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Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
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Features and evolution of patients with systemic amyloidosis and cardiac involvement

Marcelina Carretero1, Ma A Aguirre2, Eugenia Villanueva3

  • 1Servicio de Clínica Médica, Área de Investigación en Medicina Interna, Hospital Italiano de Buenos Aires, BA, Argentina.

Archivos De Cardiologia De Mexico
|June 29, 2021
PubMed

Insights

Cardiac amyloidosis affects 63% of systemic amyloidosis patients, significantly lowering survival rates. Early identification of heart involvement is crucial for prognosis in systemic amyloidosis.

Area of Science:

  • Cardiology
  • Hematology
  • Internal Medicine

Background:

  • Systemic amyloidosis is a rare disease characterized by amyloid protein deposition in organs.
  • Cardiac involvement is a common complication of systemic amyloidosis, impacting patient outcomes.
  • Understanding the prevalence and prognostic significance of cardiac involvement is vital for patient management.

Purpose of the Study:

  • To determine the prevalence of cardiac amyloidosis in patients diagnosed with systemic amyloidosis.
  • To compare survival rates between patients with and without cardiac involvement.
  • To identify factors influencing mortality in systemic amyloidosis.

Main Methods:

  • Retrospective cohort study utilizing data from the Institutional Amyloidosis Registry (2010-2019).
  • Cardiac involvement defined by symptoms or imaging consistent with amyloidosis, excluding other causes.
  • Survival analysis performed using Kaplan-Meier curves and Cox regression models; heart transplantation assessed via competing risk regression.

Main Results:

  • Prevalence of cardiac involvement was 63% among systemic amyloidosis patients.
  • The 5-year survival rate was significantly lower for patients with heart involvement (44%) compared to those without (67%, p=0.02).
  • Heart involvement (HR=2.09, p=0.02) and age (HRa=1.06, p<0.01) were significant predictors of mortality.

Conclusions:

  • Cardiac involvement is a critical prognostic factor in systemic amyloidosis.
  • Patients with cardiac involvement exhibit significantly poorer survival outcomes.
  • Further research into managing cardiac complications in systemic amyloidosis is warranted.
Abstract

Keywords:
AmyloidosisImmunoglobulin light chain amyloidosisFamilial amyloidosisRestrictive cardiomyopathy

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