In 1987 Dr. James published a paper in the British Heart Journal, where he showed the interaction of the different components of the heart, contributing to the development of cardiomyopathy. The molecular deficit, caused by Frataxin deficiency, could very well lead to cardiac neuropathy, indicating impaired nerve supply to the heart, coronary artery disease and changes in the myocardium, and resulting in cardiomyopathy. The diseased nervous system and coronary artery system can also contribute to the development of the cardiomyopathy. In discussing the cardiac problems, we will focus on the symptoms of the various cardiac diseases, the diagnosis and, in addition, the therapeutic interventions. First of all, the correct diagnosis for Friedreich's Ataxia involves molecular genetics to show the Frataxin mutations.
Electrocardiography is used to evaluate the cardiac rhythm and conduction.
Echocardiography can be used to visualise the cardiac compartments and the cardiac tissue. A similar more advanced technique is provided by magnetic resonance imaging, which reveals even more details. In some instances it may be necessary to obtain a tissue sample. The appropriate therapy would involve a pacemaker to correct the slow heart rate. Tachycardias can be recognised through the increased heart rate.