin Amyloid Cardiomyopathy.Journal of the American Heart Association · 2024Article
- Advancing Transthyretin Amyloidosis Drug Development in an Evolving Treatment Landscape: Amyloidosis Forum Meeting Proceedings.Advances in therapy · 2024Review
Corrections and comments
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Authors and funding
21 authors at 3 institutions in 3 countries.
Funding
Abstract
aimsTransthyretin amyloid cardiomyopathy (ATTR-CM) is increasingly diagnosed at an early stage of the disease natural history, defined as National Amyloidosis Centre (NAC) ATTR Stage I. The natural history of early-stage ATTR-CM remains poorly characterized. METHODS AND
resultsA retrospective multi-centre observational study of 879 patients with ATTR-CM, either wild-type TTR genotype or carrying the p.V142I TTR variant, and NAC ATTR Stage I biomarkers at the time of diagnosis who did not receive disease-modifying therapy for amyloidosis. Disease characteristics at diagnosis that were independently associated with mortality by Cox regression analysis were N-terminal pro-B-type natriuretic peptide (NT-proBNP), TTR genotype, and troponin T. Patients were categorized into NAC ATTR Stage Ia, defined as a furosemide equivalent diuretic requirement of <0.75 mg/kg and an NT-proBNP ≤500 ng/L or ≤1000 ng/L in the presence of atrial fibrillation, and NAC ATTR Stage Ib comprising all remaining Stage I patients. Median estimated survival among the 88% NAC ATTR Stage Ib patients was 75 (95% CI 57-93) months compared with >100 months in the 12% with Stage Ia disease [hazard ratio for death 5.06 (95% confidence interval 1.23-20.87); P = 0.025] despite significant cardiovascular morbidity at the time of diagnosis which increased during follow-up, including among patients diagnosed in NAC ATTR Stage Ia. Estimated survival among UK NAC ATTR Stage Ia patients was comparable to UK general population controls (P = 0.297).
conclusionPatients with NAC ATTR Stage I ATTR-CM can be further stratified according to NT-proBNP concentration and diuretic requirement at diagnosis. Patients with Stage Ia ATTR-CM have significant cardiovascular morbidity despite good short- and mid-term survival.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.