Heart failure & cardiomyopathiesCohort study

Prevalence and Prognostic Significance of Restriction Versus Systolic Dysfunction in Patients With Transthyretin and Light Chain Cardiac Amyloidosis

Circ Heart FailPublished

  • retrospectively analyzed
  • real-world cohort
  • assessed at the time of diagnosis

Authors' conclusion

Restriction was the most common presenting phenotype, while preserved LV function represented approximately one-third. The rate of progression from preserved LV function towards restriction was high, whereas it was limited towards systolic dysfunction. Although patients with preserved LV function presented the best event-free survival, considering all-cause mortality and heart transplantation, compared with restriction or systolic dysfunction, these phenotypes are not independent predictors of this composite end point.

Study context

Population
Patients with transthyretin (TTR) and light chain (AL) cardiac amyloidosis (CA)TTR-CA 540 · AL-CA 280
Exposure
Left ventricular (LV) systolic dysfunction versus restriction

Main finding

As reported in the abstract

Prevalence of LV phenotypes, TTR-CA · at the time of diagnosis

Observational data: associations, not effects.

Further findings

Freedom from the composite end point of all-cause mortality and heart transplantation, TTR-CA · 3-year

Prevalence of LV phenotypes, AL-CA · at the time of diagnosis

Freedom from the composite end point of all-cause mortality and heart transplantation, AL-CA · 3-year

Conversion rate among patients with preserved LV function, TTR-CA · at the last evaluation

to restriction
16.3%
to systolic dysfunction
1.8%

Conversion rate among patients with preserved LV function, AL-CA · at the last evaluation

to restriction
12.9%
to systolic dysfunction
none

Original publication

Prevalence and Prognostic Significance of Restriction Versus Systolic Dysfunction in Patients With Transthyretin and Light Chain Cardiac Amyloidosis. Circ Heart Fail. Published 24 February 2026. doi:10.1161/circheartfailure.125.012337

Summarised from the published abstract; every number is checked against it