PLoS ONE (Jan 2021)

Wild-type TTR amyloidosis among patients with unexplained heart failure and systolic LV dysfunction.

  • Sorel Goland,
  • Igor Volodarsky,
  • Yacov Fabricant,
  • Shay Livschitz,
  • Sagi Tshori,
  • Valeri Cuciuc,
  • Liaz Zilberman,
  • Irena Fugenfirov,
  • Valeri Meledin,
  • Sara Shimoni,
  • Sagie Josfberg,
  • Jacob George

DOI
https://doi.org/10.1371/journal.pone.0254104
Journal volume & issue
Vol. 16, no. 7
p. e0254104

Abstract

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AimTransthyretin cardiac amyloidosis (ATTR-CA) is an increasingly recognized cause of heart failure (HF) with preserved left ventricular ejection fraction (LVEF), typically presenting as restrictive cardiomyopathy. The potential co-existence of ATTR-CA with systolic heart failure has not been studied. The aim of this study is to describe the prevalence of ATTR-CA and its clinical characteristics in HF patients with reduced LVEF.MethodsPatients with an unexplained cause of LV systolic dysfunction were screened for ATTR-CA by a 99mTc-PYP planar scintigraphy. Patients in whom presence of ≥ 2 uptake was confirmed by SPECT imaging were included. Their clinical, laboratory and echocardiographic data were collected.ResultsOut of 75 patients (mean age 65±12 years, LVEF 35.8±7.9%) included in this study, 7 (9.3%) patients (mean age 75±6 years, LVEF 32.0±8.3%) had ATTR-CA. Patients with ATTR-CA were more symptomatic at diagnosis (NYHA FC 3-4 (86% vs 35% (p = 0.03)) and had a more severe clinical course evident by recurrent hospitalizations for HF, and a need for intravenous diuretic treatment (p = 0.04 and p13 mm (57.1% vs 13.1%, p = 0.02) as compared to HF patients with no ATTR-CA.ConclusionIn our study, a meaningful percentage of patients with unexplained LV dysfunction had a co-existing ATTR-CA indicating that the clinical heterogeneity of ATTR-CA is much broader than previously thought.