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Prognostic value of depressed midwall systolic function in cardiac light-chain amyloidosis

Stefano Perlini1, Francesco Salinaro, Francesco Musca

  • 1aDepartment of Internal Medicine bAmyloidosis Research and Treatment Center and Department of Molecular Medicine, Fondazione IRCCS San Matteo, University of Pavia, Pavia cIntensive Cardiac Care Unit, Heart and Vessel Department dInternal Medicine Department, University of Florence, Florence eInstitute of Cardiology, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy.

Journal of Hypertension
|February 11, 2014
PubMed

Insights

Midwall fractional shortening (mFS) is a key indicator of survival in patients with cardiac light-chain amyloidosis and preserved ejection fraction. Lower mFS values predict poorer outcomes, highlighting its prognostic significance in this condition.

Area of Science:

  • Cardiology
  • Cardiovascular Imaging
  • Amyloidosis Research

Background:

  • Cardiac amyloidosis causes restrictive heart disease and diastolic dysfunction.
  • Many patients present with heart failure with preserved ejection fraction (HFpEF).
  • Midwall fractional shortening (mFS) is a prognostic factor in pressure-overload hypertrophy HFpEF.

Purpose of the Study:

  • To evaluate the prognostic value of mFS in cardiac light-chain amyloidosis (AL) with preserved ejection fraction (PFE).
  • To identify predictors of survival in untreated AL patients with PFE.

Main Methods:

  • 221 consecutive untreated AL patients diagnosed between 2008-2010 were enrolled.
  • 181 patients had HFpEF; 121 served as controls without cardiac involvement.
  • Prognosis was assessed after a median follow-up of 561 days.

Main Results:

  • Cardiac AL patients showed increased wall thickness, reduced LV volumes, and diastolic dysfunction.
  • mFS was significantly depressed in AL patients with PFE compared to controls.
  • Multivariable analysis identified mFS, troponin I, and NT-pro-BNP as significant prognostic determinants.

Conclusions:

  • Depressed mFS is a powerful predictor of survival in cardiac AL with normal ejection fraction.
  • mFS serves as a marker of myocardial contractile dysfunction in this population.
  • mFS is a more significant prognostic factor than other diastolic or systolic function indices.
Abstract

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