The function of the heart is highly dependent on oxidative energy that is generated in mitochondria. Defects in mitochondrial structure and function can be found in association with cardiovascular diseases. Mitochondria and the Heart discusses the role that mitochondria plays in cardiovascular disease, including biogenesis and function of cardiac mitochondria during normal growth, development and aging. In addition, nonbioenergetic, biogenesis and degradation pathways are explored. Understanding these pathways and the effects that mitochondrial defects have in cardiac pathology is extremely important in establishing the diagnosis and treatment of mitochondrial-based cardiac diseases.