Management of Cardiac Involvement in Becker Muscular Dystrophy: A Case Report

Becker muscular dystrophy (BMD) is an X-linked recessive neuromuscular disorder caused by a mutation in the dystrophin gene. Cardiac involvement is a frequent finding in BMD, and manifestations may vary from asymptomatic cardiac involvement to developing symptoms of heart failure and severe cardiomyopathy. We presented the case of a 32-year-old wheelchair-dependent BMD patient who came to our cardiology clinic with a two-month history of heart palpitations, rest and nocturnal dyspnea, fatigue, and generalized muscular weakness. Upon evaluation, a 24-hour Holter rhythm showed complex ventricular arrhythmia and 300 polymorphic ventricular extrasystoles with episodes of ventricular bigeminy, while echocardiography revealed a dilated left ventricle with severe systolic dysfunction (left ventricular ejection fraction (LVEF) 23%) and impaired global contractility. An implantable cardioverter defibrillator (ICD) was implanted, and guideline direct medical therapy (GDMT), sacubitril/valsartan, bisoprolol, furosemide, spironolactone, and dapagliflozin were initiated. The patient was discharged five days later, in an improved clinical condition, without dyspnea. A follow-up appointment two weeks after discharge was recommended in order to evaluate the patient's symptoms and the effectiveness of GDMT and a follow-up echocardiography at least three months after discharge to evaluate the heart's systolic and diastolic function.