Arrhythmogenic Right Ventricular Cardiomyopathy ( ARVC )
primay disease of heart muscles
- occure in the form of fibrofatty changes in right ventricule and subepicardial space in left ventricle
- However its name, it's biventricular involvment and more common in left ventricle
- in left ventricle changes are related more to fibrosis than to fat or fibrofatty infiltration
- This condition causes part of the myocardium to break down over time, increasing the risk of an abnormal heartbeat (arrhythmia) and sudden death.
Etiology
unknown but ....
- prescence of inflammatory infiltartion suggest resolving myocarditis
- families has phenotyping alteration
- Gene mutations have been found in about 60 percent of people with ARVC. Mutations in a desmosomal gene called PKP2 appear to be most common.
- 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 (such as during vigorous exercise). These changes primarily affect the myocardium surrounding the right ventricle, one of the two lower chambers of the heart. 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 electrical signals that control the heartbeat, which can lead to arrhythmia.
findings
- symtoms and signs related to arrhythmia and conduction disturbance
- Arrhythmia and cardiac arrest are common presentation then cardiac arrest
- Autopasy is the only diagnostic test
under microscope
- moth eaten appearance
- destruction of normal myocytes via inflammatory scaring and fat replacement
