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Improving prognostic evaluations in patients with stage IIIb light chain cardiac amyloidosis: role of haemodynamic

Jingyi Li1, Yang Lu1, Xiqi Xu1

  • 1Department of Cardiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China.

Orphanet Journal of Rare Diseases
|January 13, 2025
PubMed

Insights

The PAWP/CI ratio is a key prognostic marker for stage IIIb light chain cardiac amyloidosis (AL-CA) patients, predicting mortality better than traditional measures. Echocardiography-derived pulmonary artery systolic pressure may serve as an alternative indicator.

Area of Science:

  • Cardiology
  • Amyloidosis Research
  • Hemodynamics

Background:

  • Light chain cardiac amyloidosis (AL-CA) lacks a unified prognostic system, especially for stage IIIb.
  • Accurate prognosis is crucial for managing advanced AL-CA.

Purpose of the Study:

  • To identify invasive hemodynamic markers for improved prognosis evaluation in stage IIIb AL-CA.
  • To assess the prognostic value of hemodynamic parameters and their correlation with echocardiographic findings.

Main Methods:

  • Retrospective cohort study involving invasive hemodynamic measurements and myocardial biopsies.
  • Cox regression and time-dependent ROC curve analysis for mortality prediction.
  • Logistic regression to correlate echocardiographic parameters with hemodynamic changes.

Main Results:

  • Traditional hemodynamic parameters (CI, PAWP, PAP, VR) did not correlate with mortality.
  • The PAWP/CI ratio emerged as a significant prognostic marker; a ratio >11 mmHg/L/min/m² indicated poorer survival.
  • Echocardiographically measured pulmonary artery systolic pressure (PASP) independently correlated with increased PAWP/CI ratio.

Conclusions:

  • The PAWP/CI ratio is a superior predictor of all-cause mortality in stage IIIb AL-CA compared to traditional hemodynamic indicators.
  • Echocardiography-derived PASP may offer a non-invasive alternative for reflecting the PAWP/CI ratio and assessing prognosis.
Abstract

Keywords:
EchocardiographyHaemodynamic parametersHeart failureLight chain cardiac amyloidosisSurvival analysis

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