Heart failure & cardiomyopathiesCohort study

Cardiopulmonary Exercise Testing in Evaluating Transthyretin Amyloidosis

JAMA CardiolPublished

  • Cohort study
  • single-centre

Authors' conclusion

In this study, ATTR amyloidosis was characterized by distinct patterns of functional impairment between all disease phenotypes. A high prevalence of chronotropic incompetence, EOV, and ventilatory inefficiency were characteristic of this population. CPET parameters were associated with amyloid burden by CMR and with peak VO2, and SBP, which have been shown to be independent predictors of mortality.

Study context

Population
Patients with ATTR amyloidosis undergoing cardiopulmonary exercise testing (CPET) at the National Amyloidosis Centre, May 2019 to September 20221,045 approached
Participants
506 patients
Exposure
CPET parameters across ATTR amyloidosis phenotypes and amyloid burden
Outcome
CPET parameters across disease phenotypes, by amyloid infiltration (CMR with extracellular volume mapping), and association of CPET parameters with prognosis
Follow-up
Mean (SD) 22.4 (11.6) months

Main finding

As reported in the abstract

Peak VO2
GroupValueVentilatory efficiency/VCO2 slope, mean (SD)
ATTR-CMmean 14.5 (SD 4.3) mL/kg/min38.1 (8.6)
ATTR-mixedmean 15.7 (SD 6.2) mL/kg/min

Observational data: associations, not effects.

Further findings

Prognosis (multivariable Cox regression)

ComparisonHR (95% CI)P value
Peak VO2 · independently associated with prognosis0.89 (0.81–0.99)P = 0.03
Peak SBP · independently associated with prognosis0.98 (0.97–0.99)P < 0.001

Original publication

Cardiopulmonary Exercise Testing in Evaluating Transthyretin Amyloidosis. JAMA Cardiol. Published 1 April 2024. doi:10.1001/jamacardio.2024.0022

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