4. CLASSIFICATION
1 1primary
Primary
a) Lofeller's endocarditis
b) Endomyocardial fibrosis
Secondary
a) Infiltrative disease
b) storage disease
c) Post - radiation disease
13. Arrhythmogenic right ventricular
dysplasia (ARVD) is a rare
familial disorder that may cause
ventricular tachycardia and
sudden cardiac death in young,
apparently healthy individuals.
The clinical hallmark of the
disease is ventricular
arrhythmias, arising
predominantly from the right
ventricle. The pathological
hallmark of the disease is
fibrofatty replacement of right
ventricular myocardium.
14. CAUSE
ARVD/C is caused by mutations in genes
that encode desmosomal
proteins. Desmosomes attach heart
muscle cells to one another, providing
strength to the myocardium and playing a
role in signaling between neighboring cells.
Mutations in desmosomal genes impair the
function of desmosomes. Without normal
desmosomes, cells of the myocardium
detach from one another and die,
particularly when the heart muscle is
placed under stress.These changes
primarily affect the myocardium
surrounding the right ventricle The
damaged myocardium is gradually
replaced by fat and scar tissue. As this
abnormal tissue builds up, the walls of the
right ventricle become stretched out,
preventing the heart from pumping blood
effectively. These changes also disrupt the
