Succinate dehydrogenase (SDH) is an enzyme linking the respiratory chain with the citric acid cycle. Cardiac-specific loss of SDH and other defects contributes toward molecular basis of the pathology of the cardiac disorders. Various cardiac disorders, namely ischemia, Barth syndrome, etc., have been well studied toward understanding the role of SDH in disease pathology. In animal models, inhibition of SDH with malonate at the initiation of reperfusion has reduced generation of ROS and thus lowered the size of infarct. It also lowers the opening of mitochondrial permeability transition pore. SDH inhibitors such as diazoxide and 3-nitropropionic acid have also exhibited cardioprotective effect. Among cardiac arrest cases, brain damage is identified as the major cause of death. The mechanism related to succinate accumulation during ischemia by SDH is proposed. Thus, it is apparent to target succinate metabolism toward the development of potential therapeutics to treat cardiovascular diseases.