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. 2020 Dec;7(6):3919-3928.
doi: 10.1002/ehf2.12986. Epub 2020 Oct 1.

Heart failure from ATTRwt amyloid cardiomyopathy is associated with poor prognosis

Florian Kocher  1 , Alex Kaser  2 , Felix Escher  3 , Jacob Doerler  2 , Marc-Michael Zaruba  2 , Moritz Messner  2 , Christine Mussner-Seeber  2 , Agnes Mayr  4 , Hanno Ulmer  5 , Stephanie Schneiderbauer-Porod, Christian Ebner  6 , Gerhard Poelzl  1
Affiliations

Heart failure from ATTRwt amyloid cardiomyopathy is associated with poor prognosis

Florian Kocher et al. ESC Heart Fail. 2020 Dec.

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.

Keywords: ATTRwt; Cardiac amyloidosis; Cardiomyopathy; Prognosis.

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Conflict of interest statement

P.G. has received speaker honoraria from Pfizer and AKCEA Therapeutics. All other authors have nothing to disclose.

Figures

Figure 1
Figure 1
Kaplan–Meier estimates of survival adjusted for age, gender, NYHA functional class, and LV‐EF, presented according to the underlying cause of heart failure (A) and the subtypes of amyloid CM (B). AL‐CM, AL cardiomyopathy; Amyloid CM, amyloid cardiomyopathy; ATTRwt‐CM, ATTR wild‐type cardiomyopathy; dCMP, dilated cardiomyopathy; HCM, hypertrophic cardiomyopathy; HHD, hypertensive heart disease; IHD, ischaemic heart disease; VHD, valvular heart disease.
Figure 2
Figure 2
Hazard ratios for all‐cause mortality from univariate analysis for AL‐CM, IHD, VHD, HHD, dCMP, HCM, and HFpEF/HFmrEF versus ATTRwt‐CM (in which the hazard ratio for death was 1.0, per definition). AL‐CM, AL cardiomyopathy; Amyloid CM, amyloid cardiomyopathy; ATTRwt‐CM, ATTR wild‐type cardiomyopathy; dCMP, dilated cardiomyopathy; HCM, hypertrophic cardiomyopathy; HFpEF/HFmrEF, heart failure with preserved and mid‐range ejection fraction; HHD, hypertensive heart disease; IHD, ischaemic heart disease; VHD, valvular heart disease.
See this image and copyright information in PMC

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