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Amyloidosis

Cardiac amyloidosis is the most common restrictive cardiomyopathy in the Western world. Senile amyloid deposits, which can occur without systemic amyloidosis, are common in patients over 80 years old.

Definition

A multisystem disease in which there is extracellular deposition of the amyloid protein in the kidney, heart, liver, nerve, skin, and tongue

Signs and Symptoms

Pathology

Grossly, in advanced cases, the heart has a firm, rubbery consistency and is mildly to moderately enlarged with thickened ventricular myocardium and valves, and enlarged atria. Histologic examination demonstrates focal and diffuse, microscopic and macroscopic interstitial deposition of amyloid in the myocardium, endocardium, and pericardium. Amyloid can also be found within the walls of intramural coronary arteries and within the conduction system.

Electrocardiogram

Chest X-ray

M-Mode/2-D

Doppler


Early amyloidosis:

Advanced amyloidosis:

 

 

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