Autoimmunity plays a role in the pathogenesis of numerous cardiac diseases such as dilated cardiomyopathy (DCM), myocarditis, rheumatic fever and atherosclerosis. An autoimmune response may appear following myocardial injury and exposure to self-antigens. Anti-heart autoantibodies have been identified in patients with heart disease and in low titres of healthy individuals. Troponin is the preferred marker in detecting acute coronary syndrome. In recent years, anti-troponin autoantibodies were identified in patients with DCM and ischaemic cardiomyopathy. The presence of anti-troponin autoantibodies in the serum may cause a false-negative evaluation of troponin levels and delay treatment of acute coronary syndrome. The role of anti-troponin autoantibodies in humans and their possible involvement in the pathogenesis of heart disease remains controversial. Previous studies have demonstrated that anti-troponin autoantibodies can cause cardiac dysfunction in animal models. This paper reviews current knowledge on anti-troponin autoantibodies. Future research should focus on the pathogenic role of anti-troponin autoantibodies and reactive T cells and the possible benefits of targeted therapy in acute coronary syndrome, post-myocardial infarction, myocarditis, DCM and heart failure.