(P4) Cardiac: Endocardial fibroelastosis

Question 1

Identify slide

Answer 1

Endocardial fibroelastosis

1. cardiomyocytes
2. thickened endocardium
3. Congenital disorder—>first two years of life
4. left ventricle

Question 2

identify slide

Answer 2

• Endocardial fibroelastosis*
• (Uncommon disease)*
• -Symptomatic before 2nd year of life*
• -Characterized by focal or diffuse endocardial thickening (increased amount of elastic fibers) and myocardial dysfunction*
• -It often occurs with aortic valve obstruction*

Question 3

Identify slide

and

provide histological features:

Answer 3

• Endocardial fibroelastosis*
• • The endocardial layer is approximately 2‐3 mm thick and sharply demarcated from the underlying muscle*
• -The left ventricle is markedly hypertrophic*
• -Histologically: there is an increase in collagenous and elastic fibers beneath the endocardium*
• • Etiology is still unknown*

Question 4

Identify slide

and

this condition accompanied by?

Answer 4

Endocardial fibroelastosis

• Accompanied by some form of congenital cardiac anomaly
• Congenital disorder: Focal or diffuse

Focal – may have no functional importance

Diffuse – may cause rapid and progressive decompensation and death

Question 5

• Endocardial fibroelastosis*
• Macroscopic:*

Answer 5

Pale layer in the heart

-Thickening of the endocardium (normally endocardium is translucent. Becomes thicker in disease)

—-left atrium with endocardial fibro-elastosis

—-short, thickened chordae tendinea with direct insertion of posterior mitral valve leaflet into-papillary muscle

—-thickened dysplastic mitral valve with stenosis

-endocardial thickening (formation of hyaline layer), myocardial dysfunction w/aortic valve obstruction (mitral valve)

Question 6

• Endocardial fibroelastosis*
• Primary:*
• Secondary:*

Answer 6

Primary: Newborns –> 4-10 mos old (less common)

leading to death at early age

Secondary: more common, accompanies congenital cardial anomaly

Question 7

identify

Answer 7

Question 8

The most typical anatomical location

of the lesion is?

Answer 8

Left Ventricle