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Publication Detail
Characteristics and natural history of early-stage cardiac transthyretin amyloidosis.
  • Publication Type:
    Journal article
  • Publication Sub Type:
    Article
  • Authors:
    Law S, Bezard M, Petrie A, Chacko L, Cohen OC, Ravichandran S, Ogunbiyi O, Kharoubi M, Ganeshananthan S, Ganeshananthan S, Gilbertson JA, Rowczenio D, Wechalekar A, Martinez-Naharro A, Lachmann HJ, Whelan CJ, Hutt DF, Hawkins PN, Damy T, Fontana M, Gillmore JD
  • Publication date:
    24/05/2022
  • Journal:
    Eur Heart J
  • Status:
    Published online
  • Country:
    England
  • PII:
    6591080
  • Language:
    eng
  • Keywords:
    Amyloid, Amyloidosis, Cardiomyopathy, Staging, TTR, Transthyretin
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.
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