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Left atrial function in cardiac amyloidosis.

Gianluca Di Bella1, Fabio Minutoli, Antonio Madaffari

  • 1aClinical and Experimental Department of Medicine bDepartment of Biomedical Sciences and of Morphologic and Functional Images cDepartment of Neurosciences, Psychiatry and Anaesthesiology, University of Messina, Messina dDepartment of Cardiac MRI, Fondazione CNR-Regione Toscana 'G. Monasterio,' Pisa eInstitute of Clinical Physiology, CNR Pisa fEsaote, Florence, Italy.

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Summary

Familial amyloid polyneuropathy (FAP) patients with atrial amyloidosis show impaired left atrium function. This dysfunction is linked to adverse cardiac remodeling, suggesting left atrium assessment is vital for FAP patient stratification.

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Area of Science:

  • Cardiology
  • Medical Imaging
  • Nephrology

Background:

  • Left atrium involvement is observed in familial amyloid polyneuropathy (FAP).
  • Atrial amyloidosis can lead to significant cardiac complications.
  • Assessing left atrium function is crucial for understanding FAP progression.

Purpose of the Study:

  • To evaluate left atrium function in patients with familial amyloid polyneuropathy (FAP) and atrial amyloidosis.
  • To correlate left atrium amyloid deposition with functional changes using strain echocardiography and cardiac magnetic resonance (CMR).

Main Methods:

  • Strain echocardiography and CMR were performed on 28 FAP patients and 22 controls.
  • Late gadolinium enhancement (LGE) on CMR assessed left atrium amyloid deposition.
  • Atrial longitudinal strain (ALS) parameters, including peak-ALS, early-ALS, and prec-ALS, were quantified.

Main Results:

  • FAP patients with atrial LGE exhibited significantly lower peak-ALS, early-ALS, and prec-ALS compared to controls and FAP patients without LGE.
  • Late-ALS was higher in the no-LGE-atrial group compared to controls.
  • These findings indicate impaired left atrium contractility and reservoir function in atrial amyloidosis.

Conclusions:

  • Patients with atrial amyloidosis demonstrate adverse left atrium remodeling and dysfunction.
  • Left atrium assessment using strain echocardiography and CMR provides valuable insights.
  • These assessments aid in the clinical and prognostic stratification of patients with amyloidosis.