for BTHS, and treatment of BTHS is convoluted and delayed due to the disease's varying phenotypes and its sheer complexity. Thus, many treatments focus on cardiovascular and metabolic disorders, rather than treating the symptom itself. Elamipretide, an agent which protects CL from oxidative damage to maintain mitochondrial cristae and oxidative phosphorylation, is currently being tested in clinical trials. Further, dietary fatty acids have been used to enhance bioenergetics and cardiac function in BTHS. However, severe manifestations of the symptoms in BTHS patients require heart transplantation. Statistics show nine out of 73 (12%) surviving patients who have undergone cardiac transplantation at the last update. Heart transplantation in BTHS patients has generally been successful. Cardiovascular pathology Cardiomyopathy is a prominent feature of Barth syndrome. The change in acyl chain composition and lipid peroxidation caused by defective tafazzin can cause defective sarcomeric action, which may lead to an insufficient power stroke, severely weakened tissue, enlarged left ventricle, partial or incomplete contraction, and decreased ejection volume. Such consequences contribute to the cardiomyopathic phenotypes of Barth syndrome, marked by a weakened heart and diminished contractility. Alternatively, reactive oxygen species (ROS) has been suggested as the primary cause of cardiovascular impairments in BTHS. Cardiomyopathy in BTHS is exhibited at varying levels. A cohort study of BTHS patients showed 41.5% of all diagnosed cardiomyopathies in the range from birth to one month of age, and 95% exhibited a history of cardiomyopathy. Furthermore, there have been cases with mild or late-onset cardiomyopathies, such as two infantile patients without cardiomyopathic phenotypes at the time of diagnosis. Cardiomyopathy in Barth syndrome is primarily exhibited in multiple forms, including dilated cardiomyopathy (DCM), left ventricular condition in which the heart becomes weakened and enlarged, and therefore cannot pump blood efficiently. The resulting decrease in blood flow can lead to swelling in the legs and abdomen, fluid in the lungs, and an increased risk of blood clots. Some mutations in the TAFAZZIN gene cause dilated cardiomyopathy without the other features of Barth syndrome. LVNC is a condition in which the left ventricle, characterized by a spongy structure on the ventricular wall, exhibits prominent trabeculations and deep intertrabecular recesses. INVM occurs when the lower left chamber of the heart (left ventricle) does not develop correctly. In INVM, the heart muscle is weakened and cannot pump blood efficiently, frequently causing heart failure. Abnormal heart rhythms (arrythmias) can also occur. Musculoskeletal pathology Musculoskeletal pathology is exhibited