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Kocher, Florian; Kaser, Alex; Escher, Felix; Doerler, Jacob; Zaruba, Marc-Michael; Messner, Moritz; Mussner-Seeber, Christine; Mayr, Agnes; Ulmer, Hanno; Schneiderbauer-Porod, Stephanie; Ebner, Christian and Poelzl, Gerhard (2020): Heart failure from ATTRwt amyloid cardiomyopathy is associated with poor prognosis. In: ESC Heart Failure, Vol. 7, No. 6: pp. 3919-3928

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Abstract

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 (HR 1.96;95% CI 1.24-3.22;P = 0.007), HCM (HR 2.94;95% CI 1.28-6.67;P = 0.011), HHD (HR 2.08;95% CI 1.27-3.45;P = 0.004), VHD (HR 2.38;95% CI 1.30-4.35;P = 0.005), or left ventricular ejection fraction >= 40% (HR 1.99;95% CI 1.12-3.52;P = 0.018). Conclusions: Our study demonstrates that amyloid CM is independently associated with poor survival among patients with various causes of heart failure. ATTRwt-CM had a better long-term prognosis than did AL-CM, but was associated with higher mortality than were dCMP, HCM, HHD, VHD, and heart failure with preserved or mid-range ejection fraction.

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