Defining echocardiographic predictors of outcome in cardiac amyloidosis by subtype

Curr Probl Cardiol. 2024 Sep;49(9):102729. doi: 10.1016/j.cpcardiol.2024.102729. Epub 2024 Jun 29.

Abstract

Background: Current echocardiographic risk factors for prognosis in cardiac amyloidosis (CA) do not distinguish between the two main subtypes: transthyretin cardiomyopathy (TTR) and immunoglobulin light chain cardiomyopathy (AL), each of which require distinct diagnostic and therapeutic approaches. Additionally, only traditional parameters have been studied with little data on advanced techniques. Accordingly, we sought to determine whether differences exist in 2D transthoracic echocardiography (2DE) predictors of survival between the CA subtypes using a comprehensive approach.

Methods: 220 patients (72±12 years) with confirmed CA (AL=89, TTR=131) who underwent 2DE at the time of CA diagnosis were enrolled. Left ventricular (LV) dimensions, indexed mass (LVMi), global longitudinal strain (LVGLS), apical-sparing ratio (LVASR), diastology, right ventricular (RV) size and function indices including tricuspid annular systolic excursion (TAPSE), RV free-wall (RVFWS) and global (RVGLS) strain, indexed left (LA) and right atrial volumes (LAVi and RAVi), LA strain (reservoir and booster) and RV systolic pressure (RVSP) were measured. A propensity-score weighted stepwise variable selection Cox proportional hazards model derived from NYHA class and renal impairment status at diagnosis was used to determine the associations between 2DE parameters and mortality specific to CA subtype over a median follow-up of 36-months.

Results: After adjusting for age, atrial fibrillation and treatment, parameters associated with survival were RVFWS (p=0.003, HR 1.15, 95% CI[1.053,1.245]) and RVSP (p=0.03, HR 1.03, 95% CI[1.004,1.063]) in AL and LVASR (p=0.007, HR 6.68, 95% CI[1.75,25.492]) and RAVi (p=0.049, HR 1.03, 95% CI[1.000,1.052]) in TTR.

Conclusions: Echocardiographic prognosticators for survival are specific to cardiac amyloid subtype. These results potentially provide information critical for clinical decision-making and follow-up in these patients.

Keywords: Apical sparing pattern; Light chain amyloidosis; Longitudinal strain; Prognosis; TTR amyloidosis.

Publication types

  • Review

MeSH terms

  • Aged
  • Aged, 80 and over
  • Amyloid Neuropathies, Familial / diagnosis
  • Amyloid Neuropathies, Familial / diagnostic imaging
  • Amyloid Neuropathies, Familial / mortality
  • Amyloid Neuropathies, Familial / physiopathology
  • Amyloidosis / diagnosis
  • Amyloidosis / diagnostic imaging
  • Amyloidosis / physiopathology
  • Cardiomyopathies* / diagnosis
  • Cardiomyopathies* / diagnostic imaging
  • Cardiomyopathies* / physiopathology
  • Echocardiography / methods
  • Female
  • Heart Ventricles / diagnostic imaging
  • Heart Ventricles / physiopathology
  • Humans
  • Immunoglobulin Light-chain Amyloidosis* / diagnosis
  • Immunoglobulin Light-chain Amyloidosis* / diagnostic imaging
  • Immunoglobulin Light-chain Amyloidosis* / mortality
  • Immunoglobulin Light-chain Amyloidosis* / physiopathology
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Prognosis
  • Retrospective Studies
  • Risk Factors
  • Survival Rate / trends
  • Ventricular Function, Left / physiology

Supplementary concepts

  • Amyloidosis, Hereditary, Transthyretin-Related