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Review
. 2005;32(2):178-84.

Amyloid heart disease. New frontiers and insights in pathophysiology, diagnosis, and management

Walid Hassan  1 , Hani Al-Sergani, Walid Mourad, Rashed Tabbaa
Affiliations
Review

Amyloid heart disease. New frontiers and insights in pathophysiology, diagnosis, and management

Walid Hassan et al. Tex Heart Inst J. 2005.

Abstract

Amyloidosis comprises a unique group of diseases that share in common the extracellular deposition of insoluble fibrillar proteins in organs and tissues. Cardiovascular amyloidosis can be primary, a part of systemic amyloidosis, or a result of chronic systemic diseases elsewhere in the body. The most common presentations are congestive heart failure-mainly a restrictive infiltrative pattern--and conduction system disturbances. Recent developments in imaging techniques and extracardiac tissue sampling have minimized the need for invasive endomyocardial biopsy for amyloidosis. Despite advances in treatment, the prognosis for patients with amyloidosis is still poor and depends on the underlying disease type. Herein, we present new insights and recent advances in cardiovascular amyloidosis.

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Figures

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Fig. 1 Photomicrograph of a gastric biopsy specimen of a patient with primary amyloidosis shows plasma cells with expression of kappa light chain (Immunoperoxidase, orig. ×400).
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Fig. 2 Photomicrograph of a gastric biopsy specimen of a patient with primary amyloidosis shows plasma cells lacking expression of lambda light chain (Immunoperoxidase, orig. ×400).
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Fig. 3 Doppler echocardiographic study of a patient in an early stage of cardiac amyloidosis reveals an impaired relaxation pattern with reversal of E/A ratio.
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Fig. 4 Doppler echocardiographic study of a patient in a late stage of cardiac amyloidosis reveals a severe restrictive pattern.
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Fig. 5 Electrocardiogram of a patient with cardiac amyloidosis shows a diffuse low-voltage QRS complex.
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Fig. 6 Echocardiogram of a patient with immunoglobulin (AL) amyloidosis shows thickened ventricular and atrial walls, abnormal myocardial texture, atrial dilatation, and pericardial effusion. LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle
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Fig. 7 Photomicrograph of an endomyocardial biopsy specimen in a patient with immunoglobulin amyloidosis exhibits interstitial eosinophilic amyloid deposits (arrow) (H & E, orig. ×400).
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Fig. 8 Photomicrograph of endomyocardial biopsy specimen in a patient with immunoglobulin amyloidosis exhibits the metachromatic appearance of amyloid (AL) (Congo red stain, orig. ×400).
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Fig. 9 Electron micrograph of primary amyloidosis shows the characteristic amyloid fibrils (AL) (Lead citrate, orig. ×1500).
See this image and copyright information in PMC

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