Prognostic value of left atrial function in systemic light-chain amyloidosis: a cardiac magnetic resonance study

Dania Mohty1, Cyrille Boulogne2, Julien Magne2

  • 1CHU Dupuytren, Limoges, France French National Reference Center for Light chains Amyloidosis and Other Diseases by Monoclonal Immunoglobulin Deposits, Limoges University, Limoges, France dania.mohty@chu-limoges.fr.

Abstract

Insights

Left atrial emptying fraction (LAEF) measured by cardiac magnetic resonance (CMR) is a significant predictor of survival in systemic light-chain amyloidosis (AL). Lower LAEF indicates a higher risk of mortality, highlighting its prognostic value in AL heart disease.

Area of Science:

  • Cardiology
  • Medical Imaging
  • Oncology

Background:

  • Cardiac involvement in systemic light-chain amyloidosis (AL) significantly impacts patient outcomes.
  • The left atrium (LA) is frequently affected by amyloid infiltration, leading to reduced function and enlargement.
  • Left atrial volume (LAV) is a known prognostic marker in other cardiomyopathies, but its role in AL heart disease requires further investigation.

Purpose of the Study:

  • To assess the prognostic significance of maximal left atrial volume (LAV) and total left atrial emptying fraction (LAEF) in patients with systemic AL using cardiac magnetic resonance (CMR).
  • To determine the relationship between LA parameters and disease severity, symptoms, and mortality in AL patients.

Main Methods:

  • Cardiac magnetic resonance (CMR) imaging was performed on 54 consecutive patients with confirmed systemic AL.
  • Maximal and minimal left atrial volumes (LAV) and left atrial emptying fraction (LAEF) were measured.
  • Correlation with Mayo Clinic (MC) stage, New York Heart Association (NYHA) functional class, myocardial late gadolinium enhancement (LGE), and 2-year survival rates was analyzed.

Main Results:

  • Patients with moderate to high cardiac involvement (MC stages II and III) exhibited significantly larger LAV and lower LAEF compared to those with minimal involvement (MC stage I).
  • Lower LAEF was significantly associated with the presence of myocardial late gadolinium enhancement (LGE) and symptomatic disease (NYHA class ≥ II).
  • A two-year survival rate of 37% was observed in patients with LAEF ≤ 16%, compared to 94% in those with LAEF > 16% (P = 0.001).
  • Multivariate analysis confirmed that lower LAEF independently predicted a higher risk of 2-year mortality (HR = 1.08 per 1% decrease, P = 0.003).

Conclusions:

  • Left atrial emptying fraction (LAEF), as assessed by CMR, is a valuable prognostic marker in systemic AL.
  • LAEF is associated with disease severity (MC stage), symptoms (NYHA class), and myocardial damage (LGE) in AL patients.
  • Reduced LAEF is an independent predictor of mortality in systemic AL, underscoring its clinical importance.

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