Diagnosis of Cardiac Amyloidosis: Clinical and Echocardiographic Features

Abstract

Purpose of Review

This review is geared to address the various forms of amyloidosis which have cardiac involvement and discuss the echocardiographic findings that may be present in patients with cardiac amyloidosis.

Recent Findings

The use of echocardiography has revolutionized the diagnosis of cardiac amyloidosis. The diagnosis is often first suspected due to characteristic echocardiographic findings. Although no single finding is pathognomonic, there are a constellation of features that point to the diagnosis including increased left and right ventricular wall thickness, thickened cardiac valves and atrial walls, and a pericardial effusion. Alterations in diastolic function with evidence of elevated filling pressures are common. The use of myocardial strain imaging has allowed for earlier detection of cardiac amyloidosis and has revealed a distinctive pattern, sometimes suggesting the diagnosis when more classic findings are subtle or absent. Multiple echocardiographic findings have been found to be prognostic in cardiac amyloid. Using current techniques, functional measures such as myocardial strain and stroke volume index are more powerful predictors of outcome than myocardial thickness. While other imaging modalities are complementary including cardiac magnetic resonance imaging and nuclear medicine scintigraphy techniques using bone tracers, echocardiography remains a mainstay in the diagnosis and follow-up of patients with cardiac amyloidosis.

Summary

Echocardiography is a critical tool in the evaluation of the patient with heart failure, as well as those with unexplained chest pain and arrhythmias. Key findings on an echocardiogram can help alert the clinician to the presence of cardiac amyloidosis. The degree of cardiac dysfunction in a patient with systemic amyloidosis correlates with prognosis. Recognition of subtle echocardiographic findings may allow for earlier and more aggressive treatment to improve overall mortality in patients with cardiac amyloidosis.

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Correspondence to Rekha Mankad.

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Mankad, R., Grogan, M. Diagnosis of Cardiac Amyloidosis: Clinical and Echocardiographic Features. Curr Cardiovasc Imaging Rep 11, 32 (2018). https://doi.org/10.1007/s12410-018-9472-2

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Keywords

  • Cardiac amyloidosis
  • Diastolic dysfunction
  • Light chain
  • Transthyretin
  • Global longitudinal strain
  • Pyrophosphate