Glucocorticoides y manejo cardíaco en pacientes con distrofia muscular de Duchenne

Introduction: Cardiac complications in patients with Duchenne muscular dystrophy are an important marker of morbidity and mortality. Glucocorticoids have been shown to prolong ambulation time and decrease cardiac complications, increasing life expectancy in these patients. This article makes a series of recommendations on glucocorticoid treatment and managing cardiac manifestations in patients with Duchenne muscular dystrophy. Materials and methods: An expert panel of 25 medical specialists with knowl-edge and experience in managing patients with Duchenne muscular dystrophy was formed. The experts elaborated a series of recommendations based on the review of the available evidence, their knowledge and clinical experience, and the characteristics of the Colombian health system. The recommendations were submitted to a vote with a threshold of favorability greater than 85 %. If the percentage of agreement was lower than the established threshold, the recommendation was discussed again, and a second voting cycle was carried out. Results and discussion: Initiation of glucocorticoids (deflazacort or prednisolone) is recommended when the patient’s motor function is stable (4-6 years of age), with a 25-33 % reduction in case of uncontrollable adverse events. Using clinical and functional scales helps to recognize the moment of stability. Likewise, a comprehensive cardiovascular evaluation is recommended with annual follow-up in asymptomatic patients and every 3-6 months in symptomatic patients (echocardiography is the imaging of choice). At 10 years or earlier, if there is evidence of myocardial involvement, the administration of an angiotensin-converting enzyme inhibitor or angiotensin receptor antagonist is recommended.