Treatment strategy for cardiomyopathy of Duchenne muscular dystrophy

Abstract

Cardiomyopathy is a leading cause of premature death in patients with Duchenne muscular dystrophy (DMD). First–choice medication is carvedilol for patients with tachycardia, or enalapril for patients without tachycardia. A gradual up–titration of these medications easily causes hypotension with systolic blood pressure of 80 to 90 mmHg. If the tachycardia persists, starting ivabradine with a low dose can be a treatment option. If the LV dilatation or overhydration status exists, MRA could be another option. Although SGLT2 inhibitors are expected as a new option, there are concerns that SGLT2 inhibitors may cause body weight and muscle burden losses. Indications of diuretics and digitalis should be carefully considered because not a few patients have latent or underestimated renal dysfunction. In any case, we recommend to start medical treatment in the early age (5 to 10 years of age) to prevent future progression of cardiomyopathy, for the effectiveness can be limited in the late stage of progressive cardiomyopathy.