Physicians' Academy for Cardiovascular Education

Systematic review of ATTR: cardiac amyloidosis, survival, and effects of treatment

Prevalence and clinical outcomes of transthyretin amyloidosis: a systematic review and meta-analysis

Literature - Antonopoulos AS, Panagiotopoulos I, Kouroutzoglou A, et al. - Eur J Heart Fail. 2022 Sep;24(9):1677-1696. doi: 10.1002/ejhf.2589

Introduction and methods


In transthyretin-mediated (ATTR) amyloidosis, instability of the TTR protein due to genetic or degenerative factors may lead to deposition of TTR monomers in vital organs such as the heart [1]. Currently, systematic evidence on the prevalence and clinical outcomes of this disease is lacking.

Aim of the study

The study aim was 3-fold: (1) to better understand the prevalence of cardiac amyloidosis in various patient subgroups, (2) to provide survival estimates for wild-type ATTR (wtATTR) and different hereditary ATTR (hATTR) genotypes, and (3) to explore the effects of novel ATTR therapeutics on the natural course of disease.


The authors performed a systematic review of the literature published in MEDLINE before 31 December 2021 to investigate the prevalence of cardiac amyloidosis and all-cause mortality (at 1, 2, and 5 years) in ATTR patients. A total of 62 studies (n=277,882 patients) reporting the prevalence of cardiac amyloidosis were identified. Data on ATTR mortality were extracted from 95 studies (n=18,238 ATTR patients). Subgroup analyses were performed for ATTR subtype, hATTR genotypes, and treatment subgroups.

Main results

Cardiac amyloidosis

Clinical outcomes

Effect of treatment


This systematic literature review showed that the prevalence of cardiac amyloidosis was high in patients with unexplained LV hypertrophy or a hypertrophic cardiomyopathy phenotype, HFpEF patients, and elderly with aortic stenosis. The wtATTR subtype was associated with longer survival compared with the hATTR subtype, although all-cause mortality was lower in hATTR patients with a Val30Met mutation (which is associated with early disease onset and late cardiac involvement). The survival rate was also higher in patients receiving novel ATTR therapeutics compared with the natural disease course.


Show references

Find this article online at Eur J Heart Fail.

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