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Amyloidosis and cardiac involvement.

P Cacoub1, O Axler, D De Zuttere

  • 1Department of Internal Medicine, La Pitié Hospital, Paris. patrice.cacoub@psl.ap-hop-paris.fr

Annales De Medecine Interne
|February 15, 2001
PubMed
Summary

Cardiac amyloidosis is common, often asymptomatic, and significantly worsens prognosis. Echocardiography is crucial for early detection and management of amyloid heart disease, improving survival outcomes.

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Area of Science:

  • Cardiology
  • Cardiovascular Imaging
  • Amyloidosis Research

Background:

  • Amyloidosis involves extracellular amyloid deposition, with cardiac involvement being a critical prognostic factor.
  • Cardiac manifestations of amyloidosis can be present even without overt clinical symptoms.

Purpose of the Study:

  • To analyze clinical, electrocardiographic, radiological, and echocardiographic features in patients with biopsy-proven amyloidosis and echocardiographic signs of cardiac involvement.
  • To compare survival rates between amyloidosis patients with and without cardiac involvement.

Main Methods:

  • Retrospective analysis of echocardiographic features in 47 patients with biopsy-proven amyloidosis.
  • Comparison of 30 patients with echocardiographic evidence of cardiac amyloidosis against 17 without.

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Main Results:

  • Amyloid heart disease, often AL type, predominantly affected middle-aged men, presenting as congestive heart failure in 59% of cases.
  • Echocardiography revealed diffuse ventricular thickening (70%), restrictive diastolic function (57%), pericardial effusion (40%), and impaired systolic function (27%).
  • Cardiac symptoms developed in 72% of initially asymptomatic patients with echocardiographic findings; cardiac disease caused 76% of deaths, with median survival significantly reduced by cardiac involvement.

Conclusions:

  • Echocardiography is essential for detecting cardiac involvement in amyloidosis, even in asymptomatic individuals.
  • Early detection and management of cardiac amyloidosis are critical, as it significantly impacts patient survival.
  • Histologically confirmed amyloidosis warrants echocardiographic screening due to the high prevalence and poor prognosis associated with cardiac involvement.