Article Dans Une Revue European Heart Journal Année : 2022

Characteristics and natural history of early-stage cardiac transthyretin amyloidosis

Steven Law
Aviva Petrie
Liza Chacko
Oliver Cohen
  • Fonction : Auteur
Sriram Ravichandran
Olabisi Ogunbiyi
Sashiananthan Ganeshananthan
Sharmananthan Ganeshananthan
Janet Gilbertson
  • Fonction : Auteur
Dorota Rowczenio
Ashutosh Wechalekar
Ana Martinez-Naharro
Helen Lachmann
Carol Whelan
David Hutt
  • Fonction : Auteur
Philip Hawkins
Thibaud Damy
Marianna Fontana
Julian Gillmore

Résumé

Abstract Aims Transthyretin 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 results A 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). Conclusion Patients 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.

Dates et versions

hal-04392211 , version 1 (13-01-2024)

Identifiants

Citer

Steven Law, Melanie Bezard, Aviva Petrie, Liza Chacko, Oliver Cohen, et al.. Characteristics and natural history of early-stage cardiac transthyretin amyloidosis. European Heart Journal, 2022, 43 (27), pp.2622-2632. ⟨10.1093/eurheartj/ehac259⟩. ⟨hal-04392211⟩

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