Thesis (Ph.D.) - Cairo University - Faculty of Medicine - Department of Radio-diagnosis

In this study including ischemia, microvascular dysfunction, and obstruction. However, recent observations have challenged this interpretation showing that the stimulus for myocardial fibrosis is an early manifestation of sarcomere - gene mutations that may be present before occurrence of cardiac morphological features of HCM. Myocardial fibrosis is present in the majority of patients with overt HCM, and corresponds with impairment in myocardial energy metabolism, and correlates with the severity of ventricular dysfunction. Use of late Gd imaging has become a well - accepted technique to depict focal myocardial fibrosis correlating well with the autopsy findings. There is an increasing body of evidence that the presence and extent of myocardial fibrosis, in patients with HCM, is an independent predictor of adverse outcome, i.e., SCD, sustained ventricular tachycardia or fibrillation, and heart failure. Also in patients with no, or minimal symptoms, late Gd imaging adds prognostic value to conventional criteria for risk stratification. Moreover, myocardial fibrosis is related not only to an increased risk for SCD, but also to the presence of progressive disease and prediction of systolic and diastolic dysfunction

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