• calcification as well as massive fibrosis is seen in the right ventricle of these mutants with almost 100% penetrance

• degeneration occurs from week 6 to week 10 after birth and proceeds from the outer part of the right ventricular wall to the inner part

• the left ventricular wall is unaffected

• the duration of ventricular muscle depolarization is prolonged indicating a greater susceptibility to arrhythmia