Heart failure & cardiomyopathies

Definition and Prognosis of Mitral Regurgitation in Transthyretin Amyloid Cardiomyopathy: A Multicenter International Cohort Study

CirculationPublished multicenter cohort study

Authors' conclusion

Quantitative MR thresholds in transthyretin amyloid cardiomyopathy are substantially lower than current guideline cutoffs. A validated, risk-based composite MR grading scheme improves prediction of both mortality and HFH, better reflecting the restrictive low-flow pathophysiology of transthyretin amyloid cardiomyopathy and supporting disease-specific MR grading.

Study context

Population
Patients with newly diagnosed transthyretin amyloid cardiomyopathyderivation 722 · validation 379
Participants
1,101 patients
Exposure
Disease-specific composite Mitral Regurgitation (MR) grading scheme vs American Heart Association/American College of Cardiology, European Society of Cardiology/European Association for Cardiothoracic Surgery, and American Society of Echocardiography definitions
Methodological details8 centers, 4 countries, blinded core laboratory analysis, external validation
  • 8 centers, 4 countries
  • Blinded core laboratory analysis
  • External validation

Main finding

As reported in the abstract

All-cause mortality and HFH · median follow-up of 24.9 months (interquartile range, 12.0-42.0)

Died

314 patients

28.5%

Experienced heart failure hospitalization (HFH)

244

22.2%

Observational data: associations, not effects.

Further findings

MR metrics as predictors of mortality and HFH

All MR metrics independently predicted mortality and HFH
all P <0.05

Spline-derived MR risk thresholds

Intermediate risk: vena contracta width
≥3.0 mm
Intermediate risk: effective regurgitant orifice area
≥0.10 cm 2
Intermediate risk: regurgitant volume
≥15 mL
Intermediate risk: regurgitant fraction
≥20%
High risk: effective regurgitant orifice area
>0.25 cm 2
High risk: regurgitant volume
>38 mL
High risk: regurgitant fraction
>35%

Proportion classified as severe MR

Guideline grading
≈12%-13%
Composite MR grading scheme
21%
Composite scheme provided superior discrimination for clinical outcomes at 12 and 24 months in both the derivation and validation cohort

Composite MR grading scheme, multivariable adjusted

Mortality hazard ratio
1.20 [1.07-1.36]
HFH hazard ratio
1.21 [1.05-1.38]
  • After multivariable adjustment, only the composite MR grading scheme remained strongly predictive

Original publication

Definition and Prognosis of Mitral Regurgitation in Transthyretin Amyloid Cardiomyopathy: A Multicenter International Cohort Study. Circulation. Published 13 October 2026. doi:10.1161/circulationaha.125.079085

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

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