Grant: Operating Grant

Hereditary cardiomyopathy is defined as a genetic disease of the heart that is characterized by an increase in heart size which leads to heart failure and death. Recent work from our laboratory showed in an animal model of hereditary cardiomyopathy, a weakness of blood vessels which is associated with what we call hypotension. Thus, it is possible to postulate that hereditary cardiomyopathy is not only a disease of the heart muscle but it is also a disease of blood vessels. Therefore, it is important to better understand the contribution of the weakness of blood vessels in the development of hereditary cardiomyopathy and sudden death. This will help to develop a new treatment for such a killing disease. Several drugs were reported to slow down the development of heart failure but not the survival of patients suffering from hereditary cardiomyopathy. Recent work in the literature as well as those recently reported by our group showed that the interior of heart cells was more acidic than that of normal cells and treatment to reduce the acidity of the cell completely abolishes the progression of the heart towards heart failure and premature death. The objective of the project is to test the hypothesis that as for the heart, the interior of cells of blood vessels is more acidic than normal and that reduction of the acidity with new generation of drugs will not only prevent the progression of heart disease but also the associated weakness of blood vessels. This work will for sure not only help us to better understand the reason of weakness of heart and blood vessels functioning in hereditary cardiomyopathy but also find a cure of this high killing disease.