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Cardiac Amyloidosis: The Importance of Surveillance in a Patient With an Initial Negative Cardiac Biopsy

Rajiv Ananthakrishna1, Rachael Lloyd1, Bryone J Kuss2

  • 1Department of Cardiovascular Medicine, Flinders Medical Centre, Adelaide, Australia.

JACC. Case Reports
|July 28, 2021
PubMed

Insights

Rapidly progressive light-chain cardiac amyloidosis can be challenging to diagnose. Close patient monitoring is crucial, even with non-definitive initial biopsy and imaging results for this serious heart condition.

Area of Science:

  • Cardiology
  • Nephrology
  • Oncology

Background:

  • Cardiac amyloidosis is a progressive and often challenging diagnosis.
  • Light-chain (AL) amyloidosis can affect the heart, leading to heart failure.
  • Early diagnosis is critical for effective management and improved patient outcomes.

Observation:

  • A case of rapidly progressive light-chain cardiac amyloidosis is presented.
  • Initial diagnostic findings from biopsy and imaging were not pathognomonic for amyloidosis.
  • The patient's condition progressed rapidly despite initial inconclusive results.

Findings:

  • The case underscores the diagnostic challenges in cardiac amyloidosis.
  • Emphasizes the need for vigilant monitoring in suspected cases.
  • Highlights that non-pathognomonic initial findings do not exclude the diagnosis.

Implications:

  • Increased awareness of rapidly progressive forms of cardiac amyloidosis is needed.
  • Clinicians should maintain a high index of suspicion for amyloidosis.
  • Close follow-up and reassessment are vital when initial tests are equivocal.

Keywords:
AL, light-chainATTR, transthyretinCA, cardiac amyloidosisCMR, cardiac magnetic resonanceECV, extracellular volumeMGUS, monoclonal gammopathy of unknown significanceNT-proBNP, N-terminal pro-B-type natriuretic peptideamyloidosisbiopsycardiac magnetic resonance

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