Myocardial fragmentation associated with disruption of the Z-band in hypertrophic cardiomyopathy in Noonan syndrome

Abstract

A 13-year-old female with Noonan syndrome had been diagnosed with hypertrophic cardiomyopathy, and she died of heart failure at the age of 25 years. Light microscopic and electron microscopic examination of her biopsied myocardium and autopsy heart showed myocardial fragmentation associated with Z-band disruption as well as myocardial hypertrophy and disarray with interstitial fibrosis. Myocardial fragmentation associated with Z-band disruption may be related to the progression of cardiac dysfunction.

Keywords: Dilated phase hypertrophic cardiomyopathy; Heart failure; Sarcomere.