Heart failure from ATTRwt amyloid cardiomyopathy is associated with poor prognosis

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 (

Advanced isolated light chain amyloid cardiomyopathy with negative immunofixation and normal free light chain ratio

“…AL amyloid cardiomyopathy carries the worst prognosis within the spectrum of specific cardiomyopathies. 1 In the vast majority of amyloid cardiomyopathies, the underlying cause is either TTR or AL amyloid fibril deposition. 9 Both AL and TTR amyloid infiltration can be associated with scintigraphic myocardial tracer uptake.…”

The Bioinformatical Identification of Potential Biomarkers in Heart Failure Diagnosis and Treatment

“…Cardiac remodeling is an important pathophysiological process of heart failure, which is related to the interaction of myocardial cell apoptosis, myocardial hypertrophy and myocardial fibrosis [ 19 ]. HF is characterized by a poor prognosis [ 20 ]. The prognostic factors of HF are mainly related to age, gender, physical fitness, etiology, ventricular ejection fraction, and cardiac function [ 21 ].…”

Left ventricular mass index‐to‐QRS‐voltage ratio predicts outcomes in heart failure with preserved ejection fraction

“…8 Thus, the reason for the poor prognosis of HFpEF patients with a high LVMI-to-QRS-voltage ratio may be partially explained by the presence of amyloid infiltration, which contributes to a high risk of mortality and HF rehospitalization in HFpEF. 14 Another reason may be that this index also reflects myocardial fibrosis. Previous studies have reported that, in patients with hypertrophic cardiomyopathy, low QRS voltage is associated with cardiac fibrosis as assessed by magnetic resonance imaging.…”