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
- Usually presents over age 40 with progressive biventricular failure
- Symptoms are Fatigue, Chest pain, Peripheral edema and Dyspnea
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
- Low voltage
- Pseudo-Infarction pattern
- Rhythm/conduction disturbances
Chest X-ray
- Mildly to moderately enlarged cardiac silhouette
M-Mode/2-D
- Granular appearance of the myocardium
- Thickened left ventricular wall
- Left ventricular size is normal or small
- Papillary muscles may be thickened
- Right ventricular free wall is thickened
- Global left ventricular function may be normal or decreased
- Segmental wall motion abnormalities are common (septum, inferior wall)
- Atrial enlargement
- Pericardial/pleural effusion
- Thickened valves
- Thickened interatrial septum
Doppler
Early amyloidosis:
- Insufficiency of all four valves is possible (usually mild)
- Decreased mitral E velocity
- Increased mitral A velocity
- Decreased E/A velocity ratio
- Prolonged deceleration time (> 240 msec)
- Prolonged isovolumic relaxation time (> 86 msec)
- Increased systolic, decreased diastolic filling in pulmonary vein
- Increased systolic, decreased diastolic filling in hepatic vein.
Advanced amyloidosis:
- Insufficiency of all four valves is possible (usually mild)
- Normal to increased mitral E velocity
- Decreased mitral A velocity
- Increased E/A velocity ratio
- Decreased deceleration time (< 160 msec)
- Normal to slightly decreased isovolumic relaxation time
- Decreased systolic, increased diastolic filling in pulmonary vein
- Decreased systolic, increased diastolic filling in hepatic vein