Cardiac Amyloidosis/ Amyloid Heart Disease

Fresh postmortem heart from 42 year old woman with cardiac amyloidosis showing thickened left ventricular walls with a small cavity, features that superficially resemble symmetrical hypertrophy in hypertrophic cardiomyopathy both on echocardiography and at necropsy. Source

Cardiac involvement is the most common cause of death in patients with amyloidosis associated with an immunocyte dyscrasia – typically as restrictive cardiomyopathy.

Physical examination
Physical examination reveals right heart failure with a raised jugular venous pressure (JVP), characteristically showing a prominent deep Y descent. The heart size is often normal. The physical findings are very similar in constrictive pericarditis (CCP), but the apex is frequently non-palpable due to the thick pericardium.


Investigations
The chest X-ray may show pericardial calcifications in patients with constrictive pericarditis.
The most characteristic ECG finding of restrictive cardiomyopathy is diffusely diminished voltages.
Echocardiography typically shows small thick ventricles and a thick interatrial septum due to amyloid deposits, which have a ‘granular sparkling’
appearance.

Pericardial effusion is common, but rarely causes tamponade.

Picture source

Further reading

1. Aetiology, diagnosis, investigation, and management of the cardiomyopathies.
2. Amyloidosis. Also a Heart Disease