Rimeporide (EMD-87580) is an Na/H-exchanger-1 (NHE-1) inhibitor and was initially developed for heart failure. This compound reduces the development of both necrosis and hypertrophy, and it was shown to prevent early death in hereditary cardiomyopathy. Rimeporide regulates sodium, pH, calcium overload, reduces inflammation in several muscles, and decreases skeletal, diaphragm and cardiac fibrosis, and muscle cell degeneration. It was therefore further developed as a drug for treatment in Duchenne muscular dystrophy and other muscular dystrophies. Rimeporide is expected to act as a muscle-sparing agent. A phase I study has been completed in 2018.