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Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
06:33

Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis

Published on: June 9, 2018

Focus on cardiac amyloidosis: a single-center experience with a long-term follow-up

Gherardo Finocchiaro1, Bruno Pinamonti, Marco Merlo

  • 1Cardiovascular Department, 'Ospedali Riuniti' and University of Trieste, Trieste, Italy. gherardobis@yahoo.it

Journal of Cardiovascular Medicine (Hagerstown, Md.)
|May 22, 2012
PubMed

Insights

Cardiac amyloidosis significantly impacts survival, with 50% of patients dying from cardiac causes within a median of 9.5 months. Clinical evaluation, particularly heart failure and syncope, helps predict cardiac mortality risk in these patients.

Area of Science:

  • Cardiology
  • Cardiovascular Diseases
  • Amyloidosis Research

Background:

  • Cardiac amyloidosis is a systemic disease with frequent heart involvement, often leading to poor outcomes.
  • Noninvasive tools like clinical evaluation, echocardiography, and electrocardiography are crucial for detecting cardiac involvement.

Purpose of the Study:

  • To assess clinical-laboratory features of patients with cardiac amyloidosis.
  • To evaluate the risk of cardiac mortality in patients diagnosed with cardiac amyloidosis.

Main Methods:

  • Evaluation of 48 patients with biopsy-proven amyloidosis and heart involvement.
  • Clinical-laboratory assessment at baseline and follow-up.
  • Multivariable Cox regression analysis to identify predictors of cardiac death.

Main Results:

  • 50% of patients died from cardiac causes during a median follow-up of 9.5 months.
  • Heart failure at enrollment and history of syncope were independent predictors of cardiac death.
  • Survival rates free from cardiac death were 41% at 60 months.

Conclusions:

  • Cardiac amyloidosis is associated with a poor short-term prognosis.
  • Careful clinical evaluation is essential for prognostic stratification and risk quantification in cardiac amyloidosis.
Abstract

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